Long-term outcomes of patients with propylthiouracil-induced anti-neutrophil cytoplasmic auto-antibody-associated vasculitis
Long-term outcomes of patients with propylthiouracil-induced anti-neutrophil cytoplasmic auto-antibody-associated vasculitis
复制标题
丙硫氧嘧啶诱导的抗中性粒细胞胞质自身抗体相关性血管炎患者的长期结果。
DOI：
10.1093/rheumatology/ken321
复制
发表时间：
2008-10-01
期刊：
影响因子：
5.5
通讯作者：
Zhao, M. -h.
中科院分区：
文献类型：
--
作者：
Gao, Y.;Chen, M.;Zhao, M. -h.
目标
众所周知，丙硫氧嘧啶（PTU）可诱发 ANCA 相关血管炎（AAV），并且临床明显的血管炎在停止 PTU 后无论是否接受免疫抑制治疗均可消退。然而，PTU 诱导的 AAV 患者的治疗策略仍无定论，且缺乏长期结果。我们研究的目的是总结这些数据。
方法
本研究选择了 15 名 PTU 诱导的 AAV 患者，他们接受免疫抑制剂治疗时间<12 个月且随访时间超过 24 个月。回顾性研究临床和病理数据，包括治疗方案和结果。
结果
所有患者的平均随访时间为 55.0 (25-98) 个月。在诊断出 PTU 诱导的 AAV 后，停止使用 PTU。免疫抑制治疗仅适用于肺和肾等重要器官受累的患者，且仅持续7.9+/-3.3（0.27-12）个月。即使在停止免疫抑制治疗后，随访期间也未出现血管炎复发。在最近的随访中，12 名（80%）患者仍处于完全缓解状态，1 名患者仍处于部分缓解状态。两名患者因延迟转诊和延迟停用PTU而出现治疗抵抗，两人均进展为终末期肾病。对于未受控制的甲状腺功能亢进症，六名患者改用他巴唑，没有人出现血管炎复发。
结论
PTU诱导的AAV患者的长期预后相对较好。诊断后应立即停用 PTU。免疫抑制治疗可能仅适用于重要器官受累的患者，并且可能不需要长期维持治疗。
OBJECTIVE
It was well known that propylthiouracil (PTU) could induce ANCA-associated vasculitis (AAV) and clinical evident vasculitis could resolve after cessation of PTU with or without immunosuppressive therapy. However, the treatment strategy for patients with PTU-induced AAV remained inconclusive and their long-term outcomes were lacking. The aim of our study was to summarize these data.
METHODS
Fifteen patients with PTU-induced AAV, receiving immunosuppressive agents for <12 months and following over 24 months, were selected in the current study. The clinical and pathological data, including treatment protocols and outcomes, were retrospectively investigated.
RESULTS
All the patients were followed for a mean of 55.0 (25-98) months. PTU was discontinued upon diagnosis of PTU-induced AAV. Immunosuppressive therapy was administrated only for patients with vital organ involvements, such as lung and kidney, and lasted only 7.9 +/- 3.3 (0.27-12) months. No relapse of vasculitis occurred during follow-up, even after withdrawal of immunosuppressive therapy. Twelve (80%) patients remained in complete remission and one patient remained in partial remission at the latest follow-up. Two patients were treatment resistant due to late referral and late withdrawal of PTU, both of them progressed to end-stage renal disease. For uncontrolled hyperthyroidism on presentation, six patients switched to methimazole and none of them experienced relapse of vasculitis.
CONCLUSIONS
The long-term outcomes of patients with PTU-induced AAV were relatively good. PTU should be discontinued immediately after diagnosis. Immunosuppressive therapy may be only used in patients with vital organ involvements, and a long-term maintenance therapy may not be necessary.