RESUMEN
OBJECTIVES: This study aimed to evaluate the clinical significance of pleural effusion in adult patients with idiopathic inflammatory myopathies (IIM). METHODS: We assessed a cohort of 158 consecutive patients with IIM. Clinical features and survival rates were compared between patients with and without pleural effusion. RESULTS: Of those 158 IIM patients, 28 (17.7%) developed pleural effusion. 125 (79.1%) IIM patients had interstitial lung disease (ILD), 26 (20.8%) of which developed pleural effusion. Notably, pleural effusion was associated with a higher incidence of lower lung zone consolidation, rapidly progressive ILD (RP-ILD) and elevated high-resolution computed tomography (HRCT) score, and could robustly predict RP-ILD independently [HR 7.863 (2.160-28.617), p=0.002] in IIM-ILD patients. IIM patients with pleural effusion presented with increased systemic inflammatory response, including more fever, elevated white blood cell count, neutrophil/lymphocyte ratio, C-reactive protein, and erythrocyte sedimentation rate, alongside reduced lymphocyte percentage. Pleural effusion was also associated with more ILD, lower lung zone consolidation, pericardial effusion and RP-ILD, higher HRCT score, and lower HB and albumin levels in IIM. Except for neutrophil/lymphocyte ratio, ILD and pericardial effusion, other correlative variables were potential predictors of higher mortality in IIM. Furthermore, pleural effusion remained an independent predictor of higher mortality in IIM [HR 5.05 (1.633-15.62), p=0.005]. CONCLUSIONS: Pleural effusion showed a significant positive association with severe phenotypes of ILD and was the powerful predictor of RP-ILD in IIM-ILD. Furthermore, pleural effusion could reveal adverse disease phenotypes with higher systemic inflammatory level and predict higher mortality independently in IIM.
RESUMEN
OBJECTIVES: This study aimed to evaluate the clinical significance of the coexistence of 2 or more myositis-specific antibodies (multiple MSAs) in adult patients with idiopathic inflammatory myopathies (IIM). METHODS: We assessed a cohort of 202 consecutive patients with IIM. Clinical features and survival rates were compared between patients with and without multiple MSAs. RESULTS: Of those 202 patients, 44 (21.8%) were found to have multiple MSAs. 63.6% of the 44 patients tested positive for anti-aminoacyl-tRNA synthetase antibodies (anti-ARS+) and 52.3% positive for anti-melanoma differentiation-associated protein-5 antibody (anti-MDA5+). The presence of multiple MSAs was associated with less rapidly progressive interstitial lung disease (RP-ILD), fever, rash, periungual erythema, more muscle involvement and dysphagia, higher albumin level, and higher positive rate of ANA antibody in anti-MDA5+ population. In anti-ARS+ population with multiple MSAs, there were more V-neck sign, skin ulcers, dysphagia and peripheral edema. No differences in survival rates were observed between patients with or without multiple MSAs in the overall and anti-ARS+ populations. However, the survival rate in anti-MDA5+ population with multiple MSAs was significantly higher than those without multiple MSAs (p = 0.003). Moreover, multiple MSAs remained an independent protective factor against mortality in multivariable Cox regression analysis of anti-MDA5+ population [HR 0.108 (95% CI 0.013, 0.908), p=0.041]. CONCLUSIONS: Multiple MSAs coexist in some IIM patients and their existence indicates mixed features from concomitant MSAs in anti-MDA5+ population and anti-ARS+ population. Identifying multiple MSAs could help to discover a more favourable disease phenotype with decreased mortality in anti-MDA5+ population.
