Interstitial Lung Disease in Anti–Jo-1 Patients With Antisynthetase Syndrome
Article first published online: 23 APR 2013
Copyright © 2013 by the American College of Rheumatology
Arthritis Care & Research
Volume 65, Issue 5, pages 800–808, May 2013
How to Cite
Marie, I., Josse, S., Hatron, P. Y., Dominique, S., Hachulla, E., Janvresse, A., Cherin, P., Mouthon, L., Vittecoq, O., Menard, J.-F. and Jouen, F. (2013), Interstitial Lung Disease in Anti–Jo-1 Patients With Antisynthetase Syndrome. Arthritis Care Res, 65: 800–808. doi: 10.1002/acr.21895
- Issue published online: 23 APR 2013
- Article first published online: 23 APR 2013
- Accepted manuscript online: 30 NOV 2012 03:53PM EST
- Manuscript Accepted: 23 OCT 2012
- Manuscript Received: 30 MAY 2012
To assess the outcome of interstitial lung disease (ILD) in anti–Jo-1 patients with antisynthetase syndrome, determine predictive variables of ILD deterioration in these patients, and compare features of anti–Jo-1 patients with and without ILD.
Ninety-one anti–Jo-1 patients were identified by medical records search in 4 medical centers. All of these patients had undergone pulmonary function tests (PFTs) and high-resolution computed tomography (HRCT) scans.
Sixty-six patients (72.5%) had ILD. Patients could be divided into 3 groups according to their presenting lung manifestations: acute onset of lung disease (n = 12), progressive onset of lung signs (n = 35), and asymptomatic patients exhibiting abnormalities consistent with ILD on PFTs and HRCT scans (n = 19). Sixteen patients had resolution of ILD; 39 and 11 patients experienced improvement and deterioration of ILD, respectively. ILD led to decreased functional status, since 29.8% of patients exhibited a marked reduction of activities due to ILD and 13.6% had respiratory insufficiency requiring oxygen therapy; 5 of 6 patients died due to ILD complications. Predictive parameters of ILD deterioration were HRCT scan pattern of usual interstitial pneumonia, respiratory muscle involvement, and age ≥55 years. Furthermore, anti–Jo-1 patients with ILD, compared with those without, more frequently exhibited mechanic's hands and lower creatine kinase levels.
Our findings confirm that ILD is a frequent complication in anti–Jo-1 patients, resulting in high morbidity. We suggest that patients with predictive factors of ILD deterioration may require more aggressive therapy. Finally, anti–Jo-1 patients with ILD, compared with those without, may exhibit a particular clinical phenotype.