A case of Systemic Sclerosis with Myositis Overlap Syndrome

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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

Thanx dr Md Altaf Hussain
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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.

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. Scleroderma is a more generous diagnosis for an young girl ,than SS or MCTD ,in my opinion.
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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.

Thanks Dr Dinesh Gupta
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Congratulations! Your case has been selected as Case of the day and you have been awarded 5 points for sharing the case. Keep posting your interesting cases, Happy Curofying!

It be a peculiar r/a. Ms diseases mostly lake of neutriontion and vitamins like calcium. V7. 6.12.vD3.lose of body. So our opnion as a Naturopath ayurved if u take v7. 6,12,calcium d3.. Will be relaxe spastic muscle.... U shoul take ayurved medecian like neuroplex. Neuromarma sy. Haratica. Macunna. T/d.
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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

DR.@ ASHUTOSH CHANDAN. DUBEY. JI THANKS. SIR
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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

Thanks doctor Dinesh Gupta
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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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