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目的研究丙基硫氧嘧啶(PTU)诱发的抗中性粒细胞胞质抗体(ANCA)阳性的甲状腺功能亢进症(甲亢)患者的临床和预后特点。方法对45例PTU诱发的ANCA阳性的甲亢患者进行长期随访(3~60个月,中位数27个月),观察其甲状腺功能和ANCA百分结合率的变化,对其中17例有小血管炎临床表现的患者进一步观察血管炎的病情变化。结果(1)血管炎组17例患者中13例(76·5%)肾脏受累,表现为血尿、蛋白尿及不同程度的肾功能受损,7例(41·2%)肺脏受累,多表现为肺出血。核周型ANCA(p-ANCA)阳性率为100%,其中16例(94·1%)髓过氧化物酶(MPO)-ANCA抗体阳性。所有患者均停用PTU,6例改用他巴唑治疗甲亢,13例内脏受累较重的患者应用免疫抑制剂治疗6~12个月。除2例诊治较晚的患者发展至终末期肾衰竭外,其余患者临床症状均得到缓解,随访期间(8~60个月,中位数30个月)未发现小血管炎复发。(2)无血管炎临床表现组28例患者中有27例(96·4%)为p-ANCA阳性,14例(50·0%)的MPO-ANCA阳性。24例患者停用PTU,其中15例改用他巴唑;4例患者继续应用小剂量PTU。随访期间(3~36个月,中位数20个月)均未发生小血管炎临床表现。但血管炎组及无血管炎组多数患者ANCA百分结合率下降较慢。结论PTU诱发的ANCA阳性甲亢患者应及时停用PTU,必要时改用他巴唑治疗甲亢;血管炎病情严重者应用免疫抑制治疗。停用PTU后未发现小血管炎复发,但ANCA可较长时间维持阳性。
Objective To investigate the clinical and prognostic features of propylthiouracil (PTU) -mediated anti-neutrophil cytoplasmic antibody (ANCA) -positive hyperthyroidism (Hyperthyroidism). Methods 45 cases of PTU-induced ANCA-positive patients with hyperthyroidism were followed up for 3 to 60 months with a median of 27 months. The changes of thyroid function and ANCA percentage were observed. Among them 17 cases had small blood vessels Patients with clinical manifestations of inflammation further vasculitis changes in the disease. Results (1) Thirteen patients (76.5%) in 17 patients with vasculitis had renal involvement, including hematuria, proteinuria and varying degrees of renal dysfunction. Pulmonary involvement was found in 7 patients (41.2%) with multiple manifestations Bleeding for the lungs. The positive rate of perinuclear ANCA (p-ANCA) was 100%, of which 16 cases (94.1%) were positive for myeloperoxidase (MPO) -ANCA antibody. All patients were discontinued PTU, 6 patients switched to methimazole treatment of hyperthyroidism, 13 patients with severe visceral involvement of immunosuppressive agents for 6 to 12 months. The clinical symptoms of other patients were relieved except for two cases of patients with later diagnosis and treatment of renal failure. No recurrence of vasculitis was found during the follow-up (range, 8 to 60 months, median 30 months). (2) In the clinical manifestations of vasculitis, 27 (96.4%) of 28 patients were positive for p-ANCA and 14 (50.0%) were positive for MPO-ANCA. Twenty-four patients discontinued PTU, of which 15 patients switched to methimazole; 4 patients continued to use low-dose PTU. During follow-up (3-36 months, median 20 months) did not occur in the clinical manifestations of small vasculitis. However, most patients with vasculitis and no vasculitis ANCA percentage decline slowed down. Conclusion PTU-induced ANCA-positive hyperthyroidism patients should promptly disable PTU, if necessary, switch to methimazole treatment of hyperthyroidism; vasculitis in severe cases, the application of immunosuppressive therapy. No recurrence of small vasculitis was found after PTU discontinuation, but ANCA remained positive for a longer period of time.