#diffuse scleroderma

[aelianated-star]

Hypokalemia, hypokalemia when I catch you hypokalemia.. Having to get potassium chloride replacement should be considered cruel and unusual istg

[rajpalsachin]

Abdominoplasty in Delhi : What Women Should Know About Menstrual Health Post-Surgery-Dr. Sachin Rajpal

Alopecia also known as hair loss or baldness, refers to a loss of hair from part of the head or body. The head is most commonly involved. The severity of hair loss can vary from a small area to the entire body. Hair loss may occur naturally or it may be related to disease or the use of certain medications. Symptoms of alopecia vary depending on the cause of the condition and range from a small bald patch to a complete loss of all body hair. Diffuse alopecia (thinning scalp hair) is common among seniors, both men and women. There are a number of different types of alopecia and some of them are more common.

Male-Pattern baldness

This is the most common form of alopecia. Also called androgenetic alopecia, this form of baldness is hereditary and is due to a combination of genetics and the male hormone dihydrotestosterone. In male-pattern baldness, the hairline typically recedes and the hair becomes thin.

Female-Pattern baldness

Baldness can also affect women, seems to be more likely to affect post-menopausal women, as a result of hormonal changes.

Alopecia areata

Also referred to as patchy baldness. Men, women and children of any age may be affected and usually the patches occur on the scalp. It is caused by % c+ immune system disorder and people with autoimmune conditions are more likely to be affected.

Anagen effluvium

This is a form of hair loss that may affect the whole body, scalp and face. Anagen effluvium mostly occurs after chemotherapy, radiotherapy or immunotherapy for treating cancers. The alopecia is usually temporary and hair regrows after treatment is stopped.

Telogen effluvium

In this form hair fall is more than usual. Head hair does feel thinner, but complete hair loss is unlikely. The condition is temporary. Telogen effluvium can be caused by the following:

Extreme emotional or physical stress

Hormonal changes

Chronic diseases

Dietary changes or crash dieting

Severe infections

Certain medications

Scarring alopecia

Also called cicatricial alopecia. This is a permanent hair loss caused by rare conditions such as scleroderma and discoid lupus. The hair follicles are completely destroyed and hair does not grow again.

Treatment

If your hair loss is caused by an underlying disease, treatment for that disease will be necessary.

Medications are available are:

Finasteride and minoxidil can be used to treat male-pattern baldness and the latter is also a treatment for female-pattern baldness.

Alopecia areata may be treated with steroids or immunotherapy.

PRP Therapy or platelet-rich plasma therapy is a very effective and popular treatment where PRP is derived from patients’ blood is injected in the affected area. It stimulates hair regrowth from hair follicles and stops hair fall. Low-level laser therapy and micro needling also improve hair density.

Alopecia may also be treated using surgical methods such as hair transplants or implants. Alternatively, a person may decide to use a wig, which may be synthetic or made of real hair.

For More Information: https://www.skinhairandyou.com/

[nimbanaturecure]

Understanding Pulmonary Fibrosis: 5 Key Insights for Patients

Pulmonary fibrosis is a severe, chronic disease of the lungs that results in fibrotic changes of lung parenchyma and makes it difficult to breathe. Although this disease is difficult, having proper knowledge about it can be insightful to patients and caretakers in decision-making processes. At Nimba Nature Cure, we value natural ways of handling various symptoms and forms of ailment, such as pulmonary fibrosis cure. Here are seven key aspects everyone should know about this condition:

1. What Is Pulmonary Fibrosis?

Pulmonary fibrosis is a lifelong disease that affects the human lungs and is defined by fibrosis of the lung tissues. This scarring gradually becomes relatively firm and dense in the tissue that surrounds and separates alveoli, which will limit the diffusion of oxygen into the bloodstream. This condition degeneratively impairs the delivery of oxygen and the overall oxygen concentration within the person. It is a chronic and disabling disease that may also affect medium and, sometimes, even simple movements such as walking or going up a staircase.

As of now, pulmonary fibrosis has no known cure; however, with improvements in medical practice and package management and without overlooking integrated medicine, results are slowly putting hope in patients with the disease. Those affected by the disease need to know how it develops and what can be done to minimise suffering and enhance the quality of life.

2. Symptoms to Watch For

The symptoms of pulmonary fibrosis can vary but often include:

Shortness of breath: This is one of the first and most apparent signs usually experienced during physical exertion.

A dry, persistent cough: May not respond to most commonly used medications.

Fatigue and weakness: One of the consequences of the hypoxemia that characterizes patients may be extreme fatigue at all times.

Unexplained weight loss: A slow and progressive loss of appetite and weight. 

Muscle and joint pain: These symptoms may occur because of inflammation that is associated with the condition.

Clubbing of fingers and toes: major features of advanced pulmonary diseases.

This will point to the fact that it is easier to intervene for more effective changes once these symptoms are realized. One reason is that putting off a diagnosis may enable the ailment to get worse than it would have when diagnosed earlier.

3. Causes and Risk Factors

While the exact cause of pulmonary fibrosis is often unknown (idiopathic pulmonary fibrosis), some common risk factors include:

Environmental Exposure: high-risk jobs that involve extended periods of exposure to toxic materials, inclusive of asbestos, Silica or some industrial chemicals.

Smoking: An important causative agent of lung diseases, including pulmonary fibrosis.

Medical Conditions: Other diseases like rheumatoid arthritis, scleroderma or lupus may cause lung fibrosis.

Medications: It has been identified that some drugs, particularly some types of chemotherapy agents, can cause pulmonary fibrosis as one of the side effects.

Genetics: A history of the condition or related diseases also predisposes an individual.

Infections: Possible other factors include chronic infections or exposure to viruses, for instance, the Epstein-Barr virus.

Knowledge of these risk factors can be useful in helping prevent or at least earliest identification of at-risk persons.

4. Pulmonary Fibrosis Diagnostics

Proper diagnosis of the condition is very vital in necessary management. Diagnostic tools include:

Imaging Tests: There is often evidence of scarring patterns in the lungs, and CT scans with high resolution can detect these findings.

Lung Function Tests: These record lung volume and the efficiency with which oxygen is being transported to the blood.

Bronchoscopy: This procedure checks the airways and may exclude other causes of the symptoms in question.

Biopsy: A biopsy of a lung sample is taken to ensure the fibrosis since a small sample is involved.

There is a need to seek a pulmonologist to determine the exact cause and then advise on the most appropriate management.

5. Conventional and Holistic Pulmonary Fibrosis Treatment

Treatment approaches aim to slow disease progression and alleviate symptoms. Conventional methods include:

Medications: Antifibrotic drugs like pirfenidone and nintedanib can reduce scarring.

Oxygen Therapy: Improves oxygen levels in the blood, alleviating symptoms like fatigue and breathlessness.

Pulmonary Rehabilitation: A comprehensive program that includes physical exercise, nutritional advice, and psychological support.

Lung Transplantation: In severe cases, this may be an option for eligible patients.

At Nimba Nature Cure, we incorporate holistic therapies to complement conventional treatments:

Naturopathy and Ayurveda: These ancient practices focus on restoring balance within the body.

Yoga and Breathing Exercises: Techniques such as pranayama help strengthen lung capacity and manage stress.

Dietary Interventions: Anti-inflammatory diets can mitigate symptoms and improve overall health.

Holistic treatments emphasize natural healing and aim to improve both physical and mental well-being.

At Nimba Nature Cure, we aim to provide a holistic and natural approach to managing chronic conditions like pulmonary fibrosis. Through a combination of naturopathy, yoga, and tailored therapies, our treatments focus on improving lung function and enhancing overall wellness. If you or a loved one is dealing with pulmonary fibrosis, don’t hesitate to explore holistic pulmonary fibrosis treatment options at our center.

Read More:- Understanding Pulmonary Fibrosis: 5 Key Insights for Patients.

[velammalhospital]

What Are the Main Causes of Hair Loss in Men and Women?

Hair loss or alopecia is a common condition that can affect both men and women due to a variety of causes. Below is a detailed overview of the main 

Androgenetic Alopecia

Androgenetic alopecia, also known as pattern hair loss, is the most common cause of hair loss. It is a hereditary condition influenced by the hormone dihydrotestosterone (DHT) which causes hair follicles to shrink over time. This leads to progressive thinning and hair loss typically resulting in a receding hairline and thinning at the crown in some cases or diffuse thinning along the crown and part line in others.

Alopecia Areata

Alopecia areata is an autoimmune disorder where the immune system attacks hair follicles, causing sudden and often patchy hair loss. This condition can affect any hair-bearing area, including the scalp, eyebrows and body hair. Although the exact cause is not fully understood, genetic and environmental factors are significant contributors.

Hormonal Changes

Hormonal fluctuations are a major factor in hair loss. Conditions such as pregnancy, postpartum recovery, menopause and hormonal imbalances, including increased androgens in disorders like polycystic ovary syndrome (PCOS) can disrupt the hair growth cycle and lead to shedding. In some cases hormonal medications or therapies can also impact hair health.

Stress and Telogen Effluvium

Significant physical or emotional stress can push a large number of hair follicles into the resting (telogen) phase, leading to a condition known as telogen effluvium. This results in noticeable shedding that is often temporarily triggered by events such as illness, surgery, childbirth or extreme psychological stress.

Traction Alopecia

Traction alopecia occurs due to repeated tension or pulling on the hair, often caused by tight hairstyles like braids, ponytails or buns. Over time this tension damages hair follicles causing localized thinning or bald spots. It is preventable if detected early and the tension on the hair is reduced.

Nutritional Deficiencies

Deficiencies in essential nutrients, such as iron, zinc, vitamin D and B12  can weaken hair follicles and contribute to hair loss. Malnutrition, restrictive diets and eating disorders often result in insufficient nutrients necessary for healthy hair growth.

Medical Conditions and Medications

Chronic medical conditions like thyroid disorders, diabetes  and autoimmune diseases such as lupus can lead to hair loss. Additionally medications including chemotherapy, beta-blockers, antidepressants and hormonal treatments may have hair shedding or thinning as a side effect.

Scalp Conditions

Scalp health is vital for healthy hair. Conditions such as fungal infections, seborrheic dermatitis, psoriasis and other inflammatory disorders can weaken the hair follicles and cause shedding or thinning.

Age-Related Hair Loss

As people age hair growth naturally slows down and the hair follicles shrink. This process often leads to thinner weaker hair and in some cases complete cessation of hair growth in certain areas.

Environmental and Lifestyle Factors

Environmental pollutants, smoking and the excessive use of heat styling tools, chemical treatments and harsh hair products can weaken hair shafts and follicles. Poor hair care routines and exposure to harmful substances contribute to hair thinning and breakage.

Autoimmune Disorders

Beyond alopecia areata other autoimmune conditions like lupus and scleroderma can cause systemic inflammation disrupting the hair growth cycle and leading to hair loss.

Hair loss can be challenging, but effective solutions are available at Velamma Hospital. With advanced treatments like PRP therapy, hair restoration procedures and scalp care options we address a variety of hair concerns.

Our team provides precise diagnoses and evidence-based treatments for long-lasting results. Whether it's pattern hair loss, thinning or alopecia. We aim to bring visible improvements.

Schedule a consultation today at Velamma Hospital https://www.velammalhospital.com/appointment.php

[rheumresidentnotes]

Notes on scleroderma

*Sclerodactyly Vs Scleroderma (sys.sclerosis)

Dryness doesn’t exceed MCPs vs exceed MCPs

Limited Vs diffuse sys.sclerosis

Dryness doesn’t exceed elbow vs exceed elbow

**Check 2 sys. Organs

Lung by HRCT chest + pulmonary function

Heart ( pulmonary HTN ) by Echo

*** TTT: Epilat Retard for Raynaud’s (CCB) ?

متابعة الضغط ممكن يوطى نبدأ تركيز ١٠ ٣ أيام و بعدها تركيز ٢٠

**** Check Scleroderma Mimics & criteria for Dx

[healthpediaindia]

Scleroderma

Scleroderma is an autoimmune connective tissue and rheumatic disease that causes inflammation in the skin and other areas of the body. When an immune response tricks tissues into thinking they are injured, it causes inflammation, and the body makes too much collagen, leading to scleroderma. Too much collagen in your skin and other tissues causes patches of tight, hard skin. Scleroderma involves…

[doctorfoxtor]

the way it makes me feel like, the way it makes me feel i never seem to act so stupid... oh here we go

100 days of productivity

day 46 + 47

CVS/RS

vitamin A oversupplementation is actually assoc w/ ↑risk of ca lung

MTX lung tox occurs within 4 months of starting, but rheumatoid organising pneumonia can occur at any point in the disease

inhaled steroids have one major systemic side effect and it is skin changes (thinning, fragility, easy bruising)

transbronchial biopsy is useless in IPF; only fibrosis w/ nothing to suggest aetiology will be seen, so take a surgical biopsy if needed

severe mitral stenosis → percutaneous mitral commissurotomy (if not suitable, transcatheter replacement)

CNS/Ophthal/Psych

low CSF pressure headache: LP, but also minor trauma to meninges, idiopathic (insidious); orthostatic headache, N/V, ↔ diplopia, ataxia/vertigo, stiff neck, back pain, VF abnormalities; ↓CSF opening pressure; MRI: diffuse pachymeningeal enhancement, tonsilar descent, post fossa crowding; conservative → epidural blood patch

vitreous haemorrhage in DM is an indication for photocoagulation and a hallmark of proliferative disease

vitamin E def → cerebellar ataxis, dysarthria!, �� myopathy

fluctuating eyesight in DM → fluctuating ocular glucose leading to variable osmotic pressures → fluctuating lens hydration → fluctuating lens shape

schizophrenia spectrum: if tx is started, it is effective if there is marked improvement within 2 wks and complete remission within 4-6 wks

Rheum/Derm/Immuno

lichen sclerosus → atrophy, 'cigarette paper' texture of indurated plaques, marked hypopigmentation

morphoea = 'localised scleroderma' → indurated annular plaques w/ advancing inflammatory edge and central atrophy

erythema nodosum causing drugs: omeprazole, sulphonamides, halides (iodides/bromides), gold (aurothiocyanate), HBV vaccine

Igs by serum concentration: G 75% > A 15% > M 10% > D 1% > E <0.1% (all approx)

GIT

vitamin B6 and D are assoc w/ reduced ca colon

autoimmune hepatitis: type 1 = nonspecific progressive fatigue w/ ↑LFTs, anti-smooth muscle; type 2 = acute episodes of jaundice, anti-LKM1

acute flare of IBD not responding to steroids: cyclosporin, infliximab take rapid effect (AZP/6-MP take weeks)

Onc/Haem

myeloma: thalidomide → bortezomib → lenalidomide; melphalan in very frail pts; cyclophosphamide as adjunct

immune thrombocytopaenia (idiopathic and lupus): platelet transfusion only if active bleeding AND plt <30k/mcl, otherwise can manage medically (steroids for ITP, HCQS → AZP/6-MP/ciclosporin for SLE)

Renal/Biochem

allopurinol actually has a role in reducing progression to diabetic nephropathy in diabetics w/ gout

vitamin E oversupplementation is assoc w/ ↑risk of ca prostate

ACEi+ARB together show benefit in only one condition: nephrotic-range proteinuria; otherwise, increased risk of renal damage and no clear benefit

Pharm/Toxo/Misc

specifically, slow acetylator status is assoc w/ N-acetyltransferase-2

incidentally, fast- status is assoc w/ INH-hepatits, characterised by microvascular fatty infiltration and necrosis

AZP can be continued in pregnancy; it is assoc w/ very light LBW but it is not otherwise life threatening or teratogenic (but withdrawing tx will be problematic for mother)

DNA polymorphism vs mutation: 1% in population is cutoff

[aelianated-star]

SOMEONE TELL ME WHY LIQUID NUTRITION/TUBE FORMULA AND HOMEHEALTH COMPANIES MAKE THEIR PACKAGES/BOXES SO DAMN HARD TO OPEN?? Like your customer base is malnourished /chronically ill/disabled people and y’all had the AUDACITY to make the boxes so hard to open that even my able bodied, male family members struggle to open it?? I know it’s expensive (god bless insurance actually paying for something for once bc there’s no way I could afford over 9 boxes a month at 72 dollars a box) and they’re trying to prevent theft, but I can guarantee that nobody who sees these shipments of giant liquid nutrition boxes is going to try and nab it.

[allergy-by-health-omni]

An Introduction to Scleroderma Allergic Reactions

Scleroderma Allergic Reactions

Scleroderma is a chronic and rare disease that lasts for a long time. It induces skin thickening, hardening and tightness and may also involve the internal organs of the body and in turn, lead to intestinal, heart, kidney and lung problems. Localized Scleroderma is involved only in the skin while generalized Scleroderma or systemic sclerosis allergic reactions involve internal organ involvement.

The most common skin Scleroderma allergic reactions are swelling of hands and toes, generalized itching, thickening, hardening and tightness of skin, change of the color of the hands and feet with cold exposure and sores over fingertips. The other Scleroderma allergic reactions include fatigue, heartburn, shortness of breath, arthritis, dryness of the eyes and mouth, cough and high blood pressure.

Scleroderma is basically a rare disease that occurs in 10 – 20 people amongst a million people in a year. Though people of all races can get Scleroderma, about 75% of the victims are women. Scleroderma can occur at any age but is most common in people between the ages of 30 and 60 years old. The exact causes of Scleroderma are not known; however it is known to be an autoimmune disease. This is where the body’s natural immune system does not actually behave normally; instead of fighting off infections from bacteria and virus, the immune system of a victim of Scleroderma attacks its own body.

With this, there is usually some damage to the blood vessels of the body. And in response to this blood vessel damage, specific proteins called collagen are produced to repair the damage to blood vessels through fibrosis or the formation of diffuse scars. There is usually an excess deposit of collagen in the skin that induces the skin changes associated with Scleroderma. The fibrosis of the blood vessels usually leads to the derangement of internal organs.

The diagnosis of Scleroderma allergic reactions is rather difficult, and can be done by specialists in autoimmune diseases. This diagnosis is based on a thorough history and physical examination of the victim along with some laboratory studies. Not only are blood and urine tests conducted, specialized lung and heart evaluations are required to evaluate the involvement of internal organs in Scleroderma.

With the diagnosis of Scleroderma, treatment can be started. There is basically no cure for Scleroderma as it is a chronic disease that requires medical therapy for many years. Usually organ specific treatments are used of treating Scleroderma with combinations of anti-inflammatory and immunosuppressant medicine. Sometimes other medication is used to control skin and internal organ problems and at times, chemotherapy may be used to control lung problems.

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[lhlr]

What is the effect of physical/exercise therapy on physical function in patients with systemic sclerosis?

Systemic sclerosis (SSc) is a relatively rare disease of the connective tissues in the body resulting in debilitating consequences. It is characterized by diffuse fibrosis and abnormalities within the connective tissue of the skin, joints, and eventually internal organs. Specific etiology is not known. Associated signs and symptoms include pain, edema, decreased range of motion, skin thickening, and joint contractures which are all associated with decreased functional ability and reduced quality of life. Unfortunately, research regarding nonpharmaceutical interventions for systemic sclerosis is extremely limited. Research and treatment that is available usually focus on skin lesions and complications to internal organs as the disease progresses. However, physical therapy and/or skilled exercise therapy may be able to address functional concerns and improve the quality of life in both the early and late stages of the disease.

Murphy, et al., utilized an eight-week, in-person, program consisting of tissue mobilizations, thermal modalities, upper extremity mobility, and a home exercise program in patients with early SSc (< 5 years) to determine possible benefits for upper extremity function. The study utilized a single group with pre- and post-test measures to determine improvement in the upper extremity QuickDASH, range of motion, coordination, skin thickening, and Patient-Reported Outcomes Measurement Information System (PROMIS). Patients, on average, had a moderate skin disease and the majority were also being treated with immunosuppressive therapy or were participating in clinical trials. Researchers found that there was not a meaningful improvement at four weeks into the program but did find significant improvement from baseline at the end of the eight weeks. On average, participants improved QuickDASH measures by 14 points, PROMIS by 3.1 points, 41.5 degrees of active motion, and were faster in completing the 9-hole peg test with the left hand. All are significant findings from baseline. However, no significant improvement was shown with right-hand coordination or active motion. Long-term effects were not evaluated, and it is unclear how many sessions are optimal for best results. Additional limitations of this study include the single test group, lack of tracking regarding home exercise program compliance, small sample size, the treating therapist was also the accessor, and that the study focused only on participants in the early stages of the disease (Murphy et al., 2018).

Waszczykowski et al., evaluated the effectiveness of supervised exercise therapy on hand and overall function in SSc with a one-year follow-up. This study evaluated a four-week program of supervised rehabilitation compared to a prescribed home exercise program alone. Assessments were performed at one-, three-, six-, and twelve-month follow-ups. Outcomes used included: QuickDASH, visual analog scale (VAS), Cochin Hand Function Scale (CHFS), Health Assessment Questionnaire Disability Index (HAQ-DI), Scleroderma-HAQ, hand grip, pinch grip, and range of motion. Massage in the whirlpool coupled with upper extremity active exercises, soft tissue massage, and hand joint manipulation was used with the goal of reducing pain and improving hand function. Results indicated significant positive effects for the intervention group at one-, three-, and six-month follow-ups, but only at the one-month follow-up for the control group. At the twelve-month follow-up, no significant improvement was noted indicating limited long-term effects. As a result, researchers recommend regular repetition of the program every six months at a minimum. Limitations of this study include lack of randomization, small sample size, and difficulty blinding participants and researchers (Waszczykowski et al., 2021).

Liem, et al., conducted a systematic review of the effectiveness and safety of exercise therapy in this patient population to help address the limitation of available research. The review consisted of nine articles, four randomized controlled trials and five observational studies. The review compared the interventions of hand exercises, aerobic exercise, and aerobic exercises with resistance training to no exercise. A variety of outcome measures were used to determine the effect on hand function, maximal mouth opening, quality of life, and oxygen uptake. Sample size of the different studies remained small, ranging from four to forty-eight participants with most of them females. It was determined that strengthening and mobility exercises when supported by telemedicine, were more beneficial in hand pain and functioning compared to a similar intervention using only common objects. In addition, two different studies evaluated the effect of exercise on maximal oral aperture. Interventions groups had significant improvement compared to the control groups, however, one group found that the results were short-lived and were not significant at the six-month follow-up. The last set of articles evaluated aerobic and/or resistance exercises. Both utilized a twice a week for twelve-week supervised high-intensity interval training (HIIT) program. After verifying the benefit of the base program, the researchers decided to evaluate the benefit of resistance training in combination with the established HIIT program. Resistance training consisted of chest press, bicep curl, triceps extension, lateral arm raise, and handgrip dynamometer. Results concluded the combined aerobic and resistance training improved muscle strength and function, resting heart rate, and workload and time of exercise at the ventilatory threshold as well as peak exercise were increased. A third study evaluated the effectiveness of an eight-week moderate intensity treadmill program on aerobic capacity and found similar beneficial results (Liem et al., 2019).

Overall, it is difficult to make evidence-based clinical decisions due to the lack of quality evidence. The rarity of the disease makes obtaining a suitable sample size to account for the required effect size, and difficulty blinding participants and researchers. Due to these factors, it is difficult to determine the true validity of the results. However, many of the participants had to travel more than thirty minutes for their sessions, and the adherence rate stayed high. This speaks to the feasibility of using the mentioned interventions in this population in the clinic. Unfortunately, the proposed intervention programs all varied in frequency, intensity, duration, and interventions with a highly individualized approach. In-clinic and home exercise programs were often individualized based on patients’ values and goals for treatment. Most of them also focused mainly on hand/upper extremity function without evaluating lower extremity and community/home-based mobility at all. Most of which also focused mainly on hand/upper extremity function without evaluating lower extremity and community/home-based mobility at all. Furthermore, it is challenging to determine the true effects across multiple studies because of the wide variety of outcome measures used. For example, the Functional Index for Hand OsteoArthritis was used in one study but not in others. Use of this type of outcome measure also poses its own questions with validity considering it has been validated for a different patient population than the one being examined. Even well-known and validated outcome measures, such as the QuickDASH, have a debate regarding the minimal clinically important difference score ranging from eight to fourteen. Although the evidence is weak for exercise therapy in patients with SSc, by culminating all the evidence it can be determined that exercise therapy is safe for these patients with no to minimal adverse effects. Results seem to indicate that using a combination of modalities, supervised exercise, and home exercise programs provides the most benefit and that these programs should be continued intermittently long-term because of the short-lived effects.

References

Liem, S. I., Vliet Vlieland, T. P., Schoones, J. W., & de Vries-Bouwstra, J. K. (2019). The effect and safety of exercise therapy in patients with systemic sclerosis: A systematic review. Rheumatology Advances in Practice, 3(2). https://doi.org/10.1093/rap/rkz044

Murphy, S., Barber, M., Homer, K., Dodge, C., Cutter, G., & Khanna, D. (2018). Occupational therapy treatment to improve upper extremity function in individuals with early systemic sclerosis: A pilot study. Arthritis Care & Research , 70(11), 1653–1660. https://doi.org/10.1002/acr.23522

Waszczykowski, M., Dziankowska-Bartkowiak, B., Podgórski, M., Fabiś, J., & Waszczykowska, A. (2021). Role and effectiveness of complex and supervised rehabilitation on overall and hand function in systemic sclerosis patients—one-year follow-up study. Scientific Reports, 11(15174). https://doi.org/10.1038/s41598-021-94549-y 

What is the effect of physical/exercise therapy on physical function in patients with systemic sclerosis?

               Systemic sclerosis (SSc) is a relatively rare disease of the connective tissues in the body resulting in debilitating consequences. It is characterized by diffuse fibrosis and abnormalities within the connective tissue of the skin, joints, and eventually internal organs. Specific etiology is not known. Associated signs and symptoms include pain, edema, decreased range of motion, skin thickening, and joint contractures which are all associated with decreased functional ability and reduced quality of life. Unfortunately, research regarding nonpharmaceutical interventions for systemic sclerosis are extremely limited. Research and treatment that is available, usually focuses on skin lesions and complications to internal organs as the disease progresses. However, physical therapy and/or skilled exercise therapy may be able to address functional concerns and improve quality of life in both the early and late stages of the disease.

Murphy, et al.,utilized an eight week, in-person, program consisting of tissue mobilizations, thermal modalities, upper extremity mobility, and a home exercise program in patients with early SSc (< 5 years) to determine possible benefit for upper extremity function. The study utilized a single group with pre- and post-test measures to determine improvement on the upper extremity QuickDASH, range of motion, coordination, skin thickening, and Patient-Reported Outcomes Measurement Information System (PROMIS). Patients, on average, had moderate skin disease and the majority were also being treated with immunosuppressive therapy or were participating in clinical trials. Researchers found that that there was not a meaningful improvement at four weeks into the program but did find significant improvement from baseline at the end of the eight-week period. On average, participants improved QuickDASH measures by 14 points, PROMIS by 3.1 points, 41.5 degrees of active motion, and were faster in completing the 9-hole peg test with the left hand. All are significant findings from baseline. However, no significant improvement was shown with right hand coordination or active motion. Long-term effects were not evaluated, and it is unclear how many sessions are optimal for best results. Additional limitations of this study include the single test group, lack of tracking regarding home exercise program compliance, small sample size, the treating therapist was also the accessor, and that the study focused only on participants in the early stages of the disease (Murphy et al., 2018).

Waszczykowski et al., evaluated the effectiveness of supervised exercise therapy on hand and overall function in SSc with a one-year follow-up. This study evaluated a four-week program of supervised rehabilitation compared to a prescribed home exercise program alone. Assessments were performed at one-, three-, six-, and twelve-month follow-ups. Outcomes used included: QuickDASH, visual analog scale (VAS), Cochin Hand Function Scale (CHFS), Health Assessment Questionnaire Disability Index (HAQ-DI), Scleroderma-HAQ, hand grip, pinch grip, and range of motion. Massage in the whirlpool coupled with upper extremity active exercises, soft tissue massage, hand joint manipulation was used with the goal of reducing pain and improving hand function. Results indicated significant  positive effects for the intervention group at one-, three-, and six-month follow-ups, but only at the one-month follow-up for the control group. At the twelve-month follow-up, no significant improvement was noted indicating limited long-term effects. As a result, researchers recommend regular repetition of the program at every six-months at minimum. Limitations of this study include lack of randomization, small sample size, and difficulty blinding participates and researchers (Waszczykowski et al., 2021).

Liem, et al., conducted a systematic review of the effectiveness and safety of exercise therapy in this patient population to help address the limitation of available research. The review consisted of nine articles, four randomized controlled trials and five observational studies. The review compared the interventions of hand exercises, aerobic exercise, and aerobic exercises with resistance training to no exercise. A variety of outcome measures were used to determine the effect on hand function, maximal mouth opening, quality of life, and oxygen uptake. Sample size of the different studies remained small, ranging from four to forty-eight participants with most of them females. It was determined that strengthening and mobility exercises, when supported by telemedicine, was more beneficial in hand pain and functioning compared to a similar intervention using only common objects. In addition, two different studies evaluated the effect of exercise on maximal oral aperture. Interventions groups had significant improvement compared to the control groups, however, one group found that the results were short lived and were not significant at the six-month follow-up. The last set of articles evaluated aerobic and/or resistance exercises. Both utilized a twice a week for twelve-weeks supervised high-intensity interval training (HIIT) program. After verifying a benefit from the base program, the researchers decided to evaluate the benefit of resistance training in combination with the established HIIT program. Resistance training consisted of chest press, bicep curl, triceps extension, lateral arm raise, and handgrip dynamometer. Results concluded the combined aerobic and resistance training improved muscle strength and function, resting heart rate, and workload and time of exercise at ventilatory threshold as well as peak exercise were increased. A third study evaluated the effectiveness of an eight-week moderate intensity treadmill program on aerobic capacity and found similar beneficial results (Liem et al., 2019).

Overall, it is difficult to make evidence-based clinical decisions due to the lack of quality evidence. The rarity of the disease makes obtaining a suitable sample size to account for the required effect size, and difficulty blinding participants and researchers. Due to these factors, it is difficult to determine the true validity of the results. However, many of the participants had to travel more than thirty minutes for their sessions, and the adherence rate stayed high. This speaks to the feasibility of using the mentioned interventions in this population in the clinic. Unfortunately, the proposed intervention programs all varied in frequency, intensity, duration, and interventions with a highly individualized approach. In-clinic and home exercise programs were often individualized based on patients’ values and goals for treatment. Most of which also focused mainly on hand/upper extremity function without evaluating lower extremity and community/home-based mobility at all. Furthermore, it is challenging to determine the true effects across multiple studies because of the wide variety of outcome measures used. For example, the Functional Index for Hand OsteoArthritis was used in one study but in no others. Use of this type of outcome measure also poses its own questions with validity considering it has been validated for a different patient population than the one being examined. Even well-known and validated outcome measures, such as the QuickDASH, have debate regarding the minimal clinically important difference score ranging from eight to fourteen. Although the evidence is weak for exercise therapy in patients with SSc, by culminating all the evidence it can be determined that exercise therapy is safe for these patients with none to minimal adverse effects. Results seem to indicate that using a combination of modalities, supervised exercise, and home exercise program provides the most benefit, and that these programs should be continued intermittently long-term because of the short-lived effects.

  References

Liem, S. I., Vliet Vlieland, T. P., Schoones, J. W., & de Vries-Bouwstra, J. K. (2019). The effect and safety of exercise therapy in patients with systemic sclerosis: A systematic review. Rheumatology Advances in Practice, 3(2). https://doi.org/10.1093/rap/rkz044

Murphy, S., Barber, M., Homer, K., Dodge, C., Cutter, G., & Khanna, D. (2018). Occupational therapy treatment to improve upper extremity function in individuals with early systemic sclerosis: A pilot study. Arthritis Care & Research , 70(11), 1653–1660. https://doi.org/10.1002/acr.23522

Waszczykowski, M., Dziankowska-Bartkowiak, B., Podgórski, M., Fabiś, J., & Waszczykowska, A. (2021). Role and effectiveness of complex and supervised rehabilitation on overall and hand function in systemic sclerosis patients—one-year follow-up study. Scientific Reports, 11(15174). https://doi.org/10.1038/s41598-021-94549-y

    What is the effect of physical/exercise therapy on physical function in patients with systemic sclerosis?

Systemic sclerosis (SSc) is a relatively rare disease of the connective tissues in the body resulting in debilitating consequences. It is characterized by diffuse fibrosis and abnormalities within the connective tissue of the skin, joints, and eventually internal organs. Specific etiology is not known. Associated signs and symptoms include pain, edema, decreased range of motion, skin thickening, and joint contractures which are all associated with decreased functional ability and reduced quality of life. Unfortunately, research regarding nonpharmaceutical interventions for systemic sclerosis is extremely limited. Research and treatment that is available usually focus on skin lesions and complications to internal organs as the disease progresses. However, physical therapy and/or skilled exercise therapy may be able to address functional concerns and improve the quality of life in both the early and late stages of the disease.

Murphy, et al., utilized an eight-week, in-person, program consisting of tissue mobilizations, thermal modalities, upper extremity mobility, and a home exercise program in patients with early SSc (< 5 years) to determine possible benefits for upper extremity function. The study utilized a single group with pre- and post-test measures to determine improvement in the upper extremity QuickDASH, range of motion, coordination, skin thickening, and Patient-Reported Outcomes Measurement Information System (PROMIS). Patients, on average, had a moderate skin disease and the majority were also being treated with immunosuppressive therapy or were participating in clinical trials. Researchers found that there was not a meaningful improvement at four weeks into the program but did find significant improvement from baseline at the end of the eight weeks. On average, participants improved QuickDASH measures by 14 points, PROMIS by 3.1 points, 41.5 degrees of active motion, and were faster in completing the 9-hole peg test with the left hand. All are significant findings from baseline. However, no significant improvement was shown with right-hand coordination or active motion. Long-term effects were not evaluated, and it is unclear how many sessions are optimal for best results. Additional limitations of this study include the single test group, lack of tracking regarding home exercise program compliance, small sample size, the treating therapist was also the accessor, and that the study focused only on participants in the early stages of the disease (Murphy et al., 2018).

Waszczykowski et al., evaluated the effectiveness of supervised exercise therapy on hand and overall function in SSc with a one-year follow-up. This study evaluated a four-week program of supervised rehabilitation compared to a prescribed home exercise program alone. Assessments were performed at one-, three-, six-, and twelve-month follow-ups. Outcomes used included: QuickDASH, visual analog scale (VAS), Cochin Hand Function Scale (CHFS), Health Assessment Questionnaire Disability Index (HAQ-DI), Scleroderma-HAQ, hand grip, pinch grip, and range of motion. Massage in the whirlpool coupled with upper extremity active exercises, soft tissue massage, and hand joint manipulation was used with the goal of reducing pain and improving hand function. Results indicated significant positive effects for the intervention group at one-, three-, and six-month follow-ups, but only at the one-month follow-up for the control group. At the twelve-month follow-up, no significant improvement was noted indicating limited long-term effects. As a result, researchers recommend regular repetition of the program every six months at a minimum. Limitations of this study include lack of randomization, small sample size, and difficulty blinding participants and researchers (Waszczykowski et al., 2021).

Liem, et al., conducted a systematic review of the effectiveness and safety of exercise therapy in this patient population to help address the limitation of available research. The review consisted of nine articles, four randomized controlled trials and five observational studies. The review compared the interventions of hand exercises, aerobic exercise, and aerobic exercises with resistance training to no exercise. A variety of outcome measures were used to determine the effect on hand function, maximal mouth opening, quality of life, and oxygen uptake. Sample size of the different studies remained small, ranging from four to forty-eight participants with most of them females. It was determined that strengthening and mobility exercises when supported by telemedicine, were more beneficial in hand pain and functioning compared to a similar intervention using only common objects. In addition, two different studies evaluated the effect of exercise on maximal oral aperture. Interventions groups had significant improvement compared to the control groups, however, one group found that the results were short-lived and were not significant at the six-month follow-up. The last set of articles evaluated aerobic and/or resistance exercises. Both utilized a twice a week for twelve-week supervised high-intensity interval training (HIIT) program. After verifying the benefit of the base program, the researchers decided to evaluate the benefit of resistance training in combination with the established HIIT program. Resistance training consisted of chest press, bicep curl, triceps extension, lateral arm raise, and handgrip dynamometer. Results concluded the combined aerobic and resistance training improved muscle strength and function, resting heart rate, and workload and time of exercise at the ventilatory threshold as well as peak exercise were increased. A third study evaluated the effectiveness of an eight-week moderate intensity treadmill program on aerobic capacity and found similar beneficial results (Liem et al., 2019).

Overall, it is difficult to make evidence-based clinical decisions due to the lack of quality evidence. The rarity of the disease makes obtaining a suitable sample size to account for the required effect size, and difficulty blinding participants and researchers. Due to these factors, it is difficult to determine the true validity of the results. However, many of the participants had to travel more than thirty minutes for their sessions, and the adherence rate stayed high. This speaks to the feasibility of using the mentioned interventions in this population in the clinic. Unfortunately, the proposed intervention programs all varied in frequency, intensity, duration, and interventions with a highly individualized approach. In-clinic and home exercise programs were often individualized based on patients’ values and goals for treatment. Most of them also focused mainly on hand/upper extremity function without evaluating lower extremity and community/home-based mobility at all. Most of which also focused mainly on hand/upper extremity function without evaluating lower extremity and community/home-based mobility at all. Furthermore, it is challenging to determine the true effects across multiple studies because of the wide variety of outcome measures used. For example, the Functional Index for Hand OsteoArthritis was used in one study but not in others. Use of this type of outcome measure also poses its own questions with validity considering it has been validated for a different patient population than the one being examined. Even well-known and validated outcome measures, such as the QuickDASH, have a debate regarding the minimal clinically important difference score ranging from eight to fourteen. Although the evidence is weak for exercise therapy in patients with SSc, by culminating all the evidence it can be determined that exercise therapy is safe for these patients with no to minimal adverse effects. Results seem to indicate that using a combination of modalities, supervised exercise, and home exercise programs provides the most benefit and that these programs should be continued intermittently long-term because of the short-lived effects.

[noreenmadelineruns-blog]

Running with Chronic Pain

It was thrilling how little time it took my feet to fly when they hit the trailhead. The ground beneath me felt more like a spring board catapulting me forward than a slippery, challenging opponent. Mud season, as it's call in the Northeast, had arrived. That glorious time of year when two feet of snow melted off the mountains, depositing oozing, brown mounds of wet dirt on the trails. The air was warm and the sun stung my pale cheeks through the trees. I pushed up on the center of my sister's Oakley sunglasses I had stolen from her car that morning. Sweat was pouring from my temples as I elongated my stride. I smiled.

My heart was so free. Right there, in that moment, I was exactly where I needed to be . This joy that built up in the center of my chest was too difficult to contain and I chuckled to myself as I broke out of the trees and headed to my apartment. Bounding up the steps, I tore off my T-shirt, pushed through the front door, and tripped over the pile of shoes I had apparently left as a trap for myself. Running had always been my safe place. The place I would go to if I had a bad day or a good day. It had never been about the distance run, the time achieved, or the calories burned. It was always about how it made me feel. It made me feel strong. It took me to beautiful places, and brought me closer to good friends. It taught me patience. It taught me that the best medicine was the scent of decaying leaves in the fall. On this particular day, it was everything I needed. 

Gripping my soaking wet, mud-covered shirt in one hand I noticed something, I couldn't fully close my fingers. I shrugged it off as a side effect of a challenging workout and stripped down for a shower. 

Over the coming weeks, however, the stiffness and swelling in my fingers and hands spread to my elbows, knees, jaw, and feet. My skin stung as if I had gotten a bad sunburn and my muscles ached. I was unable to sit up or walk without pain. I began experiencing overwhelming fatigue. I left the house every morning for work only to duck out early and hop in bed. My hair fell out. I lost about 15lbs, which, on my lean frame, was particularly noticeable. I left my job as a laboratory technician and move back home temporarily where my family could care for me. I could no longer deny that something wasn't right and I made an appointment with my primary care physician, who referred me to a rheumatologist. 

I was able to see a rheumatologist four months after my initial symptoms appeared, a rather long wait when one is experiencing persistent pain. During my initial visit I extended my swollen fingers to my doctor who mumbled, "Hm, that looks like scleroderma." For those of you who are unfamiliar with autoimmune diseases, systemic sclerosis (SSc) is a chronic condition causing fibrosis of the skin and internal organs. SSc patients with the diffuse form of the disease typically die within 10 years of diagnosis. I paused, as a young scientist studying autoimmune diseases I understood better than most patients the gravity of my physicians observation. 

The next few months brought with it bi-weekly visits to my physicians, pharmacist, pulmonary technicians, and  X-ray technicians. After hundreds of dollars of testing later, my physicians diagnosed me with polyarticular psoriatic arthritis (PsA) and Hashimoto's thyroiditis. My PsA is categorized as severe, meaning more than four joints were affected. In fact, there were few joints that had not been inflamed to the point of immobility. Given the severity of my disease, my doctors prescribed me a TNF-a blocker, which come from a class of biologics indicated for patients with PsA, Rheumatoid arthritis (RA), Crohn's Disease (CD), and several other inflammatory conditions. Within five weeks of treatment initiation I felt amazing. The swelling and pain in my joints, muscles, and skin diminished to a dull ache. My energy returned. My hair grew back, albeit with a few more grays, but I'll take it. I could see my friends and family. I had had the energy to write and read books. I could cook food for myself without becoming immediately exhausted. Most importantly, I could run again. 

I pulled my long sleeve, yellow T-shirt over my head. I stole it from my sister's closet, but told myself I would wash it after my run. She would never know. I slipped into my blue and gray Brooks and jogged out the door to my favorite trail. It was late spring and the leaves on the trees swayed like waves above my head. The nearby creeks were trickling with the sound of the last patches of snow melting off the mountains. The air smelled clean and I pumped my arms as I raced up to the peak of the hill overlooking a reservoir. I stopped. I was home. My heart, my heart, my heart. I was free again. I smiled. 

[step2ckmap]

Scleroderma

Cytokines cause fibroblasts to deposit excessive amount of collagen

Diffuse (20%) vs Limited (80%)

ANA sensitive to both but not specific

Limited (CREST)--> spares the trunk, Anti-centromere antibody, better prognosis

Diffuse--> Anti-Topoisomerase I 

Lung fibrosis/HTN, GERD, Colonic diverticula, PBC (15%), Restrictive cardiomyopathy, Renal disease

Barium swallow and PFTs to detect complications

Most common cause of death is pulmonary complications

Renal- ACE inhibitors

Pulm HTN- Bosentan (endothelin antagonists)

Raynauds- CCB

GERD- PPIs

Lung Fibrosis- Cyclophosphamide

No cure, treat with NSAIDS and complications

*20% of patients with scleroderma have Sjögrens

[doctorfoxtor]

lies und schreib solang' dir's schmeckt, schon unzählbare Male sind uns're Gehirne verreckt

100 days of productivity

day 33 + 34

CVS/RS

stimulants are assoc w/ pulmonary hypertension, especially appetite suppressants such as fenfluramine

the mucociliary elevator moves at the rate of 2 cm/min

the anatomical definition of a bronchiole is a generation of airway that no longer contains cartilage in the wall with calibre of typically 1 mm

pulmonary haemosiderosis: seen in childhood, characterised by recurrent intrapulmonary bleeding (triad of haemoptysis, sudden drop in PCV → IDA, diffuse pulmonary infiltrates); can be associated w/ Goodpasture's, w/ cow milk protein allergy, w/ von Willebrand or idiopathic

CNS/Ophthal/Psych

the 15-year risk of develop MS in a patient with optic neuritis with: a. normal T2-MRI brain = 25%; b. ≥1 lesion on T2-MRI brain = 50%

Barthel index (ability to carry out ADLs) is closely related to post-stroke mortality (80-100 = independent (best), 50-80 = moderate, 25-50 = low, <25 = v low (worst))

when deciding on the cause of blurring of vision, a dull aching eye pain should raise concern for demyelination > ischaemia > compression

Rheum/Derm

the major prognostic factor for recurrence of BCC is the presence of perineural invasion, which is especially high in micronodular and morphoeic (infiltrative) subtypes

in an alcoholic with normal RBC indices and LFTs, low libido and osteoporosis should point to (nonalcoholic) hypogonadism as a cause

ID/Immuno

brucellosis can cause an AnkSpon like sacroiliitis/spondylosis but it usually causes a multitude of nonspecific ssx (including lymphoma like adenopathy + hepsplen)

EBV latency specifically occurs in B-cells; this way it avoids immune surveillance

the one thing i hate the MOST in ID is rickettsial infections fuck them seriously

hyper IgD syndrome is characterised by periodic fevers

Onc/Haem

PNH can devolve into an aplastic anaemia; suspect this in pts w/ h/o anaemia + thrombosis who suddenly have pancytopaenia

the major difference between myelofibrosis and CML (which can present clinically identically) is the WBC count, which is often markedly elevated in CML and typically normal to low in myelofibrosis; also a peripheral smear shows WBC precursors only in myelofibrosis (and can show basophilia in CML)

Renal/Biochem

calcium is of no use when hyperkalaemia has already resulted in vtach/vfib (the purpose of calcium is to prevent vtach/vfib); at this point it is better to go for electricity and then give calcium as secondary prophylaxis if and when the rhythm has converted and a modicum of haemodynamic stability is recovered

hepatorenal syndrome is typically NOT associated with proteinuria despite glomerular damage

ACE-is/ARBs have flipped mortality statistics in scleroderma; now, CKD is less mortal than lung fibrosis/PAHTN

Pharm/Toxo

gold (not given for RA anymore) ADRs: skin, lips, kidney damage (as both nephrosis and nephritis) and blood dyscrasias

acyclovir is specifically converted by viral thymidine kinase to Acy-monophosphate and then by host thymidylate kinases to Acy-triphosphate, which is the active form

the neurotoxic effects of acyclovir are mediated by 9-carboxy-methoxy-methyl-guanine

TEN is associated with hyperthermia and also VTEs (due to rapid fluid contraction → ↑viscosity)

[allergy-by-health-omni]

An Introduction to Scleroderma Allergic Reactions

Scleroderma Allergic Reactions

Scleroderma is a chronic and rare disease that lasts for a long time. It induces skin thickening, hardening and tightness and may also involve the internal organs of the body and in turn, lead to intestinal, heart, kidney and lung problems. Localized Scleroderma is involved only in the skin while generalized Scleroderma or systemic sclerosis allergic reactions involve internal organ involvement.

The most common skin Scleroderma allergic reactions are swelling of hands and toes, generalized itching, thickening, hardening and tightness of skin, change of the color of the hands and feet with cold exposure and sores over fingertips. The other Scleroderma allergic reactions include fatigue, heartburn, shortness of breath, arthritis, dryness of the eyes and mouth, cough and high blood pressure.

Scleroderma is basically a rare disease that occurs in 10 – 20 people amongst a million people in a year. Though people of all races can get Scleroderma, about 75% of the victims are women. Scleroderma can occur at any age but is most common in people between the ages of 30 and 60 years old. The exact causes of Scleroderma are not known; however it is known to be an autoimmune disease. This is where the body’s natural immune system does not actually behave normally; instead of fighting off infections from bacteria and virus, the immune system of a victim of Scleroderma attacks its own body.

With this, there is usually some damage to the blood vessels of the body. And in response to this blood vessel damage, specific proteins called collagen are produced to repair the damage to blood vessels through fibrosis or the formation of diffuse scars. There is usually an excess deposit of collagen in the skin that induces the skin changes associated with Scleroderma. The fibrosis of the blood vessels usually leads to the derangement of internal organs.

The diagnosis of Scleroderma allergic reactions is rather difficult, and can be done by specialists in autoimmune diseases. This diagnosis is based on a thorough history and physical examination of the victim along with some laboratory studies. Not only are blood and urine tests conducted, specialized lung and heart evaluations are required to evaluate the involvement of internal organs in Scleroderma.

With the diagnosis of Scleroderma, treatment can be started. There is basically no cure for Scleroderma as it is a chronic disease that requires medical therapy for many years. Usually organ specific treatments are used of treating Scleroderma with combinations of anti-inflammatory and immunosuppressant medicine. Sometimes other medication is used to control skin and internal organ problems and at times, chemotherapy may be used to control lung problems.

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[underfag]

Autoimmune Illness as well as Your Skin (Part 3)

Below at Advanced Skin Treatment as well as Dermatology Physicians, I see autoimmune conditions often. The reason results from traditional searchings for or signs on the skin. As I have actually noted before, this is very essential to acknowledge because, these indications might likewise point to other troubles within the body.

In my first blog post about autoimmune illness, I talked about lupus as well as noted some of the various other diseases that may appear with skin findings. In the 2nd post, I dealt with 2 of one of the most typical autoimmune illness I see as a skin specialist: alopecia location and vitiligo. In this third message, I will talk about two really important autoimmune diseases that have unique skin adjustments.

Scleroderma

Scleroderma essentially implies "difficult skin." For reasons we do not comprehend, the body immune system occupies a war versus the skin or inner body organs that brings about unusual down payments of collagen, a.k.a., scleroderma. It can be a really major illness if advanced..

There are 2 major kinds of scleroderma: localized scleroderma and systemic scleroderma. Localized scleroderma is called morphea and appears on the skin as tough, waxy-looking areas. Usually, there is a violet border around it that represents it is energetic. However, this type of scleroderma is not connected with organ issues. Systemic scleroderma, on the various other hand, indicates that the illness has the potential to "solidify" various other body organ systems. Within this type, there is a "restricted" and "scattered" form. The restricted type is called "CREST disorder," which means:.

C: Calcinosis is when alcium down payments occur in the skin. R: Raynaud's: This happens when the blood vessels on the fingers as well as toes spontaneously restrict in feedback to also minor changes in temperature or emotional stress. The skin turns white, after that blue and then red. In some cases these episodes are so severe that they can bring about abscess or irreversible death of a few of the skin (gangrene). E: Esophageal dysmotility is when individuals may have difficulty ingesting or absorbing food. S: Sclerodactyly: Over time, because of solidifying of the skin-- generally on the hands as well as feet, the fingers and also toes agreement as well as come to be tough to use. T: Telangiectasias: Broken capillaries cover the skin with time. I see these at all times in people however, in scleroderma, they happen in collections..

" Diffuse" systemic sclerosis is various than CREST disorder and can have destructive impacts on a wide range of organs consisting of the heart, lungs, kidneys and digestion system.

Dermatomyositis.

Dermatomyositis is an inflammatory autoimmune condition that can affect either youngsters or grownups. In many cases, it causes severe muscle inflammation as well as muscle mass weakness. This is often gone along with by a rash that is really classic-looking as well as shows up around the eyes, hands, breast and thighs. It is a lot more obvious in areas that obtain sun as well as has a traditional violet-looking shade.

An unusual condition connected with this is proximal muscle mass weak point, indicating weakness in the shoulders and also hips. Though primarily unusual, I do see it on a relatively routine basis. A member of the family of mine has it, which is among the reasons I am so curious about this illness. Kids obtain a particular kind of dermatomyositis that can result in unpleasant calcifications in the muscular tissues that can be very incapacitating. It affects adults in a different way that can affect various other organs in the body too. The primary concern in grownups with this disease is if they likewise have cancer cells. Somehow, this autoimmune condition can be an indicator of an underlying cancer, so I always make sure individuals are up-to-date on their cancer testings. Nevertheless, youngsters that obtain this condition do not have a boosted risk of cancer.

Have you had any type of experiences with these autoimmune diseases? We would certainly like to hear your tale in the remarks

.

Dr. Hayes- Board Qualified Skin Specialist

.

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[drjatinderjuneja]

Systemic Sclerosis: Symptoms, Diagnosis, and Treatment

The systemic sclerosis also known as scleroderma, is a chronic autoimmune disease that primarily affects the skin.

Systemic sclerosis or scleroderma, in addition to affecting the skin, can affect the internal organs (lungs, intestines, kidney or heart). Skin changes are caused by the increase and accumulation of collagen fibers and other proteins. The skin is sclerous (it becomes hard and rigid).

At first, the injuries consist of an inflammation, which later turns into a hardening.

Systemic sclerosis also hinders blood supply by occluding the small arteries and capillaries that carry blood to the tissues, explains the best rheumatologist in Delhi.

What are the usual symptoms?

The most visible effects are skin lesions. It usually begins with a slight swelling of the skin of the hands and feet, sometimes also of the face, which is spreading. Subsequently, the skin becomes stiff and hard, becomes difficult to pinch, and sometimes limits joint movements. 

However, in those with whole-body involvement, scars and thickening extend to the underlying structures (fat, muscle, and rarely bone). There are usually changes in the color of the hands that, when exposed to the cold, become excessively pale and later purplish, sometimes accompanied by pain or tingling. These symptoms are called Raynaud's phenomenon. Poor circulation can cause serious damage, such as ulcers on the fingers or gangrene.

There may be dilated blood vessels on the face, hands, nail folds, and other places. Some patients develop calcium deposits (calcinosis) on the skin and other organs such as the lungs, muscles, and kidneys.

Systemic sclerosis can also lead to joint pain, fatigue, digestive problems, such as difficulty swallowing, heartburn, constipation or diarrhea, and cardiorespiratory problems such as respiratory distress, hypertension, or chest pain, states the top rheumatologist in Delhi.

The most common symptoms are:

Morphea.

Rigid and hard skin.

Joint pain

Raynaud's phenomenon.

How is it diagnosed?

The diagnosis is made through a specialized medical examination by the rheumatologist in Delhi, which will include a report of the symptoms, a skin examination and a general examination.

Complementary examinations are always performed such as analysis and a chest x-ray, and only in some cases other digestive or cardiorespiratory tests.

The purpose of these tests is to better classify the type of disease and assess its severity and extent.

How do we treat it?

No drug has been shown to be effective in 100% of patients with systemic sclerosis, although there are partial responses to immunosuppressants in cases of systemic involvement.

Treatment of the inflammatory stage of the skin lesion usually requires the administration of corticosteroids. 

Pulmonary interstitial involvement may improve, initially, with oral cyclophosphamide / mycophenolate mofetil and steroids.

In the case of pulmonary hypertension, calcium blockers, prostacyclin, or the like can be used.

Gastrointestinal involvement improves with anti-reflux postural measures, H2 blockers, prokinetics, and erythromycin. Broad spectrum antibiotics can be used for malabsorption.

Calcium blockers together with antiplatelet agents are effective in Raynaud's phenomenon.

Regular monitoring of blood pressure is essential in patients with diffuse sclerosis. Treatment should be started when blood pressure is above 140/90 mmHg. Cases of marked calcinosis may require microsurgery.

For the treatment of muscle involvement, the use of corticosteroids is recommended in vigorous guidelines.

The treatment of arthralgias, arthritis or tenosynovitis requires the use of non-steroidal anti-inflammatory drugs (NSAIDs) and rest. The use of local infiltrations can be very useful.