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Hyperkeratotic ulcerating papules on the dorsal metacarpophalangeal joints. Ulcerating papules on lateral aspects of digits.
Citations
... Documented cardiac manifestations in IIM patients are diverse including myocardial ischemia, arrhythmias, conduction defects, cardiomyopathies, pericardium diseases, and pulmonary hypertension (5), whereas reports of cardiac involvement in anti-MDA5 Ab+ dermatomyositis/clinically amyopathic dermatomyositis (DM/CADM) patients are scarce. In the past decades, only three cases of anti-MDA5 Ab+ DM patients with severe myocardial defects have been reported (6)(7)(8). A recent study revealing distinctive electrocardiography (ECG) changes in anti-MDA5 Ab+ DM/ CADM patients (9) further indicates cardiac involvement of anti-MDA5 Ab+ DM/CADM patients. ...
Objectives
Studies 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.
Methods
In 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.
Results
Seventy-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.
Conclusion
Myocardial 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.
Dear Editor, Anti-melanoma differentiation–associated gene 5 (anti-MDA5) antibody (Ab) is a myositis-specific autoantibody closely associated with clinically amyopathic DM with interstitial lung disease (ILD). Although myocarditis associated with DM is common, severe myocarditis is rarely reported. We report a case of anti-MDA5 Ab–positive DM with fatal myocarditis expressing myxovirus-resistance protein 1 (MxA).
A 48-year-old man started experiencing rashes, stiff fingers, and MCP joint pain 6 months before admission to our department. Eyelid oedema, proximal limb muscle weakness and pain, and shortness of breath on exertion occurred subsequently. On admission, typical skin manifestations with DM were noted, including a heliotropic rash on the upper eyelids; Gottron’s and palmar papules on the finger joints (Fig. 1A and B); and multiple excavating skin ulcers with necrotic tissues reaching the s.c. tissues in the shoulders, elbows (Fig. 1C), finger joints, left buttock, and scrotum. The upper and lower limb proximal muscle strength decreased. Though the serum creatine kinase and aldolase values were normal, MRI revealed a widespread increase in the signals in the upper and lower limb proximal muscles, suggesting myopathy. Blood examination revealed seropositivity for anti-MDA5 Abs (5140 index, normal: <32 index, measured by ELISA) and seronegativity for AMA, PR3-ANCA and MPO-ANCA. Increased serum levels of ferritin (5380 ng/ml, normal: 13–277 ng/ml), Krebs von den Lungen-6 (KL-6; 2380 U/ml, normal: 105–401 U/ml), and brain natriuretic peptide (BNP; 373 pg/ml, normal: <40 pg/ml) were noted. Skin biopsy of the erythema on the shoulder showed interface dermatitis without vasculitis. CT revealed moderate ILD.
