Minami Kota, Yamashita Shun, Uno Sumika, Motomura So, Aoki Shigehisa, Kato Seiichi, Tago Masaki
Medical Training Center, Saga University Hospital, Saga, JPN.
Education and Research Center for Community Medicine, Faculty of Medicine, Saga University, Saga, JPN.
Cureus. 2025 May 7;17(5):e83628. doi: 10.7759/cureus.83628. eCollection 2025 May.
Adult patients with immunoglobulin A vasculitis (IgAV) may present with more severe renal symptoms than pediatric patients. Renal biopsy may be difficult to perform in patients with a high risk of bleeding, leading to delayed diagnosis and treatment, and poor prognosis. However, administering steroids before the diagnosis is confirmed may be avoided because of the possibility of the disappearance of the pathological findings of IgAV. A 22-year-old Japanese male was admitted to our hospital with abdominal pain extending from the epigastric to the umbilical region for 10 days, watery diarrhea, and rapidly progressive renal dysfunction. On admission, palpable purpura was observed on both palms and the dorsum of the feet. Blood and urine tests revealed disseminated intravascular coagulation (DIC) and nephrotic syndrome. Although continuous hemodiafiltration was initiated, renal function did not improve. Skin and duodenal biopsies performed for suspected IgAV failed to confirm the diagnosis, and renal biopsy was difficult to perform because of the high risk of bleeding. Steroid pulse therapy was initiated before a definitive diagnosis was made, and DIC began to improve. A renal biopsy performed on the 13th day after starting steroid therapy showed mesangial cell proliferation, and immunofluorescence showed IgA and C3 deposition, confirming IgAV. Steroids were gradually tapered, dialysis was discontinued, and the patient was discharged on the 62nd day of hospitalization. In cases of IgAV with DIC, it is necessary to prioritize steroid administration over renal biopsy, and the biopsy can be performed after the bleeding tendency improves.
成年免疫球蛋白A血管炎(IgAV)患者可能比儿科患者出现更严重的肾脏症状。对于出血风险高的患者,可能难以进行肾活检,从而导致诊断和治疗延迟以及预后不良。然而,由于IgAV的病理表现可能消失,因此可以避免在确诊前使用类固醇。一名22岁的日本男性因上腹部至脐周疼痛10天、水样腹泻和快速进展的肾功能不全入住我院。入院时,双手掌和双足背可见可触及的紫癜。血液和尿液检查显示弥散性血管内凝血(DIC)和肾病综合征。尽管开始了持续血液透析滤过,但肾功能并未改善。因疑似IgAV进行的皮肤和十二指肠活检未能确诊,且由于出血风险高难以进行肾活检。在确诊前开始了类固醇冲击治疗,DIC开始改善。在开始类固醇治疗后的第13天进行的肾活检显示系膜细胞增生,免疫荧光显示IgA和C3沉积,确诊为IgAV。类固醇逐渐减量,停止透析,患者在住院第62天出院。在伴有DIC的IgAV病例中,有必要在肾活检之前优先给予类固醇治疗,待出血倾向改善后再进行活检。