Autoimmune disease presented as complicated c/o of PSS with myositis a/w hypothyroidism In my good old days this was described as progressive sclerosis scleroderma Principally this was covered by dermatologists as research advanced and seen it is is affecting the more than one system hence find the new nomenclature Pt usually reports with weakness tiredness loss of appetite infact dysphagia with stiffness in skin and joint pains Yes paleness anaemia stiff TM joint progressive thickening of skin in different parts of body with submucosal thickening of oesophagus developing dysphagia and ROM of TM joints and polyartheralgia It can effect valves as MS or MR may be coexisting In this case it is hypothyroidism a/w Usually ladies are predominantly affected Ix are now enumerous to do dx we were just more clinically approaching Now ANA crp ck and ckmb etc are helpful Treatment wise i see still we are confined to steroid and hcqs beside supplimentary prescription It goes life long and except to improve anaemia or symptomatic treatment and marginal improvement of quality of life nothing more we could achieve. This needs dedicated research work
1.ScL70 -ve ,centromere positive findings suggest that the disease is limited to skin only. 2 Abssense of any of the symptoms of systemic sclerosis also suggests that the disease is lited to skin only. 3. So more appropriately the case,at this stage may be called Sclerderma. 4. ANA +ve suggests auto immune disease including scleroderma / SS etc. 5. The myositis overlap is also doubtful because none of the features of the 2 types of myositis ie polymyositis (which is bilateral disease )and dermatomyositis (age is against an heliottopic rash is abscent ) the features are present. 6. The myositis is brought into diagnosis because of raised creatine kinase and girdle muscles weakness. Scleroderma ,by tightening of skin over the muscles may limit the movements associated with the muscles involved. Muscle enzyme may be raised in scleroderma . Biopsy is necessary for proving myositis and scleroderma . Further systemic investigations are needed to label it as systemic sclerosis. Though scleroderma and SS are talked as synonyms,it is better to call cases with skin involvement only as scleroderma. There is no description of thickening,titightening of skin and contracture of fingers due to destructive fibrosis. The history of no difficulty in putting on buttons might indicate that there is no typical hand lesions and no difficulty in opening mouth and absent mask like. :(expressionless face) . Could be possible that it is an early case of scleroderma. Limb girdle type myopathy should also br kept in mind as the pt's symotoms(difficulty in climbing the stairs) and age suggest. Biopsy clears all doubts.
Good case some points to be noted The diagnosis of scleroderma myositis overlap syndrome is not correct, its not fullfill myositis criteria. other DD 1. MCTD 2. SCLERDODERMA ASSOCIATED MYOPATHY raised CPK with mild proximal weakness can be seen in hypothyroidism mild proximal wekness can be seen in low Vitamin D please do a ANA pannel some findings to searched thoroughly like acrosclerosis, raynauds, pitted scar in finger tips history should be more detailed ECHO TO R/O PAH In my opnion, this case is limited scleroderma with NO ILD RAISED CPK AND MUSCLE WEAKNESS CAN BE DUE TO HYPOTHYROIDISM AND LOW VITAMIN D OR SCLERDERMA ASSOCIATED MYOPATHY.
SYSTEMIC SCLEROSIS WITH MUCOSITIS OVERLAP SYNDROME. AUTOIMMUNE DISEASE. NICELY DESCRIBED FROM INTRODUCTION ,CASE PROFILE AND HISTORY IN DETAILED FOLLOWED BY PHYSICAL AND SYSTEMIC EXAMINATION , INVESTIGATION TO CONCLUDE THAN DIFFERENTIAL DIAGNOSIS,MANAGEMENT AND REGULAR FOLLOW UP AND DISCUSSION KEY POINTS LEARNING AND CHALLENGES IN COMPRESSIVE WITH DETAILED ILLUSTRATION. treatment and physiotherapy are life long and periodcal assessment of complications follow up. Treatment is symptomatic and reassurance and councelling required to improve the quality of life . Steroids ,HCQ and correction of anaemia and physiotherapy are mainstay of treatment. A long period has elapsed to be changed the treatment it needs serious studies and elaborate experimental and dedicated team to find some medicines to cure or a pt leads a good quality life without any problems.
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MCTD is due to Autoimmunity to components of the U1-70 kd snRNP is a hallmark of disease. Anti-RNP antibodies can precede overt clinical manifestations of MCTD, but overt disease generally develops within 1 year of anti-RNP antibody induction. In an young girl,the diagnosis of Scleroderma is more generous than SS or MCTD. .The former diagnosis can be reserved if systemic features develop and the later if ani RNP Aantibodies are detected. Biopsy is necessary. Till then the case can not be treated as closed .
SUGGESTIVE. OF SYSTEMIC SCLEROSIS. MYOSITIS OVERLAP. SYNDROME NICELY ILLUSTRATED. SIR VERY. USEFUL INDEED
Congratulations Doctor Dr. @Dr. Ashutosh Chandan Dubey ( Dr Strange) A clinical case report on Systemic sclerosis with Myositis overlap syndrome Systemic sclerosis is unique among other rheumatic diseases due to challenge of managing a Chronic multisystem autoimmune disease The treatment is Hollistic yet focused on dominant organ disease. Thank you Doctor!
Nice information useful information updated.. Sclerosis with Myositis Overlap Syndrome.. management.. Thanks doctor
Excellent presentation Dr.Herbal(Alovera for pigmentation of skin)+tt&follow up of Hypothyroidism+rule out Hypo parathyroid state-renal&Hepatic abnormalities '+Comprehensive alternate medical management-
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SYSTEMIC SCLEROSIS. Systemic sclerosis (ssc) /Scleroderma is a rare chronic disease of unknown etiology characterised by diffuse fibrosis. Degenerative changes and Vascular abnormalities in the skin ,joints and internal organs. COMMON SYMPTOMS. Raynaud's phenomenon. Polyarthritis. Dysphagia. Heartburn. Swelling of fingers. Skin tightening and contracture of fingers. Lung ,kidney and heart involved. ETIOLOGY. Heridity and immunological mechanisms play a role in etiology. Scleroderma like syndrome can also result from exposure to vinyl chloride ,bleomycin ,pentazocine , epoxy ,aromatic hydrocarbons ,contaminated rapeseed oil or L -tryptophan. PATHOPHYSIOLOGY Pathophysiology involves vascular damage and activation of fibroblasts. Changes in the skin. *skin develops compact collagen fibers in the reticular dermis, *epidermal thinning. *loss of rete pegs. *atrophy of dermal appendages. *accumulation of T cells. *extensive fibrosis of the subcutaneous and dermal layers. *capillary loops are dilated and microvascular loops are lost kn the nail bed. *chronic inflammation and fibrosis of synovial membranes. Changes in the GIT. *esophageal motility is impaired. *lower esophageal sphincter becomes incompetent. *secondary strictures develop. *intestinal muscularis mucosa degenerates. leading to pseudo diverticula in thecolon and ileum. Changes in respiratory tract. *interstitial and peri bronchiolar fibrosis. *intimal hyperplasia of small pulmonary atteries. *pulmonary hypertension. Changes in heart. *diffuse myocardial fibrosis. *cardiac conduction abnormalities. There are Two types of systemic sclerosis. 1) LIMITED CUTANEOUS SYSTEMIC SCLEROSIS / LIMITED SCLERODERMA. Thid is also known as CREST SYNDROME Calcinosis. Raynaud's phenomenon. Esophageal dysmotility. Sclerodactyly. Telangiectasia. 2)DIFFUSE CUTANEOUS SYSTEMIC SCLEROSIS. OR DIFFUSE SCLERODERMA. SYMPTOMS AND SOGNS. The most common symptoms are Raynaud's phenomenon and insidious swelling of distal exyremities. SKIN AND NAIL MANIFESTATIONS *Symmetric swelling of the skin which progresses to induration. *Sclerodactyly. *salt and pepper appearance of skin. *subcutaneous calcifications. *digital ulcers. *skin becomes shiny ,taut ,hypopigmented or hyperpigment JOINT MANIFESTATIONS. *Polyarthralgia. *flexion contractures . GI MANIFESTATIONS. *Esophageal dysfunction. *Retro sternal Dysphagia. *diverticula in the colon. CARDIO PULMONARY MANIFESTATIONS. *Lung fibrosis. Acute alveolotis. Pericarditis with pleural effusion. *Cardiac arrhythmias. RENAL MANIFESTATIONS. Renal disease. DIAGNOSIS *Clinical evaluation Usually ANTI NUCLEAR ANTIBODIES (ANA ), Scl.-70 (topoisomerase. Anti centromere antibodies. PROGNOSIS. Slow progression of diseasr. 10 yr survival rate is 65 % TREATMENT. Treatment is directed at symptoms and dysfunctional organs. KEY POINTS. Pathologic changes include skin and joint changes. Raynaud's phenomenon. Esophageal dysfunction. Involvement of lung,heart and kidneys. Consider the diagnosis in Raynaud's phenomenon. Typical musculoskeletal or skin manifestations. Unexplained Dysphagia. Malabsorption. Pulmonary fibrosis. Pulmonary hypertension. Cardiomyopathies. Conduction disturbances. Test for ANA ,Scl-70 (topoisomerase), and anti centromere antibodies. Because there is no clear disease modifying therapy ,treatment is directed at the involved organs.Dr. Suvarchala Pratap17 Likes11 Answers
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ABC OF : SYSTEMIC SCLEROSIS. ( II ). ( Scleroderma ). MAY BE USEFUL. ** CORTICOSTEROIDS (in inflammatory myositis, pericarditis, refractory arthritis, or alveolitis) METHOTREXATE (15 mg/wk) CHLORAMBUCIL. MYCOPHENOLATE MOFETIL may benefit patients with diffuse progressive cutaneous systemic sclerosis of recent onset, a small prospective study suggested. Myositis may be treated cautiously with steroids (first choice), or with methotrexate or azathioprine in corticosteroid-resistant cases or when there are contraindications to corticosteroid use. Doses of prednisone greater than 40 mg/d are associated with a higher incidence of SCLERODERMA RENAL CRISIS. ** Research indicates that there is a susceptibility gene, which raises the likelihood of getting scleroderma, but by itself does not cause the disease. CURRENTLY, THERE IS NO CURE FOR SCLERODERMA, BUT THERE ARE MANY TREATMENTS AVAILABLE TO HELP PARTICULAR SYMPTOMS. ** Lung problems are usually the most serious complications of systemic scleroderma. They are now the leading cause of death in scleroderma patients. Two major lung conditions associated with scleroderma, pulmonary fibrosis and pulmonary hypertension, can occur either together or independently. ** Immunosuppressants fall under a category of drugs referred to as DMARDs (Disease-Modifying Anti-Rheumatic Drugs). IMMUNOSUPPRESSANTS commonly used for scleroderma and rheumatic diseases include METHOTREXATE, AZATHIOPRINE, CYCLOSPORINE, MYCOPHENOLATE MOFETIL and CYCLOPHOSPHAMIDE....... ** Calcium channel blockers are reported to help blood flow to the skin and heart; angiotensin converting enzyme inhibitors (ACE) INHIBITORS reverse the vasospasm of the scleroderma renal crisis; and BOSENTAN (a new endothelin-1 receptor inhibitor) or EPOPROSTENOL (prostacyclin) improve blood flow in the....... *** Slide 9 : HRCT.......Dr. Puranjoy Saha16 Likes11 Answers