The online version of this article (doi:https://doi.org/10.1186/s13075-017-1506-7) contains supplementary material, which is available to authorized users.
Interstitial lung disease (ILD) is the principal cause of death in polymyositis/dermatomyositis (PM/DM). Here we investigated prognostic factors for death and serious infection in PM/DM-ILD using the multicenter database.
We retrospectively reviewed baseline demographic, clinical and laboratory findings, treatment regimens and outcomes in patients with PM/DM-ILD. The distribution of ILD lesions was evaluated in four divided lung zones of high-resolution computed tomography images.
Of 116 patients with PM/DM-ILD, 14 died within 6 months from the diagnosis. As independent risk factors for early death, extended ILD lesions in upper lung fields (odds ratio (OR) 8.01, p = 0.016) and hypocapnia (OR 6.85, p = 0.038) were identified. Serious infection was found in 38 patients, including 11 patients who died of respiratory or multiple infections. The independent risk factors were high serum KL-6 (OR 3.68, p = 0.027), high initial dose of prednisolone (PSL) (OR 4.18, p = 0.013), and combination immunosuppressive therapies (OR 5.51, p < 0.001).
The present study shows the progression of ILD at baseline is the most critical for survival and that infection, especially respiratory infection, is an additive prognostic factor under the potent immunosuppressive treatment.
Additional file 1: Table S1. The demographic data of patients with PM/DM with ILD. (PDF 37 kb)13075_2017_1506_MOESM1_ESM.pdf
Additional file 2: Table S2. Comparison of demographic data among each PM/DM subtype in PM/DM-ILD patients. (PDF 102 kb)13075_2017_1506_MOESM2_ESM.pdf
Additional file 3: Table S3. Comparison of demographic data between survivors and non-survivors of PM/DM-ILD patients. (PDF 92 kb)13075_2017_1506_MOESM3_ESM.pdf
Additional file 4: Table S4. Comparison of demographic data between PM/DM-ILD patients with or without complication of serious infection. (PDF 92 kb)13075_2017_1506_MOESM4_ESM.pdf
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- The predictive prognostic factors for polymyositis/dermatomyositis-associated interstitial lung disease
- BioMed Central
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