A Ruptured Jejunal Arterial Aneurysm in a Young Woman Undergoing Chronic Hemodialysis Due to Myeloperoxidase-antineutrophil Cytoplasmic Antibody-associated Vasculitis

Abstract

A 21-year-old woman was admitted to our hospital because of massive intestinal bleeding. She started hemodialysis due to myeloperoxidase antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) at 18 years of age. Her ANCA titers remained stable; however, her C-reactive protein increased on 5 mg/day prednisolone before admission. Computed tomography angiography revealed a ruptured jejunal arterial aneurysm. Transcatheter arterial embolization, blood transfusion and the reinforcement of steroid therapy resolved her symptoms of AAV. Our case of a young patient with AAV and medium-sized arterial vasculitis is rare and emphasizes that the ANCA titer does not always rise, especially in patients with nonrenal vasculitis flare-ups.

Keywords: aneurysm; antineutrophil cytoplasmic antibody-associated vasculitis; dialysis; flare-up; gastrointestinal bleeding; hematochezia.

Figure 1.

Computed tomography of the chest. A, B: Multiple nodules in a random pattern and patchy ground-glass opacities are found in her lungs.

Figure 2.

Light microscopic findings of a kidney biopsy. Most of the glomeruli shows global sclerosis and some glomeruli show fibrous crescents with segmental sclerosis and collapse of glomerular capillaries. Tubular atrophy, destruction of tubules and inflammatory cell infiltration in the interstitial areas are found. Periodic acid-Schiff staining. Original magnification ×200.

Figure 3.

Clinical course after introduction of corticosteroid therapy. PSL: prednisolone, CRP: C-reactive protein, MPO-ANCA: myeloperoxidase-antineutrophil cytoplasmic antibody, arrows: steroid pulse therapy (500 mg methylprednisolone, daily boluses given for 3 days)

Figure 4.

Computed tomography angiography in the arterial phase. A: Multiple microaneurysms at the mesenteric arterial branches (arrows) and active bleeding with contrast medium extravasation in the jejunum (asterisk) are found. B: No apparent microaneurysms at the mesenteric arterial branches are observed. Arrow shows high-density spot, indicating that n-butyl 2-cyanoacrylate (NBCA) and a lipiodol mixture is packed in the 2nd jejunal branch of the superior mesenteric artery.

Figure 5.

Digital subtraction angiography. A: Multiple microaneurysms at the mesenteric arterial branches (arrows) and contrast agent extravasation into the jejunum (asterisk). B: The jejunal artery aneurysm rapture (arrow) and contrast agent extravasation into the jejunum (asterisks). C: Active bleeding with contrast medium extravasation is not observed after transcatheter arterial embolization of the 2nd jejunal branch of the superior mesenteric artery.