Department of Respiratory Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, #1 Shuaifuyuan Street, Beijing, Dongcheng District 100730, China.
BMC Pulm Med. 2014 Jan 28;14:8. doi: 10.1186/1471-2466-14-8.
Pulmonary involvement is a common feature of MPA. Although alveolar hemorrhage is the most common pulmonary manifestation of MPA, a few recent studies have described instances of MPA patients with pulmonary fibrosis. Pulmonary fibrosis was seen to predate, be concomitant with, or occur after the diagnosis of MPA. The goal of this study was to describe the clinical features and prognosis of microscopic polyangiitis (MPA) patients whose initial respiratory presentation was pulmonary fibrosis.
We conducted a retrospective analysis of 19 MPA patients who presented with pulmonary fibrosis at Peking Union Medical College Hospital between 1990 and 2012.
Of 67 total MPA cases, 19 patients presented with pulmonary fibrosis. There were 8 males and 11 females, with a median age of 63.6 years. Common clinical manifestations included fever (89.5%), cough (84.2%), dyspnea (78.9%) and velcro rales (84.2%). Eleven patients experienced weight loss, several had kidney involvement, and most had an increased erythrocyte sedimentation rate and C-reactive protein. All were positive for myeloperoxidase-anti-neutrophil cytoplasmic antibody (ANCA), with 6 patients being positive at the time of their initial diagnosis of pulmonary fibrosis. Every patient had typical features of usual interstitial pneumonia on High-resolution CT. All were treated with corticosteroids and cyclophosphamide, which lead to an improvement in twelve cases. One of the remaining patients progressed slowly, whereas six died.
Patients with MPA, who also presented with pulmonary fibrosis in our cohort, were more likely to be older, female, and have extrapulmonic involvement. Most patients had a delayed positive ANCA. Corticosteroids plus cyclophosphamide was the remission-induction treatment scheme for all cases. The current prognosis for MPA patients with pulmonary fibrosis appears to be poor, suggesting that they may be candidates for new therapies.
肺脏受累是显微镜下多血管炎（MPA）的常见特征。尽管肺泡出血是 MPA 最常见的肺部表现，但最近有几项研究描述了 MPA 患者出现肺纤维化的情况。肺纤维化的出现早于、同时或晚于 MPA 的诊断。本研究的目的是描述以肺纤维化为初始呼吸表现的显微镜下多血管炎（MPA）患者的临床特征和预后。
我们对 1990 年至 2012 年期间在北京协和医院就诊的以肺纤维化为初始表现的 MPA 患者进行了回顾性分析。
在 67 例 MPA 患者中，19 例患者表现为肺纤维化。其中男性 8 例，女性 11 例，中位年龄为 63.6 岁。常见的临床表现包括发热（89.5%）、咳嗽（84.2%）、呼吸困难（78.9%）和湿啰音（84.2%）。11 例患者出现体重减轻，部分患者出现肾脏受累，大多数患者红细胞沉降率和 C 反应蛋白升高。所有患者均为髓过氧化物酶-抗中性粒细胞胞质抗体（MPO-ANCA）阳性，其中 6 例在肺纤维化的初始诊断时即为阳性。所有患者高分辨率 CT 均表现为典型的间质性肺炎特征。所有患者均接受了糖皮质激素和环磷酰胺治疗，12 例患者病情改善。其余 1 例患者病情进展缓慢，6 例患者死亡。
在我们的研究队列中，以肺纤维化为首发表现的 MPA 患者更可能为老年、女性，且伴有肺外表现。大多数患者的 MPO-ANCA 呈延迟阳性。糖皮质激素联合环磷酰胺是所有患者的诱导缓解治疗方案。目前，肺纤维化 MPA 患者的预后似乎较差，这提示他们可能是新疗法的候选人群。
