Clinical features of anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis with macrophage activation syndrome
Authors' conclusion
Among anti-MDA5-positive DM, Infection, DM typical rashes, and higher serum levels of ferritin, AST, LDH, and CK were more common in patients complicated with MAS. The mortality of patients with MAS was high, particularly among patients who were older, given more combination therapy with TOF, and had RP-ILD, infection, heart failure and renal impairment.
Study context
- Population
- Patients with anti-MDA5 antibody-positive dermatomyositis (DM)
- Participants
- 44 patients
- Comparison
- Anti-MDA5-positive DM with macrophage activation syndrome (MAS) vs without MAS
- MASn = 11
- Without MASn = 33reference
- Outcome
- Clinical features of anti-MDA5-positive DM with MAS
Methodological detailscontrols selected randomly in the same year at a ratio of 3:1
- Controls selected randomly in the same year at a ratio of 3:1
Main finding
As reported in the abstract
Clinical features, MAS vs without MAS
- MAS had significantly higher incidence of infection, heliotrope sign, Gottron's papule, V-neck sign, and higher serum ferritin, AST, lactic dehydrogenase (LDH) and creatine kinase (CK) than without MAS
- p<0.05
Observational data: associations, not effects.
Further findings
- Mortality among patients with MAS
- 36.4% · 4 of 11
Features of non-survivors vs survivors with MAS
- Deceased patients were older, given more combination therapy with TOF and had higher incidence of RP-ILD, infection, HF and renal impairment than survivors
- P < 0.05
Original publication
Clinical features of anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis with macrophage activation syndrome. Clin Exp Rheumatol. Published 24 August 2023. doi:10.
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