Mortality risk in patients with anti-MDA5 dermatomyositis is related to rapidly progressive interstitial lung disease and anti-Ro52 antibody

Abstract

Background This study aimed to describe the clinical characteristics and analyze the poor prognostic factors in patients with anti-MDA5 dermatomyositis. Methods A total of 126 adults with anti-MDA5 dermatomyositis were enrolled in this retrospective study. Information on survival time, cause of death, and baseline characteristics was collected. Patients were divided into two groups: a survival group and a non-survival group. Items with clinical significance that showed significant differences between the two groups were screened by Kaplan–Meier and Cox regression analyses to identify the predictors of poor survival. Results Thirty-two patients were included in the non-survival group, most of whom died from respiratory failure, with pulmonary infection accounting for half. Epstein–Barr virus infection was relatively common in both groups. Aspartate transaminase, lactate dehydrogenase, and ferritin levels; erythrocyte sedimentation rate; and anti-Ro52 antibody levels were significantly higher, while the lymphocyte count was lower in the non-survival group compared with the survival group. Notably, patients in the non-survival group were more likely to present with rapidly progressive interstitial lung disease than those in the survival group. Kaplan–Meier and Cox multivariate regression analyses revealed that the prevalence of rapidly progressive interstitial lung disease, levels of anti-Ro52 antibody, and age > 57 years were important prognostic factors independent of multiple clinical parameters. Conclusions Rapidly progressive interstitial lung disease, anti-Ro52 antibody levels, and age > 57 years are possible predictors of mortality risk in patients with anti-MDA5 dermatomyositis.

MDA5-autoimmunity and Interstitial Pneumonitis Contemporaneous with the COVID-19 Pandemic (MIP-C)

“…Our study is the largest one to document the features and outcomes of this clinical syndrome, especially in 2021. Approximately 42% of our MDA5+ cases have thus far had progressive ILD, with a third of these proving fatal so far, in keeping with the known aggressive course of MDA5 + -ILD 48,49 . Our clinical epidemiologic observations, together with the transcriptomic analyses suggest that increased incidence of MDA5 autoimmunity and ILD that presented contemporaneously during COVID-19 could be due to an abberant type 1-centric IFN responses that are shared with autoimmune ILD, but not IPF, which plays out across diverse cell types leading to severe ILD ( Figure 6 ).…”

Assessing Time of Onset for Interstitial Lung Disease in Anti-MDA5 Antibody-Positive Dermatomyositis

“…MDA5 antibody-positive DM is characterized by its association with interstitial lung disease (ILD) categorized into 2 types: rapidly progressive ILD (RPILD) and chronic ILD (CILD). [1][2][3] RPILD in patients with MDA5 antibody-positive DM has received considerable attention due to its high mortality rate. RPILD is defined as worsening dyspnea accompanied by radiographic changes within 3 months of initial pulmonary symptom onset.…”

“…RPILD is defined as worsening dyspnea accompanied by radiographic changes within 3 months of initial pulmonary symptom onset. [1][2][3] However, the time interval between ILD and MDA5 antibody-positive DM diagnoses has not been well described, as most studies look at incidence or mortality rates vs time of onset. 1,2 Methods | We conducted a retrospective cohort study using electronic medical records from 2008 to 2023 for patients of the Department of Dermatology at the University of Miami.…”

“…Research suggests that short intervals (≤1.16 months) between MDA5 antibody-positive DM and ILD diagnoses indicate poorer prognosis. 3 High-resolution computed tomography showing nonspecific interstitial pneumonia and diffuse alveolar damage may also be associated with poorer prognosis. 4 Therefore, timely imaging in patients with MDA5 antibody-positive DM is pertinent for accurate classification of RPILD/CILD and treatment.…”

“…RPILD in patients with MDA5 antibody-positive DM has received considerable attention due to its high mortality rate. RPILD is defined as worsening dyspnea accompanied by radiographic changes within 3 months of initial pulmonary symptom onset . However, the time interval between ILD and MDA5 antibody-positive DM diagnoses has not been well described, as most studies look at incidence or mortality rates vs time of onset …”