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Diffuse Systemic Sclerosis with Left Ventricular Diastolic Dysfunction: A Case Report

Systemic sclerosis is a connective tissue disease characterized by wide-spread vascular lesions and fibrosis of the skin and internal organs. It is an immune mediated rheumatic disease with the presence of an immunological dysfunction of T lymphocytes, especially Th1 and Th17 subtypes. It affects ga...

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Detalles Bibliográficos
Autores principales: Neupane, Asmita, Luintel, Prabesh, Dhakal, Subodh Sagar
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Journal of the Nepal Medical Association 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7580412/
https://www.ncbi.nlm.nih.gov/pubmed/32335662
http://dx.doi.org/10.31729/jnma.4768
Descripción
Sumario:Systemic sclerosis is a connective tissue disease characterized by wide-spread vascular lesions and fibrosis of the skin and internal organs. It is an immune mediated rheumatic disease with the presence of an immunological dysfunction of T lymphocytes, especially Th1 and Th17 subtypes. It affects gastrointestinal, pulmonary, vascular, musculoskeletal, cardiac and various other systems. This disease is rare but has high morbidity and mortality with less known effective management. We report a case of 70-year-old female with systemic sclerosis presented with pain along with swelling over multiple joints since 18 months which exacerbated since last 6 months and wound over finger tips since last 2 weeks. We present here other various signs, investigations and management of this uncommon disease systemic sclerosis, also known as scleroderma. Various systems are evident to be involved including cardiac (left ventricular diastolic dysfunction) and peripheral vascular system (Raynaud's phenomenon).