Interstitial lung disease in polymyositis and dermatomyositis: clinical course and response to treatment.

To assess the prevalence, clinical characteristics, and treatment options of patients with interstitial lung disease (ILD) in polymyositis and dermatomyositis (PM/DM). Sixty-three consecutive patients with PM/DM underwent standardized screening. Patients with ILD were monitored prospectively, and graded immunosuppression was administered according to the rate of clinical progression. ILD was diagnosed in 20 of 63 patients (32%). Generally, the clinical and serologic findings of the anti-Jo1 syndrome were present. Follow-up evaluation disclosed either a progressive or a nonprogressive course. The 10 patients with progressive ILD were distinguished from the nonprogressive group by extensive ground-glass opacities on high-resolution computed tomography (HRCT) and by bronchoalveolar lavage (BAL) neutrophilia. Intravenous pulse cyclophosphamide prevented further progression in all 10 patients and led to some functional improvement. In the 10 patients without rapidly progressive lung disease, immunosuppression of moderate intensity stabilized pulmonary findings during a median 35 months of follow-up. The prevalence of ILD in our patients with PM/DM was 32%; this emphasizes the need for pulmonary screening in all PM/DM patients. Progressive disease, featuring ground-glass opacities on HRCT and an inflammatory BAL cell profile, is amenable to intensive immunosuppression. Conversely, patients who do not have these HRCT and BAL features appear to have a low risk of pulmonary deterioration. Because the treatment for ILD seems to depend on the rate of clinical progression, future therapeutic trials of lung disease in PM/DM should stratify patients accordingly.

Schnabel A ，Reuter M ，Biederer J ，Richter C ，Gross WL ... -  《SEMINARS IN ARTHRITIS AND RHEUMATISM》

Short-term and long-term outcomes of interstitial lung disease in polymyositis and dermatomyositis: a series of 107 patients.

This study was undertaken to assess the characteristics and outcome of interstitial lung disease (ILD) in polymyositis/dermatomyositis (PM/DM) and to determine variables predictive of ILD deterioration in PM/DM. Among 348 consecutive patients with PM/DM, 107 patients with ILD were identified by medical records search in 4 medical centers. All patients underwent pulmonary function tests (PFTs) and pulmonary high-resolution computed tomography (HRCT) scan. ILD onset preceded PM/DM clinical manifestations in 20 patients, was identified concurrently with PM/DM in 69 patients, and occurred after PM/DM onset in 18 patients. Patients with ILD could be divided into 3 groups according to their presenting lung manifestations: patients with acute lung disease (n = 20), patients with progressive-course lung signs (n = 55), and asymptomatic patients with abnormalities consistent with ILD evident on PFTs and HRCT scan (n = 32). We observed that 32.7% of the patients had resolution of pulmonary disorders, whereas 15.9% experienced ILD deterioration. Factors that predicted a poor ILD prognosis were older age, symptomatic ILD, lower values of vital capacity and diffusing capacity for carbon monoxide, a pattern of usual interstitial pneumonia on HRCT scan and lung biopsy, and steroid-refractory ILD. The mortality rate was higher in patients with ILD deterioration than in those without ILD deterioration (47.1% versus 3.3%). Our findings indicate that ILD results in high morbidity in PM/DM. Our findings also suggest that more aggressive therapy may be required in PM/DM patients presenting with factors predictive of poor ILD outcome.

Marie I ，Hatron PY ，Dominique S ，Cherin P ，Mouthon L ，Menard JF ... -  《-》

Differences in clinical features and prognosis of interstitial lung diseases between polymyositis and dermatomyositis.

Fujisawa T ，Suda T ，Nakamura Y ，Enomoto N ，Ide K ，Toyoshima M ，Uchiyama H ，Tamura R ，Ida M ，Yagi T ，Yasuda K ，Genma H ，Hayakawa H ，Chida K ，Nakamura H ... -  《JOURNAL OF RHEUMATOLOGY》

Interstitial lung disease in polymyositis and dermatomyositis: longitudinal evaluation by pulmonary function and radiology.

Fathi M ，Vikgren J ，Boijsen M ，Tylen U ，Jorfeldt L ，Tornling G ，Lundberg IE ... -  《-》

Combination therapy with corticosteroids, cyclosporin A, and intravenous pulse cyclophosphamide for acute/subacute interstitial pneumonia in patients with dermatomyositis.

Kameda H ，Nagasawa H ，Ogawa H ，Sekiguchi N ，Takei H ，Tokuhira M ，Amano K ，Takeuchi T ... -  《JOURNAL OF RHEUMATOLOGY》