title: 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.affiliation: Department of Pathology, Armed Forces Medical College, Delhi, India.
authors.forename: Sonia
authors.initials: S
authors.lastname: Badwal
authors.forename: Jyoti
authors.initials: J
authors.lastname: Kotwal
authors.forename: Prem Prakash
authors.initials: PP
authors.lastname: Varma
mesh_terms: D000328:Adult; D001344:Autopsy; D017809:Fatal Outcome; D005921:Glomerulonephritis / Q000150:complications / Q000209:etiology* / Q000473:pathology*; D006470:Hemorrhage / Q000150:complications / Q000209:etiology* / Q000473:pathology*; D006801:Humans; D006973:Hypertension; D008171:Lung Diseases / Q000150:complications / Q000209:etiology* / Q000473:pathology*; D008297:Male; D051437:Renal Insufficiency; D045743:Scleroderma, Diffuse / Q000150:complications* / Q000175:diagnosis*