A retrospective study of microscopic polyangiitis patients presenting with pulmonary fibrosis in China.
BACKGROUND
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.


METHODS
We conducted a retrospective analysis of 19 MPA patients who presented with pulmonary fibrosis at Peking Union Medical College Hospital between 1990 and 2012.


RESULTS
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.


CONCLUSIONS
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.
Hui
H
Huang
Yan Xun
YX
Wang
Chun Guo
CG
Jiang
Jia
J
Liu
Ji
J
Li
Kai
K
Xu
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. xuzj@hotmail.com.
Zuo Jun
ZJ
Xu
D000328:Adult; D000368:Aged; D002681:China; D005260:Female; D006801:Humans; D008297:Male; D055953:Microscopic Polyangiitis / Q000150:complications* / Q000175:diagnosis*; D008875:Middle Aged; D011379:Prognosis; D011658:Pulmonary Fibrosis / Q000209:etiology*; D012189:Retrospective Studies