Medical College of Georgia at Augusta University, Augusta, GA, USA.
J Investig Med High Impact Case Rep. 2020 Jan-Dec;8:2324709620925565. doi: 10.1177/2324709620925565.
Immunoglobulin A vasculitis (IgAV), formerly known as Henoch-Schönlein purpura, is an immune-mediated small vessel vasculitis characterized by palpable purpura, arthralgia, abdominal pain, and renal disease. It is primarily a childhood disease and usually resolves spontaneously with supportive therapy. Treatment of IgAV in adults is controversial with no clearly established guidelines. We report a rare case of IgAV in an adult male who developed gut necrosis and perforation while receiving glucocorticoid therapy for treatment of acute glomerulonephritis. A 44-year-old male was admitted with joint pain, leg swelling, mild abdominal pain, and a diffuse rash. Laboratory values revealed acute kidney injury with significant proteinuria and hematuria. The patient was started on glucocorticoid therapy for suspected IgAV nephritis, which was confirmed by kidney biopsy. Several days later, he complained of worsening abdominal pain. Imaging demonstrated bowel ischemia and perforation requiring multiple abdominal surgeries. The patient was critically ill in the intensive care unit with worsening renal failure requiring dialysis. He was discharged a month later after gradual recovery with stable but moderately impaired kidney function. IgAV is less common in adults; however, the disease is more severe with a higher risk of long-term complications. Adult patients with renal involvement may benefit from glucocorticoid therapy in preventing progression to end-stage renal disease. However, glucocorticoids may mask the symptoms of abdominal complications like gut necrosis and perforation causing delay in diagnosis and treatment. Therefore, vigilance to detect early signs of gut ischemia is imperative when treating an adult case of IgAV nephritis with glucocorticoids.
免疫球蛋白 A 血管炎（IgAV），以前称为过敏性紫癜，是一种免疫介导的小血管血管炎，其特征为可触及性紫癜、关节炎、腹痛和肾脏疾病。它主要是一种儿童疾病，通常在支持治疗下自发缓解。IgAV 成人患者的治疗存在争议，没有明确的既定指南。我们报告了一例罕见的 IgAV 成人病例，该患者在接受糖皮质激素治疗急性肾小球肾炎时发生肠坏死和穿孔。一名 44 岁男性因关节痛、腿部肿胀、轻度腹痛和弥漫性皮疹入院。实验室值显示急性肾损伤，伴有大量蛋白尿和血尿。该患者因疑似 IgAV 肾炎接受了糖皮质激素治疗，肾脏活检证实了这一点。几天后，他抱怨腹痛加剧。影像学检查显示肠缺血和穿孔，需要多次腹部手术。患者在重症监护病房病情危重，肾功能衰竭恶化，需要透析。一个月后，他逐渐恢复并稳定出院，但肾功能中度受损。IgAV 在成人中较少见；然而，疾病更为严重，长期并发症的风险更高。有肾脏受累的成年患者可能受益于糖皮质激素治疗以预防进展为终末期肾病。然而，糖皮质激素可能会掩盖腹部并发症（如肠坏死和穿孔）的症状，导致诊断和治疗延迟。因此，在使用糖皮质激素治疗 IgAV 肾炎的成年患者时，必须警惕早期肠缺血的迹象。
