Time-dependent changes in RPILD and mortality risk in anti-MDA5+ DM patients: a cohort study of 272 cases in China.

Publication date: Aug 12, 2022

Anti-melanoma differentiation-associated gene 5 positive (anti-MDA5+) dermatomyositis (DM) has a close relationship with rapidly progressive interstitial lung disease (RPILD) and is associated with high mortality. However, data regarding the time-dependent risk of RPILD and deaths during disease progression are limited. We conducted this study to investigate whether the risk of RPILD and death were time-dependent or not in anti-MDA5+ DM. We assessed a cohort of 272 patients with anti-MDA5+ DM. The clinical characteristics of patients with anti-MDA5+ were collected, and COX regression was used to analyze independent risk factors for RPILD and death. We also described changes in risk of RPILD and death over time and their potential clinical implications. There were 272 anti-MDA5+ DM patients enrolled in this study. According to the multivariate cox regression analysis, short disease course, high CRP level, anti-Ro52 positive, and anti-MDA5 titer ( ++∼ +++) were independent risk factors of RPILD. High CK level, high CRP level, and RPILD were independent risk factors for death. >90% RPILD and 84% mortality occurred in the first 6 months after disease onset. Notably, the first 3-months is a particularly high-risk period, with 50% RPILD and 46% death occurring. Hazards regarding RPILD and mortality diminished over time during a median follow-up of 12 months. These results suggest significant, time-dependent changes in RPILD and mortality risk in anti-MDA5+ DM patients, providing a cutoff time window to estimate disease progression and poor prognosis.

Concepts Keywords
China Medicine
Dermatomyositis Medical specialties
Months Clinical medicine
Oxford Intracellular receptors
Prognosis MDA5
Autoimmune diseases
Connective tissue diseases
RTT
C-reactive protein
Dermatomyositis

Semantics

Type Source Name
disease MESH melanoma
pathway KEGG Melanoma
disease MESH dermatomyositis
disease MESH interstitial lung disease
disease MESH disease progression
disease MESH death

Original Article

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