Petrou Dimitra, Karagiannis Minas, Nikolopoulos Petros, Liapis George, Lionaki Sophia
Department of Nephrology, National and Kapodistrian University of Athens, Attikon Hospital, 12462 Athens, Greece.
Department of Pathology, National and Kapodistrian University of Athens, Laiko Hospital, 11527 Athens, Greece.
Antibodies (Basel). 2022 May 9;11(2):33. doi: 10.3390/antib11020033.
Early diagnosis and initiation of appropriate immunosuppressive treatment remain the cornerstone of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis at the cost of significant toxicity. In this report, we present a case of a 69-year-old female who presented with advanced renal insufficiency and evidence of pulmonary hemorrhage and was MPO-ANCA-positive with a clinical phenotype of granulomatosis with polyangiitis. Organ involvement included rapidly progressive glomerulonephritis (GN), along with extrarenal manifestations (skin, upper and lower respiratory system involvement, and onset of saddle-nose deformity). Kidney biopsy established the diagnosis of pauci-immune crescentic, sclerotic GN. She received therapy with glucocorticoids and cyclophosphamide, mainly due to life-threatening extra-renal manifestations, such as pulmonary hemorrhage. She avoided vasculitis-related death but she developed severe therapy-related toxicity, resulting in the discontinuation of immunosuppressive therapy. Continuous re-evaluation of patients with ANCA-associated vasculitis in terms of response to immunosuppressive therapy and treatment-related toxicity is crucial for their management.
早期诊断并开始适当的免疫抑制治疗仍然是以显著毒性为代价的抗中性粒细胞胞浆抗体（ANCA）相关血管炎的基石。在本报告中，我们介绍了一名69岁女性病例，该患者出现晚期肾功能不全及肺出血证据，髓过氧化物酶抗中性粒细胞胞浆抗体（MPO-ANCA）阳性，具有肉芽肿性多血管炎的临床表型。器官受累包括快速进展性肾小球肾炎（GN）以及肾外表现（皮肤、上下呼吸道系统受累，出现鞍鼻畸形）。肾脏活检确诊为寡免疫性新月体性、硬化性GN。她接受了糖皮质激素和环磷酰胺治疗，主要是因为存在威胁生命的肾外表现，如肺出血。她避免了血管炎相关死亡，但出现了严重的治疗相关毒性，导致免疫抑制治疗中断。对ANCA相关血管炎患者在免疫抑制治疗反应和治疗相关毒性方面进行持续重新评估对其管理至关重要。
