Myocardial involvement is not rare in anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis/clinically amyopathic dermatomyositis: a retrospective study

Abstract

ObjectivesStudies concerning myocardial involvement (MI) in patients with anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis/clinically amyopathic dermatomyositis (anti-MDA5 Ab+ DM/CADM) are scarce. We aimed to characterize MI in our anti-MDA5 Ab+ DM/CADM cohort and to investigate its association with prognosis.MethodsIn this single-center retrospective study, anti-MDA5 Ab+ hospitalized DM/CADM patients who underwent transthoracic echocardiography (TTE) were enrolled. Myocardial involvement was diagnosed according to abnormal cardiac structure and function detected by TEE. Clinical features and cardiac examination findings of patients with MI were analyzed. Clinical features, laboratory findings, complications, and treatments were compared between MI and non-MI, deceased, and survival patients. Logistic regression analysis was used to explore the independent risk factors for the occurrence of MI and prognostic factors for these patients.ResultsSeventy-six hospitalized patients with anti-MDA5 Ab+ DM/CADM were enrolled. Twelve (15.8%) patients were diagnosed with MI. Of the 12 patients, three underwent cardiac magnetic resonance imaging (CMR) and late gadolinium enhancement (LGE) were noted for them. TEE revealed that eight (66.7%) patients had left atrial and/or ventricular enlargement, three (25.0%) had cardiac hypertrophy, six (50.0%) had diffuse ventricular wall dyskinesia, and seven (58.3%) had diastolic dysfunction. Six (50.0%) patients with MI developed heart failure (HF) during treatment. Of the 12 patients, one patient died of HF caused by myocarditis, three died of infection, and four died of exacerbation of rapidly progressive interstitial lung disease (RP-ILD). Logistic regression analysis revealed that dysphagia (OR 3.923, 95% CI 1.085, 14.181), NT-proBNP >600 pg/ml (OR 18.333, 95% CI 1.508, 222.875), and increased peripheral white blood cells (OR 1.201, 95% CI 1.003, 1.438) were risk factors for the occurrence of MI, but plasma albumin (OR 0.892, 95% CI 0.796, 0.999) was a protective factor. Both MI (OR 5.984, 95% CI 1.174, 30.496) and RP-ILD (OR 11.875, 95% CI 2.796, 50.411) were independent risk factors for the mortality of these anti-MDA5 Ab+ DM/CADM patients.ConclusionMyocardial involvement is not rare and is an independent poor prognostic factor of anti-MDA5 Ab+ DM/CADM patients. Cardiac abnormality screening is necessary for them.

An externally validated clinical-laboratory nomogram for myocardial involvement in adult idiopathic-inflammatory-myopathy patients

“…The correlations between positivity of age, AMAs, LDH and MI have previously been reported [ 13 – 15 , 38 ]. Elderly age was found associated with complication of carcinoma, infection, MI, ILD, etc.…”

“…By our observation, the frequency of anti-MDA5 antibody was comparably higher in different cohorts in southern China than those in northern China. The frequent development of RP-ILD, the scarce but fatal secondary hemophagocytic lymphohistiocytosis (sHLH), occurrence of opportunistic infection, involvement of pharyngeal and respiratory muscle, all contributed to the unfavorable outcome in these patients [ 15 , 32 – 34 ]. Previously patients with positivity of anti-SRP antibody were reported to be vulnerable to cardiac involvement [ 35 ].…”

“…In 2022, Zeng, etc. reminded researchers and clinicians of the complication of MI in anti-MDA5 patients (15.8%) [ 15 ]. With more anti-MDA5 patients in our cohort, incidence of MI in anti-MDA5 patients was even higher (28.7%).…”

“…In addition, positivity of anti-MDA5 antibody was also recognized as a risk factor for MI. In preceding studies, MDA5 seems to play a protective role in acute viral myocarditis since MDA5 is an RNA sensor that can induce transcription of type I IFN genes and thus defense against viruses [ 15 ]. However, in ischemic/reperfusion-injury and myocardial-infarction model, activation of type I IFN pathway aggravates myocardial injury [ 36 , 37 ].…”

“…Due to different populations and diagnostic criteria, the incidence of MI in IIMs patients ranged from 5.5% to over 50%, which is frequently subclinical in its initial stage [ 11 – 13 ]. In preceding studies, multiple clinical and laboratory factors have been identified as risk factors for MI [ 13 – 15 ]. Besides, new imaging tools have been used for early identification [ 16 , 17 ].…”