Objective To explore the clinical and serological features of patients with pneumom-diastinum (PNM) and dermatomyositis associated interstitial lung disease (DM-ILD). Methods A total of 145 polymyositis/dermatomyositis (PM/DM) patients hospitalized in our department from March, 2010 to December, 2012 were recruited. The sera, clinical and laboratory data were collected. Anti-aminoacyl-tRNA synthetase (anti-ARS) and anti-melanoma differentiation-associated gene 5 (MDA5) antibodies were detected by RNA-immunoprecipitation (RNA-IP) and ELISA, respectively. Anti-NXP2, anti-TIF1γ, anti-SRP, anti-SAE and anti-Mi2 antibodies were detected by immunoprecipitation-Western blotting. Variables were compared between DM patients with and without PNM, as well as between DM-ILD patients with and without PNM. Chi square test, Fisher's exact test, t test, Mann-Whitney U test and a multivariate logistic regression model were used for statistical analysis. Results A total of 11 DM patients (10 patients positive for anti-MDA5 and 1 patient positive for anti-Mi2) developed spontaneous PNM. No PM patient developed PNM. No differences of sex, age at the onset of DM, serum ferritin levels and C reactive protein (CRP) levels was observed between DM patients with and without PNM. Compared with DM patients without PNM, DM patients with PNM had significantly higher frequencies of rapidly progressive ILD (RP-ILD) (63.6% vs 24.4%, χ2=7.25, P=0.01), anti-MDA5 antibodies (90.9% vs 52.4%, χ2=5.86, P=0.02), clinically amyopathic DM (CADM) (63.6% vs 22.0%, χ2=8.57, P=0.007) and cutaneous ulcers (36.4% vs 11%, χ2=5.20, P=0.04), but significantly lower creatine kinase (CK) levels [58.5 (30.5, 394.3) U/L vs 284 (73.0, 917.0) U/L, t=207.5, P=0.04]. The logistic multivariate analysis indicated that cutaneous ulcer was the only independent risk factor for the occurrence of PNM in DM [OR=5.98, 95% confidence interval (CI) (1.12, 31.98), P=0.037]. In the comparisons between DM-ILD patients with and without PNM, a higher frequency of CADM was observed in the PNM group (63.6% vs 27.9%, χ2=5.37, P=0.03), but no significant difference of the frequencies of RP-ILD, anti-MDA5 antibodies, cutaneous ulcers and CK levels was found in these two groups. All patients with PNM were treated with corticosteroids and immunosuppressants. During the follow-up period, 6 patients died of respiratory failure. Conclusion Spontaneous PNM is a refractory complication with poor prognosis, and tends to occur in DM patients with RP-ILD, anti-MDA5 antibody, CADM diagnosis and low CK level, especially in patients with cutaneous ulcers. Key words: Dermatomyositis; Lung diseases, interstitial; Mediastinal emphysema
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