PDF(Pubmed)
Abstract：
We report a case of a 34-year-old male with severe multisystemic involvement (including the testis, musculoskeletal system, skin, upper respiratory tract, ocular system, peripheral nerves, abdomen, and kidney) due to granulomatosis with polyangiitis (GPA) and a high proteinase 3 (PR3)-antineutrophil cytoplasmic antibodies (PR3ANCA) titer. A renal biopsy showed pauci-immune glomerulonephritis (GN). Systemic corticotherapy combined with cyclophosphamide was chosen for induction therapy. During the induction phase, clinical deterioration occurred in the form of severe alveolar hemorrhage, leading to admission to the intensive care unit (ICU). Influenza A (H1N1) was detected in the respiratory tract. Furthermore, blood sampling revealed an invasive Klebsiella pneumoniae infection that persisted despite multiple antibiotic regimens. A CT scan showed splenic vascular compromise, assumed to be the primary source of the infection, with sustained improvement after splenectomy. Maintenance therapy included a tapering dose of corticotherapy for 36 months and azathioprine 100mg daily for five years, which achieved full and sustained remission. The patient has been in full remission for nine years, with mild renal sequelae, including proteinuria and secondary hypertension.
摘要：
我们报告了一例34岁的男性，患有严重的多系统受累（包括睾丸，肌肉骨骼系统,皮肤,上呼吸道,眼系统,周围神经,腹部，和肾脏）由于肉芽肿性多血管炎（GPA）和高蛋白酶3（PR3）-抗中性粒细胞胞浆抗体（PR3ANCA）滴度。肾活检显示浅层免疫性肾小球肾炎（GN）。选择全身皮质治疗联合环磷酰胺用于诱导治疗。在诱导阶段,临床恶化以严重肺泡出血的形式发生，导致入住重症监护病房（ICU）。在呼吸道中检测到甲型H1N1流感。此外，血液采样显示侵袭性肺炎克雷伯菌感染尽管有多种抗生素治疗方案,但仍持续存在.CT扫描显示脾血管受损,假设是感染的主要来源，脾切除术后持续改善。维持治疗包括逐渐减少剂量的皮质治疗36个月和硫唑嘌呤100mg每日5年，实现了全面和持续的缓解。病人已经完全缓解了九年,有轻微的肾脏后遗症，包括蛋白尿和继发性高血压。
