Wünderlich syndrome from lupus-associated vasculitis
Journal
American Journal of Kidney Diseases
Journal Volume
61
Journal Issue
1
Pages
167-170
Date Issued
2013
Author(s)
Abstract
Spontaneous perinephric hemorrhage is a clinically rare life-threatening condition, also known as W?nderlich syndrome. Presentations can be variable, but patients typically display symptoms and signs including flank or abdominal pain, abdominal mass, and hypovolemia. It is important to diagnose the syndrome early because untreated cases carry a high mortality risk, and prompt intervention with an endovascular procedure or surgery is life saving. Causes range from anatomical anomalies, including vascular diseases (vasculitides and aneurysms) and renal tumors, to functional coagulation defect (bleeding tendency). The most common causes of W?nderlich syndrome are renal angiomyolipoma and renal cell carcinoma, constituting 60%?70% of cases. Vascular causes of W?nderlich syndrome are infrequent, and the culprit most frequently is vasculitis resulting from polyarteritis nodosa. Other vasculitides presenting as W?nderlich syndrome are infrequent. We describe a 39-year-old woman with end-stage renal disease from lupus nephritis and spontaneous renal hemorrhage, ascribed to lupus-related vasculitis after serologic testing, computed tomography, and angiographic studies. ? 2012 National Kidney Foundation, Inc.
SDGs
Other Subjects
antinuclear antibody; calcium acetate; complement component C3; complement component C4; double stranded DNA antibody; erythropoietin; phosphate; prednisolone; abdominal pain; adult; antibody blood level; antibody titer; article; backache; case report; computer assisted tomography; contrast enhancement; contrast medium extravasation; female; hematocrit; hematoma; hematuria; hemodialysis; human; hypocomplementemia; kidney hemorrhage; kidney polycystic disease; lower abdominal pain; lupus erythematosus nephritis; orthostatic hypotension; pallor; phosphate blood level; postural orthostatic tachycardia syndrome; proteinuria; rash; supine position; vasculitis; wunderlich syndrome; Adult; Angiography; Female; Hemorrhage; Humans; Kidney Failure, Chronic; Lupus Erythematosus, Systemic; Lupus Nephritis; Renal Dialysis; Tomography, X-Ray Computed; Vasculitis
Type
journal article