Risk Prediction Modeling in MDA5 Dermatomyositis Save

Patients with Melanoma differentiation-associated gene 5 (MDA5) positive dermatomyositis (DM) are complicated and prone to interstitial lung disease, poor prognosis, and a high mortality. A new study suggests a risk prediction model that may identify of high-risk patients to promote timely diagnosis and treatment.

Studies of anti-MDA5-positive DM have shownan increased risk of rapidly progressive ILD with a high mortality. Yet the diagnosis and is often delayed due to atypical early clinical features.  The current cohort study aimed to identify clinical features, risk factors, treatment strategy, to construct a survival prognosis model for such patients.

The study included 40 anti-MDA5 antibody + DM-ILD patients from 2018-2022 who were retrospectively analyzed. 

Comparison of the survival group and the non-survival group found significant differences in age, oxygenation, lung lesions, diffuse alveolar damage (DAD) and nonspecific interstitial pneumonia (NSIP), and labs (LDH, GLU, CEA, ferritin, CRP levels in serum) (P < 0.05).

Risk factors for a poor prognosis included having NSIP on high-resolution computed tomography (HRCT) with ALT,LDH,CEA,CRP (P < 0.05). 

A nomogram diagram was constructed to predict survival and yielded a higher net benefit compared to other single prognostic factors. The predictive model-dependent variables included treatment style, NSIP, ALT, AST, LDH, CEA, and CRP (with cutoff values). These along with combined treatment (glucocorticoids and immunosuppressants) were protective factor for long-term survival. 

Survival analysis indicated that patients with anti-MDA5 + DM-ILD could benefit from combined treatment for longer survival.

Rapidly progressive disorders like anti-MDA5 antibody + DM may benefit from risk prediction models to allow allow for the early identification of high-risk patients and promote timely diagnosis and treatment.