Clinical manifestations, outcomes, and antibody profile of Brazilian adult patients with dermatomyositis: a single-center longitudinal study

Author(s)

Carboni Truzzi, Natassia Cristina ; Hoff, Leonardo Santos ; Pires Borges, Isabela Bruna ; Carlos de Souza, Fernando Henrique ; Shinjo, Samuel Katsuyuki

• Document type: Journal article
• Source: ADVANCES IN RHEUMATOLOGY; v. 62, n. 1, p. 9-pg., 2022-11-12.

Abstract

Background Currently, only a few retrospective cohort or cross-sectional studies have described the general characteristics of Brazilian patients with classical dermatomyositis (DM). In contrast, we aimed to longitudinally assess a large sample of these patients, and several myositis autoantibodies. Methods This single-center longitudinal study included 91 Brazilian adults with defined DM (EULAR/ACR 2017) who underwent follow-up appointments in our tertiary center from 2012 to 2021. Myositis autoantibody analysis was performed using a commercial kit. Results The mean age of the patients was 47.3 +/- 15.4 years, with a predominance of female (67.0%) and White (81.3%) patients. As an initial treatment, 76.9% of the patients received methylprednisolone pulse therapy, 59.3% received intravenous immunoglobulin, and 54.9% received both drugs. The median follow-up duration was 44 months (interquartile 17-67) months. There were 92 severe episodes of infection, and neoplasms were identified in 20 patients (22.0%). Hypertension was identified in 46.2% of patients, whereas diabetes mellitus and myocardial infarction occurred in 19.8% and 4.4%, respectively. Nine patients died during the follow-up. At the last visit, one-third of the patients had disease activity, half had a complete clinical response, and one-fifth were in disease remission. In a univariate logistic regression, anti-aminoacyl-tRNA synthetase antibodies (n = 13) were associated with interstitial lung disease, "mechanic's hands", and anti-Ro-52, and had an inverse association with "V"-neck and "shawl" signs. Anti-MDA-5 (n = 10) were associated with male gender, digital ulcers, vasculitis, arthritis, anti-Ro-52, and active disease. Anti-Ro-52 (n = 26) were associated with "mechanics' hands", arthritis, interstitial lung disease, anti-tRNA synthetases, and anti-MDA-5. No association was found for anti-Mi-2 (n = 10). Conclusions This study shows the general profile of a significant sample of Brazilian patients with DM as well as the association of some antibodies with clinical and laboratory manifestations of this myositis. (AU)

• FAPESP's process: 21/04892-0 - Prevalence and reactivity of the myositis-specific autoantibodies (anti-TIF-1g, anti-NXP-2 and anti-SAE) in adult patients with dermatomyositis: a prospective cohort sutdy
• Grantee: Natássia Cristina Carboni Truzzi
• Support Opportunities: Scholarships in Brazil - Scientific Initiation
• FAPESP's process: 19/11776-6 - Impact and safety of noninvasive cerebral neuromodulation in patients with systemic autoimmune diseases: double-blind, randomized, placebo-controlled study
• Grantee: Samuel Katsuyuki Shinjo
• Support Opportunities: Regular Research Grants
• FAPESP's process: 19/11367-9 - Molecular and metabolic analysis of Atorvastatin in the muscle tissues of patients with Systemic Autoimmune Myopathies
• Grantee: Isabela Bruna Pires Borges
• Support Opportunities: Scholarships in Brazil - Doctorate