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RISS 인기검색어
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- 저자 : ( Mridulata Prajapati ) (검색결과 1 건)
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( Girish Ramteke ),( Sanjay Dubey ),( Neeraj Dayani ),( Mridulata Prajapati ),( Paridhi Shivde ) 대한내과학회 2014 대한내과학회 추계학술대회 Vol.2014 No.1
Introduction: Disorders of collagen tissue are caused by humoral and cellular mediated immune responses mediated by cytokines like TGF ß, TNF a etc. The various disease manifestations include mixed connective tissue diseases, rheumatoid arthritis, sjogren`s syndrome, systemic sclerosis etc. One of the associations is that of cardiac valvesstenotic lesion-though extremely rare. We report one such rare case. Case presentation: 60 year old female presented with a history of tightness of skin, contractures of fingers, inability to open mouth and pitting scars over the tip of digits. There was no history of breathlessness. There is a history of rheumatic heart disease-mitral stenosis-open mitral valvulotomy 7 years ago. On evaluation she had features suggestive of Mauskopf`s facies, microstomia and other features of systemic sclerosis and the ACR criteria suggested systemic sclerosis. Modified Rodnan skin thickness score was 43 out of 51. Her Antiscl-70 antibody was positive and skin biopsy showed dermal sclerosis. HRCT chest showed- septal thickening with ground glass appearance. Though the patient was asymptomatic her echo revealed chordal shortening, cuspal retraction which was an additional feature compared to earlier echo 7 years ago. This association of valvular involvement in systemic sclerosis is an extremely rare association with only 5 cases reported in literature. Conclusion: Association of valve- stenotic has been very rarely associated with collagen disease like systemic sclerosis; though other collagenous tissues of the body are involved cardiac valves are generally spared. With only 5 cases reported in the literature we report one such case of this rare association where in an initially affected and treated mitral valve due to rheumatic heart disease has been now involved due to systemic sclerosis.
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