OBJECTIVES: To assess the prevalence, clinical characteristics, and treatment options of @entity1 with @entity401 ( @entity401 ) in @entity6558 and @entity6017 (PM/ @entity3918 ). @entity1 AND METHODS: Sixty-three consecutive @entity1 with PM/ @entity3918 underwent standardized screening. @entity1 with @entity401 were monitored prospectively, and graded immunosuppression was administered according to the rate of clinical progression. RESULTS: @entity401 was diagnosed in 20 of 63 @entity1 (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 @entity1 with progressive @entity401 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 @entity786 prevented further progression in all 10 @entity1 and led to some functional improvement. In the 10 @entity1 without rapidly progressive @entity358 , immunosuppression of moderate intensity stabilized pulmonary findings during a median 35 months of follow-up. CONCLUSIONS: The prevalence of @entity401 in our @entity1 with PM/ @entity3918 was 32%; this emphasizes the need for pulmonary screening in all PM/ @entity3918 @entity1 . Progressive disease, featuring ground-glass opacities on HRCT and an inflammatory BAL cell profile, is amenable to intensive immunosuppression. Conversely, @entity1 who do not have these HRCT and BAL features appear to have a low risk of @entity358 . RELEVANCE: Because the treatment for @entity401 seems to depend on the rate of clinical progression, future therapeutic trials of @entity358 in PM/ @entity3918 should stratify @entity1 accordingly.

@entity401 in @entity6558 and XXXX : clinical course and response to treatment.

@entity1 :: ('9606', 'Species') :: ['Patients', 'patients', 'PATIENTS'] @entity6017 :: ('MESH:D003882', 'Disease') :: ['dermatomyositis'] @entity786 :: ('MESH:D003520', 'Chemical') :: ['cyclophosphamide'] @entity358 :: ('MESH:D008171', 'Disease') :: ['lung disease', 'pulmonary deterioration'] @entity401 :: ('MESH:D017563', 'Disease') :: ['ILD', 'interstitial lung disease'] @entity3918 :: ('MESH:D009223', 'Disease') :: ['DM'] @entity6558 :: ('MESH:D017285', 'Disease') :: ['polymyositis']
@entity6017 :: (MESH:D003882,Disease) :: ['dermatomyositis']
