A Case of IgA-related Enteropathy Complicated with Gastrointestinal Bleeding and Progressive IgA Nephropathy: A Possible Variant Henoch-Schoenlein Purpura?

• Published in: Internal medicine (Tokyo, 1992) Vol. 49; no. 16; pp. 1755 - 1761
• Main Authors: Nakamura, Shoko, Hisamatsu, Tadakazu, Kikuchi, Jun, Adachi, Masayuki, Yamagishi, Yoshiyuki, Imaeda, Hiroyuki, Hosoe, Naoki, Naganuma, Makoto, Ebinuma, Hirotoshi, Okamoto, Susumu, Kanai, Takanori, Ogata, Haruhiko, Hanaoka, Hironari, Furuya, Yoshiaki, Kawano, Yoshinaga, Bokuda, Kanako, Sasamura, Hiroyuki, Uchida, Hiroshi, Endo, Takashi, Hashiguchi, Akinori, Kameyama, Kaori, Mukai, Makio, Hibi, Toshifumi
• Format: Journal Article
• Language: English
• Published: 01.01.2010
• ISSN: 1349-7235

Here, we report an adult patient with IgA-related enteropathy complicated with massive intestinal bleeding and acute renal failure, but without skin lesions. Surgical resection of the small intestine and steroid pulse therapy was performed. Histopathology revealed significant deposition of IgA and C3 in the small vessels of the intestine and the kidney mesangium. Although skin purpura was absent, the histopathology and clinical manifestations suggested that the pathophysiology was similar to Henoch-Schoenlein purpura (HSP), implying IgA-related enteropathy as a subclass of HSP. Retrospective analysis indicates that terminal ileum lesions may be a poor prognostic indicator.