Unusual case of pulmonary renal syndrome with autopsy findings.

Abstract

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.

• Authors: Sonia Badwal, Jyoti Kotwal, Prem Prakash Varma
• Journal: Indian journal of pathology & microbiology (Indian J Pathol Microbiol) 2013 Jul-Sep Vol. 56 Issue 3 Pg. 294-6 ISSN: 0974-5130 [Electronic] India
• PMID: 24152515 (Publication Type: Case Reports, Journal Article)

Topics

• Adult
• Autopsy
• Fatal Outcome
• Glomerulonephritis (complications, etiology, pathology)
• Hemorrhage (complications, etiology, pathology)
• Humans
• Hypertension
• Lung Diseases (complications, etiology, pathology)
• Male
• Renal Insufficiency
• Scleroderma, Diffuse (complications, diagnosis)