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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
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author Neupane, Asmita
Luintel, Prabesh
Dhakal, Subodh Sagar
author_facet Neupane, Asmita
Luintel, Prabesh
Dhakal, Subodh Sagar
author_sort Neupane, Asmita
collection PubMed
description 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).
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spelling pubmed-75804122020-11-30 Diffuse Systemic Sclerosis with Left Ventricular Diastolic Dysfunction: A Case Report Neupane, Asmita Luintel, Prabesh Dhakal, Subodh Sagar JNMA J Nepal Med Assoc 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 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). Journal of the Nepal Medical Association 2019 2019-12-31 /pmc/articles/PMC7580412/ /pubmed/32335662 http://dx.doi.org/10.31729/jnma.4768 Text en © The Author(s) 2018. http://creativecommons.org/licenses/by/4.0/ This is an Open-Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Neupane, Asmita
Luintel, Prabesh
Dhakal, Subodh Sagar
Diffuse Systemic Sclerosis with Left Ventricular Diastolic Dysfunction: A Case Report
title Diffuse Systemic Sclerosis with Left Ventricular Diastolic Dysfunction: A Case Report
title_full Diffuse Systemic Sclerosis with Left Ventricular Diastolic Dysfunction: A Case Report
title_fullStr Diffuse Systemic Sclerosis with Left Ventricular Diastolic Dysfunction: A Case Report
title_full_unstemmed Diffuse Systemic Sclerosis with Left Ventricular Diastolic Dysfunction: A Case Report
title_short Diffuse Systemic Sclerosis with Left Ventricular Diastolic Dysfunction: A Case Report
title_sort diffuse systemic sclerosis with left ventricular diastolic dysfunction: a case report
topic Case Report
url 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
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