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DOI：
10.1002/art.40782
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发表时间：
2019
影响因子：
13.3
通讯作者：
Crofford Leslie J.
中科院分区：
文献类型：
--
作者：
Wilfong Erin M.;Bayram Kevin W.;Crofford Leslie J.
我们感谢Novikov博士及其同事对我们最近的审查提出的深思熟虑的意见[1]。我们同意IPAF的ATS/ERS分类标准[2]肯定需要进行审查、修订和验证。我们也完全同意风湿病学家和肺病学家在这些患者的管理中的重要合作。在我们正在进行的前瞻性纵向队列研究中，> 93%的患者同时接受风湿病学和肺病学治疗。我们在全球范围内强烈提倡这种方法，以便在出现时提供准确的分类/诊断，并监测明确定义的结缔组织病（CTD）的出现。我们同意ANCA检测应在新发间质性肺病（ILD）的情况下进行，尽管2018年特发性肺纤维化诊断指南中未普遍推荐ANCA检测[3]。然而，ILD和ANCA阳性的患者是否是未分化的，还不太清楚。2014年，Saadoun及其同事对肺纤维化和ANCA相关血管炎（AAV）患者进行了回顾性研究，髓过氧化物酶抗体特异性远比蛋白酶-3特异性更常见。他们报告称，约40%的患者在临床上明显的血管炎之前有肺纤维化的证据，另外40%的患者同时存在肺纤维化和系统性血管炎的证据，通常是显微镜下多血管炎。存在多种放射学模式。有趣的是，肺纤维化患者的预后较差，总体死亡率为56%，通过糖皮质激素联合环磷酰胺或利妥昔单抗与糖皮质激素单独治疗组成的诱导治疗，这在一定程度上有所改善[4]。AAV的诱导治疗方案与其他CTD-ILD的初始治疗不同，因此这些患者可能会从靶向血管炎治疗中获益。此外，ACR/EULAR草案
We thank Dr. Novikov and colleagues for their thoughtful comments on our recent review [1]. We agree that the ATS/ERS classification criteria for IPAF [2] will most certainly need to be reviewed, revised, and validated moving forward. We also agree whole-heartedly with the critical collaboration between rheumatologists and pulmonologists in the management of these patients. In our ongoing prospective longitudinal cohort,> 93% of patients are comanaged by rheumatology and pulmonology. We strongly advocate this approach worldwide both to provide accurate classification/diagnosis at the time of presentation and to monitor for the emergence of a clearly defined connective tissue disease (CTD).We agree that ANCA testing should occur in the setting of new interstitial lung disease (ILD), although ANCA testing was not universally recommended in the 2018 idiopathic pulmonary fibrosis diagnostic guidelines [3]. Whether or not a patient with ILD and positive ANCA is undifferentiated, however, is much less clear. In 2014, Saadoun and coworkers conducted a retrospective review of patients with pulmonary fibrosis and ANCA-associated vasculitis (AAV), with myeloperoxidase antibody specificity being far more common than proteinase-3 specificity. They reported that approximately 40% of patients had evidence of pulmonary fibrosis ahead of clinically apparent vasculitis, and another 40% presented with both pulmonary fibrosis and evidence of systemic vasculitis, usually microscopic polyangiitis. A variety of radiographic patterns were present. Interestingly, patients with pulmonary fibrosis had a poor prognosis with a global mortality of 56%, which was somewhat improved by induction therapy consisting of glucocorticoids plus either cyclophosphamide or rituximab versus glucocorticoids alone [4]. The induction treatment regimens for AAV differ from initial treatment of other CTD-ILDs, so these patients would likely benefit from targeted vasculitis therapy. Additionally, draft ACR/EULAR