Department of Gastroenterology, National Hospital Organization Kyoto Medical Center, 1-1 Mukaihatacho, Fukakusa, Kyoto, 612-8555, Japan.
Department of Diagnostic Pathology, National Hospital Organization Kyoto Medical Center, Kyoto, Japan.
Clin J Gastroenterol. 2021 Aug;14(4):1090-1095. doi: 10.1007/s12328-021-01422-6. Epub 2021 May 5.
Immunoglobulin A (IgA) vasculitis mainly affects the joints, skin, kidneys, and gastrointestinal tract; however, purpura is an essential diagnostic criterion. Here, we report an unusual case of IgA vasculitis without purpura in an elderly woman. A 76-year-old woman was admitted to our hospital complaining of diarrhea and abdominal pain. No skin rash, purpura, jaundice, or peripheral lymphadenopathy was observed. Endoscopy of the small intestine revealed severe mucosal sloughing in the duodenum, and a biopsy specimen showed severe erosive duodenitis. A decrease in coagulation factor XIII (FXIII) activity was also observed during laboratory blood tests. IgA immunostaining revealed granular IgA deposition on the walls of the interstitial small blood vessels. Although the patient showed no purpura or renal involvement, a diagnosis of IgA vasculitis was made based on the histopathology findings from biopsies. The administration of purified FXIII concentrate improved her symptoms immediately and facilitated regeneration of the duodenal villi. When gastroenterologists encounter severe erosive duodenitis or inflammation of the small intestine, IgA vasculitis should be listed as part of the differential diagnosis even without purpura and/or renal involvement. For a definitive diagnosis, measurement of FXIII and IgA immunostaining using duodenal biopsy specimens should be performed actively.
免疫球蛋白 A(IgA)血管炎主要影响关节、皮肤、肾脏和胃肠道;然而,紫癜是一个重要的诊断标准。在这里,我们报告一例老年女性无紫癜的 IgA 血管炎的不寻常病例。一名 76 岁女性因腹泻和腹痛入住我院。未观察到皮疹、紫癜、黄疸或外周淋巴结病。小肠内镜检查显示十二指肠严重黏膜脱落,活检标本显示严重侵蚀性十二指肠炎。实验室血液检查还发现凝血因子 XIII(FXIII)活性下降。IgA 免疫染色显示间质小血管壁上有颗粒状 IgA 沉积。尽管患者没有出现紫癜或肾脏受累,但根据活检的组织病理学发现做出了 IgA 血管炎的诊断。纯化 FXIII 浓缩物的给药立即改善了她的症状,并促进了十二指肠绒毛的再生。当胃肠病学家遇到严重的侵蚀性十二指肠炎或小肠炎症时,即使没有紫癜和/或肾脏受累,也应将 IgA 血管炎列入鉴别诊断。为了明确诊断,应积极进行 FXIII 和 IgA 免疫染色测量,使用十二指肠活检标本。