Unusual case of pulmonary renal syndrome with autopsy findings

Scleroderma renal crises (SRC) is a serious complication of systemic sclerosis whose prognosis remains serious despite management with angiotensin-converting enzyme inhibitors, antihypertensives and dialysis. Pulmonary renal syndrome (PRS), characterised by diffuse alveolar hemorrhage (DAH) and SRC,...

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Main Authors: Sonia Badwal, Jyoti Kotwal, Prem Prakash Varma
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2013-01-01
Series:Indian Journal of Pathology and Microbiology
Subjects:
Online Access:http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2013;volume=56;issue=3;spage=294;epage=296;aulast=Badwal
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author Sonia Badwal
Jyoti Kotwal
Prem Prakash Varma
author_facet Sonia Badwal
Jyoti Kotwal
Prem Prakash Varma
author_sort Sonia Badwal
collection DOAJ
description Scleroderma renal crises (SRC) is a serious complication of systemic sclerosis whose prognosis remains serious despite management with angiotensin-converting enzyme inhibitors, antihypertensives and dialysis. Pulmonary renal syndrome (PRS), characterised by diffuse alveolar hemorrhage (DAH) and SRC, is rare and carries a grave prognosis. This case report discusses the clinicopathological features of a 43-year-old male presenting with severe hypertension and rapidly progressive renal failure who subsequently developed DAH and died. The clinical course, exhaustive investigative work-up and autopsy findings led to a diagnosis of diffuse systemic sclerosis with PRS subcategorized into PRS with thrombotic microangiopathy. The index case came without a prior diagnosis of systemic sclerosis, thereby posing a serious diagnostic challenge and management issues.
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spelling doaj.art-7e0abbdee2204884a5c7bf009bdfd3f82022-12-22T00:33:48ZengWolters Kluwer Medknow PublicationsIndian Journal of Pathology and Microbiology0377-49292013-01-0156329429610.4103/0377-4929.120403Unusual case of pulmonary renal syndrome with autopsy findingsSonia BadwalJyoti KotwalPrem Prakash VarmaScleroderma renal crises (SRC) is a serious complication of systemic sclerosis whose prognosis remains serious despite management with angiotensin-converting enzyme inhibitors, antihypertensives and dialysis. Pulmonary renal syndrome (PRS), characterised by diffuse alveolar hemorrhage (DAH) and SRC, is rare and carries a grave prognosis. This case report discusses the clinicopathological features of a 43-year-old male presenting with severe hypertension and rapidly progressive renal failure who subsequently developed DAH and died. The clinical course, exhaustive investigative work-up and autopsy findings led to a diagnosis of diffuse systemic sclerosis with PRS subcategorized into PRS with thrombotic microangiopathy. The index case came without a prior diagnosis of systemic sclerosis, thereby posing a serious diagnostic challenge and management issues.http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2013;volume=56;issue=3;spage=294;epage=296;aulast=BadwalDiffuse alveolar hemorrhagesystemic sclerosisthrombotic microangiopathy
spellingShingle Sonia Badwal
Jyoti Kotwal
Prem Prakash Varma
Unusual case of pulmonary renal syndrome with autopsy findings
Indian Journal of Pathology and Microbiology
Diffuse alveolar hemorrhage
systemic sclerosis
thrombotic microangiopathy
title Unusual case of pulmonary renal syndrome with autopsy findings
title_full Unusual case of pulmonary renal syndrome with autopsy findings
title_fullStr Unusual case of pulmonary renal syndrome with autopsy findings
title_full_unstemmed Unusual case of pulmonary renal syndrome with autopsy findings
title_short Unusual case of pulmonary renal syndrome with autopsy findings
title_sort unusual case of pulmonary renal syndrome with autopsy findings
topic Diffuse alveolar hemorrhage
systemic sclerosis
thrombotic microangiopathy
url http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2013;volume=56;issue=3;spage=294;epage=296;aulast=Badwal
work_keys_str_mv AT soniabadwal unusualcaseofpulmonaryrenalsyndromewithautopsyfindings
AT jyotikotwal unusualcaseofpulmonaryrenalsyndromewithautopsyfindings
AT premprakashvarma unusualcaseofpulmonaryrenalsyndromewithautopsyfindings