Kantachuvesiri Pitchaporn, Chalermsanyakorn Panas, Phakdeekitcharoen Bunyong, Lothuvachai Thitima, Niticharoenpong Kannika, Radinahamed Piyanuch, Turner Neil, Kantachuvesiri Surasak
Division of Nephrology, Department of Medicine, Ramathibodi Hospital, Mahidol University, Rama VI Road, Phaya Thai, Bangkok, 10400, Thailand.
Department of Pathology, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
CEN Case Rep. 2015 Nov;4(2):180-184. doi: 10.1007/s13730-014-0163-9. Epub 2014 Dec 31.
We report a case of propylthiouracil (PTU)-induced double antineutrophil cytoplasmic antibody (ANCA) and anti-glomerular basement membrane antibody (anti-GBM antibody) disease causing pulmonary-renal syndrome in a 35-year-old Thai woman with 10-year history of PTU treatment for thyrotoxicosis. She developed clinical symptoms of vasculitis upon receiving long-term PTU treatment. Prednisolone treatment and the switching from PTU to methimazole resulted to short-term clinical improvement. Nevertheless following termination of steroid treatment, she developed recurrent pulmonary hemorrhage and rapidly progressive glomerulonephritis. The kidney biopsy showed crescentic glomerulonephritis with linear IgG deposit on the glomerular basement membrane although transbronchial lung biopsy showed no immune deposit along the alveolar basement membrane. Serum testing for p-ANCA was positive and western blot showed positive antibody to the alpha-3 chain of collagen type IV. Both ANCA and anti-GBM antibody may play a role in the development of end organ damage. To facilitate early and specific intervention, clinicians should be aware of the propensity of PTU to cause lupus-like syndromes with renal involvement. In patients with PTU-induced ANCA-associated glomerulonephritis, serum anti-GBM antibody test may be useful in the early diagnosis of double positive antibodies disease and plasmapheresis should be performed without delay.
我们报告了一例丙硫氧嘧啶(PTU)诱发的双抗中性粒细胞胞浆抗体(ANCA)和抗肾小球基底膜抗体(抗GBM抗体)疾病导致肺肾综合征的病例,患者为一名35岁泰国女性,有10年因甲状腺毒症接受PTU治疗的病史。她在接受长期PTU治疗后出现了血管炎的临床症状。泼尼松龙治疗以及从PTU换用甲巯咪唑导致了短期的临床改善。然而,在停止类固醇治疗后,她出现了反复的肺出血和快速进展的肾小球肾炎。肾活检显示新月形肾小球肾炎,肾小球基底膜上有线性IgG沉积,尽管经支气管肺活检显示沿肺泡基底膜无免疫沉积物。血清p-ANCA检测呈阳性,western blot显示对IV型胶原α-3链的抗体呈阳性。ANCA和抗GBM抗体可能在终末器官损伤的发生中起作用。为了便于早期和特异性干预,临床医生应意识到PTU导致伴有肾脏受累的狼疮样综合征的倾向。在PTU诱发的ANCA相关性肾小球肾炎患者中,血清抗GBM抗体检测可能有助于双阳性抗体疾病的早期诊断,应立即进行血浆置换。