SEGMENTAL MEDIOLYTIC ARTERIOPATHY OF THE SPLENIC AND HEPATIC ARTERIESMIMICKING SYSTEMIC NECROTIZING VASCULITIS

Segmental mediolytic arteriopathy, a rare, non-inflammatory arterial d isease, is fundamentally a variant of fibromuscular dysplasia. The cha racteristic angiographic findings of segmental mediolytic arteriopathy include the ''string of beads'' and microaneurysms which are indistin guishable from those of vasculitis, and the correct diagnosis can be m ade only after histopathologic evaluation of the arterial lesions. Thr ombosis, arterial wall hemorrhage, and dissection are among the compli cations of segmental mediolytic arteriopathy. We describe herein a pat ient with segmental mediolytic arteriopathy who presented with hemoper itoneum. The patient underwent urgent surgical repair of a ruptured he patic artery aneurysm. The postoperative visceral arteriography findin gs led to a postoperative visceral arteriography findings led to a cli nical diagnosis of polyarteritis nodosa, and immunosuppressive therapy was initiated. This treatment was stopped as soon as the correct biop sy diagnosis of segmental mediolytic arteriopathy was obtained through outside consultation. The patient recovered without drug treatment an d was spared the potentially life-threatening complications of immunos uppression.