Cuirong Ba, Chunguo Jiang, Yanli Gao, Qiuhong Fang
Patients hospitalized with DM/PM-AEILD suffer high in-hospital mortality, which is further elevated in anti-MDA5 Ab+ individuals. Reduced CD4⁺T lymphocyte count, anti-MDA5 Ab+, pleural effusion, elevated N%, and elevated CRP are independent adverse prognostic factors. Decreased CD4⁺ T lymphocyte count also predicts poor prognosis in patients with anti-MDA5 Ab+ DM-AEILD. Key Points • In DM/PM-AEILD hospitalizations, the in-hospital mortality rate was 42.9%; their symptoms exhibit characteristics of both AEILD and DM/PM (Beijing Chao-yang Hospital Inpatient Sample 2017.1-2022.6). • CD4⁺ T lymphocyte count, anti-MDA5 Ab⁺, pleural effusion, N%, and CRP independently predict in-hospital death, among which CD4⁺ T lymphocyte count has the highest predictive value. • Lower CD4⁺ T lymphocyte count indicates worse clinical outcomes in patients with anti-MDA5 Ab⁺ DM-AEILD.
INTRODUCTION/OBJECTIVES: Acute exacerbation of interstitial lung disease secondary to dermatomyositis or polymyositis (DM/PM-AEILD) is an intractable, life-threatening disease with unsatisfactory therapeutic efficacy, particularly in patients positive for anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody (Ab). Identifying prognostic risk factors for DM/PM-AEILD might help clinicians to stratify high-risk patients at an early stage and deliver targeted standardized treatment.
METHODS: We retrospectively collected clinical data of 35 hospitalized patients diagnosed with DM/PM-AEILD, including 18 patients with anti-MDA5-Ab+ DM-AEILD. Clinical indicators were compared between survivors and non-survivors. Binary multivariate logistic regression was performed to screen independent risk factors for in-hospital mortality, and receiver operating characteristic (ROC) curves were drawn to evaluate predictive efficacy. Subgroup analyses were repeated for patients with anti-MDA5-Ab+ DM-AEILD.
RESULTS: The cohort consisted of 21 females and 14 males; the mean age was 62.11 ± 10.78 years. The overall in-hospital mortality of DM/PM-AEILD reached 42.9%, and the mortality climbed to 66.7% among anti-MDA5 Ab+ subgroup. The clinical symptoms of DM/PM-AE-ILD were dominated by typical AE-ILD symptoms such as cough and respiratory failure. Peripheral blood CD4+T lymphocyte count exhibited the strongest predictive capacity for in-hospital death (OR = 0.987, cut-off = 373 cells/µL, AUC = 0.933). Model B combining pleural effusion, neutrophil percentage (N%), and C-reactive protein (CRP) ranked second (AUC = 0.923), followed by Model A integrating anti-MDA5 Ab+ and N% (AUC = 0.893). Decreased CD4+T lymphocyte count also presented favorable predictive performance for adverse prognosis in anti-MDA5 Ab+ DM-AEILD subgroup (OR = 0.990, AUC = 0.847).
CONCLUSIONS: Patients hospitalized with DM/PM-AEILD suffer high in-hospital mortality, which is further elevated in anti-MDA5 Ab+ individuals. Reduced CD4⁺T lymphocyte count, anti-MDA5 Ab+, pleural effusion, elevated N%, and elevated CRP are independent adverse prognostic factors. Decreased CD4⁺ T lymphocyte count also predicts poor prognosis in patients with anti-MDA5 Ab+ DM-AEILD. Key Points • In DM/PM-AEILD hospitalizations, the in-hospital mortality rate was 42.9%; their symptoms exhibit characteristics of both AEILD and DM/PM (Beijing Chao-yang Hospital Inpatient Sample 2017.1-2022.6). • CD4⁺ T lymphocyte count, anti-MDA5 Ab⁺, pleural effusion, N%, and CRP independently predict in-hospital death, among which CD4⁺ T lymphocyte count has the highest predictive value. • Lower CD4⁺ T lymphocyte count indicates worse clinical outcomes in patients with anti-MDA5 Ab⁺ DM-AEILD.