A case of IgA-related enteropathy complicated with gastrointestinal bleeding and progressive IgA nephropathy: a possible variant Henoch-Schönlein purpura?
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-Schönlein purpura (HSP), implying IgA-related enteropathy as a subclass of HSP. Retrospective analysis indicates that terminal ileum lesions may be a poor prognostic indicator.
Nakamura
Shoko
S
The Division of Gastroenterology and Hepatology, Department of Internal Medicine, School of Medicine, Keio University, Tokyo, Japan.
Hisamatsu
Tadakazu
T
Kikuchi
Jun
J
Adachi
Masayuki
M
Yamagishi
Yoshiyuki
Y
Imaeda
Hiroyuki
H
Hosoe
Naoki
N
Naganuma
Makoto
M
Ebinuma
Hirotoshi
H
Okamoto
Susumu
S
Kanai
Takanori
T
Ogata
Haruhiko
H
Hanaoka
Hironari
H
Furuya
Yoshiaki
Y
Kawano
Yoshinaga
Y
Bokuda
Kanako
K
Sasamura
Hiroyuki
H
Uchida
Hiroshi
H
Endo
Takashi
T
Hashiguchi
Akinori
A
Kameyama
Kaori
K
Mukai
Makio
M
Hibi
Toshifumi
T
D003937:Diagnosis, Differential; D018450:Disease Progression; D017809:Fatal Outcome; D006471:Gastrointestinal Hemorrhage / Q000150:complications / Q000175:diagnosis*; D005922:Glomerulonephritis, IGA / Q000150:complications / Q000175:diagnosis*; D006801:Humans; D011695:IgA Vasculitis / Q000150:complications / Q000175:diagnosis*; D007070:Immunoglobulin A / Q000009:adverse effects; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies
D007070:Immunoglobulin A