Abstract
Backgrounds: Systemic vasculitis is known to show variable lung manifestations such as diffuse alveolar hemorrhage, nodule, bronchial stenosis and pulmonary fibrosis. However, clinical features, courses and outcome of vasculitis related interstitial lung disease (Vasculitis-ILD) have not well been defined. The aim of our study was to investigate clinical course and outcome of Vasculitis-ILD.
Methods: Vasculitis was confirmed in 119 patients in Asan Medical Center, Seoul, Korea. Among them, those with ILD (14 patients, 12%) were included in this study. We retrospectively analyzed their clinical features, course and outcome using medical records and the record of National Health Insurance of Korea.
Results: The median follow-up period was 14 months. Mean age was 60 ± 13 years, female was 57%, and smoker was 57% (current: 21%, ex-smoker: 36%). Microscopic polyarteritis was the most common (71%), followed by polyarteritis nodosa (21%) and Wegener's granulomatosis (7%). Myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) was detected in 11 (85%) patients. Usual interstitial pneumonia (UIP) was the most common pattern seen on high resolution computed tomography (57%) and surgical lung biopsy (50%). Most of the subjects (n=13) were treated with prednisolone and the mean dose was 39 mg. After treatment, 8 patients (62%) showed stabilized lung function, but the others showed progression of the disease. The subjects had poorer outcome compared with those without lung involvement (1-year survival: 69% vs. 93%; p=0.015).
Conclusion: Microscopic polyarteritis and UIP pattern was the most common in Vasculitis-ILD. Prognosis of patients with pulmonary fibrosis was poorer than those without.
- © 2014 ERS