Wong Albert Hing, Wong Wei-Kei, Looi Lai-Meng, Ratnasingam Jeyakantha, Lim Soo-Kun
Renal Division, Department of Medicine, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia.
Department of Pathology, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia.
Case Rep Nephrol Dial. 2022 Jun 17;12(2):105-111. doi: 10.1159/000525182. eCollection 2022 May-Aug.
Propylthiouracil (PTU)-induced antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is a rare and heterogeneous disease. Moreover, optimal treatment is still lacking. We described the case of a 44-year-old lady with underlying Graves' disease who had cough, blood-streaked sputum, and impaired renal function. A strongly positive anti-myeloperoxidase antibody (>200 U/mL) along with pauci-immune glomerulonephritis and pulmonary hemorrhage resulted in the diagnosis of PTU-induced AAV, given that the patient had been on PTU for 3 years. PTU withdrawal, therapeutic plasma exchanges, and oral cyclophosphamide provided favorable clinical and biochemical outcomes. She remained well on azathioprine 50 mg daily as maintenance therapy and clinically euthyroid with carbimazole 2.5 mg daily. The effective treatment for drug-induced ANCA vasculitis remains controversial, but rapid withdrawal of the offending medication should be the mainstay of treatment. In severe drug-induced ANCA vasculitis with pulmonary hemorrhage and/or life-threatening organ involvement such as kidney failure requiring dialysis, therapeutic plasma exchange with immunosuppressants is often required. In this case, we have shown that patient achieved remission after therapeutic plasma exchange with cyclophosphamide in the acute stage of treatment and remained symptom-free with azathioprine in the maintenance phase of treatment for 24 months.
丙硫氧嘧啶(PTU)诱发的抗中性粒细胞胞浆抗体(ANCA)相关性血管炎(AAV)是一种罕见的异质性疾病。此外,目前仍缺乏最佳治疗方案。我们描述了一例44岁患有Graves病的女性患者,她出现咳嗽、痰中带血及肾功能损害。鉴于该患者已服用PTU 3年,抗髓过氧化物酶抗体强阳性(>200 U/mL),同时伴有寡免疫性肾小球肾炎和肺出血,最终诊断为PTU诱发的AAV。停用PTU、进行治疗性血浆置换及口服环磷酰胺后,患者获得了良好的临床和生化指标改善。她继续以每日50 mg硫唑嘌呤作为维持治疗,且临床甲状腺功能正常,每日服用2.5 mg卡比马唑。药物诱发的ANCA血管炎的有效治疗仍存在争议,但迅速停用致病药物应是治疗的主要手段。对于伴有肺出血和/或危及生命的器官受累(如需要透析的肾衰竭)的严重药物诱发的ANCA血管炎,通常需要进行治疗性血浆置换并联合使用免疫抑制剂。在本病例中,我们发现患者在治疗急性期进行血浆置换联合环磷酰胺治疗后病情缓解,在治疗维持期服用硫唑嘌呤24个月期间无症状。