Rupture of a hepatic artery aneurysm and renal infarction: 2 complications of fibromuscular dysplasia that mimic vasculitis.
Fibromuscular dysplasia (FMD) is one of the most important mimics of vasculitis. FMD is more prevalent in women and may cause infarcts of organ systems including the brain, intestines, and extremities. These acute clinical presentations often raise the suspicion of vasculitis and therefore rheumatologists will be asked to evaluate these patients. One of the frequent manifestations of FMD is renal artery involvement. Angiographic evaluation often shows a "string of beads" appearance of the renal arteries but sometimes only tapering of the vascular lumen or cutoffs may be seen. In contrast, hepatic artery involvement with FMD occurs much less frequently and may initially present with life threatening intraabdominal bleeding. Celiac angiograms of these patients often show aneurysms, a presentation similar to vasculitis. Only a high level of suspicion for FMD and careful evaluation of clinical facts will lead to correct diagnosis. This will prevent the wrong diagnosis of vasculitis and subsequent treatment with potentially toxic medications. We present 2 cases of FMD involving the renal and hepatic arteries that illustrate this diagnostic challenge.
Department of Medicine, University of Florida, Gainesville 32610, USA.
H J
HJ
Jones
R
R
Staud
R C
RC
Williams
D000328:Adult; D017542:Aneurysm, Ruptured / Q000150:complications*; D000792:Angiography; D003937:Diagnosis, Differential; D005260:Female; D005352:Fibromuscular Dysplasia / Q000150:complications* / Q000175:diagnosis*; D006499:Hepatic Artery / Q000000981:diagnostic imaging; D006801:Humans; D007238:Infarction / Q000150:complications*; D007668:Kidney / Q000098:blood supply; D008297:Male; D008875:Middle Aged; D012077:Renal Artery / Q000000981:diagnostic imaging; D014657:Vasculitis / Q000175:diagnosis*