0171: Cardiac Involvement in Idiopathic Inflammatory Myopathies: When Should We Look for It?

Matilde Bandeira¹, Eduardo Dourado¹, Ana Teresa Melo¹, Patricia Martins², Vanessa Fraga³, José Luís Ferraro⁴, André Saraiva⁵, Marlene Sousa⁶, Hugo Parente⁷, Catarina Soares⁸, Ana Margarida Correia⁹, Diogo Esperança Almeida⁹, Sara Paiva Dinis¹⁰, Ana Pinto¹¹, Filipe Pinheiro¹², Maria Rato¹², Tiago Beirão¹³, Beatriz Samões¹³, Bernardo Santos¹⁴, Carolina Mazeda¹⁵, Ana Teodósio Chícharo¹⁶, Margarida Faria¹⁷, Agna Neto¹⁷, Helena Lourenço¹⁸, Luísa Brites¹⁹, Marília Rodrigues¹⁹, Joana Silva-Dinis²⁰, João Madruga-Dias²¹, Filipe Araújo²², Nádia Martins²³, Maura Couto²³, Ana Valido²⁴, Maria José Santos²⁵, Sofia Barreira¹, João Eurico Fonseca¹ and Raquel Campanilho-Marques¹, ¹Serviço de Reumatologia, Centro Hospitalar Universitário Lisboa Norte, Centro Académico de Medicina de Lisboa (CAML), Lisboa, Portugal; Unidade de Investigação em Reumatologia, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, CAML, Lisboa, Portugal, ²Rheumatology department, Centro Hospitalar e Universitário de Lisboa Norte, Centro Académico de Medicina de Lisboa, Lisboa, Portugal, ³Serviço de Reumatologia, Hospital Beatriz Ângelo, Almada, Portugal, ⁴Serviço de Reumatologia, Hospital Beatriz Ângelo, Loures, Portugal, ⁵Rheumatology department, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal, ⁶Serviço de Reumatologia, Centro Hospitalar Universitário de Coimbra, Coimbra, Portugal, ⁷Rheumatology department, Unidade Local de Saúde do Alto Minho, Ponte de Lima, Portugal, ⁸Rheumatology department, Unidade Local de Saúde do Alto Minho, Ponte de Lima, Viana do Castelo, Portugal, ⁹Serviço de Reumatologia, Hospital de Braga, Braga, Portugal, Braga, Portugal, ¹⁰Rheumatology department, Unidade Local de Saúde da Guarda - Hospital Sousa Martins, Guarda, Portugal, ¹¹Local Health Unit of Guarda, Barcelos, Portugal, ¹²Rheumatology Department, Centro Hospitalar Universitário de São João, Porto, Portugal, ¹³Rheumatology Department, Centro Hospitalar de Vila Nova de Gaia / Espinho, Vila Nova de Gaia, Portugal, ¹⁴Serviço de Reumatologia, Centro Hospitalar do Baixo Vouga, Aveiro, Portugal, ¹⁵Centro Hospitalar Baixo Vouga, Ovar, Portugal, ¹⁶Serviço de Reumatologia, Centro Hospitalar Universitário do Algarve, Faro, Portugal, ¹⁷Serviço de Reumatologia, Hospital Nélio Mendonça, Serviços de Saúde da Região Autónoma da Madeira, Funchal, Portugal, ¹⁸Serviço de Reumatologia, Centro Hospitalar de Lisboa Ocidental, Lisboa, Portugal, ¹⁹Serviço de Reumatologia, Centro Hospitalar de Leiria, Leiria, Portugal, Leiria, Portugal, ²⁰Serviço de Reumatologia, Centro Hospitalar Universitário de Lisboa Central, Lisboa, Portugal, ²¹Rheumatology Service, Centro Hospitalar Médio Tejo, Tomar, Portugal, ²²Rheumatology and Osteoporosis Unit, Hospital de Sant´Ana, SCML, Lisboa, Portugal, ²³Rheumatology department, Centro Hospitalar Tondela-Viseu, Viseu, Portugal, ²⁴Serviço de Reumatologia, Unidade Local de Saúde do Litoral Alentejano, Santiago do Cacém, Portugal, ²⁵Hospital Garcia de Orta, Almada, Charneca da Caparica, Portugal

Background/Purpose: Idiopathic inflammatory myopathies (IIM) are a group of rare disorders that can affect the heart. It is unclear which patients are at higher risk. Anti-signal recognition particle (anti-SRP) antibody has been inconsistently suggested as a risk factor. This work aims to find predictors of heart involvement in IIM.

Methods: Multicenter open cohort study, including patients registered in the IIM module of the Rheumatic Diseases Portuguese Register (Reuma.pt/Myositis) until January 2022. Patients without heart involvement information were excluded. Myo(peri)carditis, dilated cardiomyopathy, conduction abnormalities and/or premature coronary artery disease were considered. Data from continuous variables are presented as mean ± standard deviation. Univariate analysis was performed using chi-square, Fisher's exact, Mann-Whitney or t-test, as appropriate. Independent predictors of cardiac involvement, adjusted for sex and age at diagnosis, were identified through binomial logistic regression. The linearity of the continuous variables was assessed via the Box-Tidwell procedure. Correlated variables, cases with missing information and outliers were excluded from the multivariate analysis to assure the validity of the regression.

Results: 229 patients were included, 163 (71.2%) females. Patients were classified as having definite [56/115 (48.7%)], likely [23/115 (20.0%)], or possible [2/115 (1.7%)] IIM by 2017 EULAR/ACR classification criteria. Cardiac involvement was present in 11 (4.8%) patients (Figure 1), of whom 42.9% were classified as likely and 57.1% as definite IIM. The mean age at disease onset was 47.1 ± 19.0 years, the mean age at diagnosis was 48.6 ± 18.7 years, and the mean disease duration at the last follow-up was 7.0 ± 6.6 years. Compared to other IIM patients (Table 1), patients with cardiac involvement were older at disease onset (57.6 ± 11.9 vs 46.6 ± 19.2 years, p=0.035) and diagnosis (58.3 ± 12.2 vs 48.0 ± 18.9 years, p=0.049), and had a shorter disease duration at the last follow-up (3.9 ± 4.1 vs 7.2 ± 6.7 years, p=0.019). Clinically, patients with cardiac involvement had a lower manual muscle testing score (MMT), comparing the lowest value throughout follow-up, and more frequently had esophageal [5/10 (50.0%) vs 34/208 (16.3%), p=0.018] and lung involvement [9/11 (81.8%) vs 68/217 (31.3%), p=0.001]. Conversely, this group less frequently had Gottron's sign [1/10 (10.0%) vs 68/153 (44.4%), p=0.045]. Anti-SRP antibodies were more commonly identified in patients with cardiac involvement [3/9 (33.3%) vs 10/175 (5.7%), p=0.018]. No differences were found between the two groups for other demographical or clinical data or serum biomarkers. In the multivariate analysis, lung involvement (OR 7.064, 95%CI: 1.246-40.057, p=0.027) and positivity of anti-SRP antibodies (OR 7.886, 95%CI: 1.333-46.666, p=0.023) were confirmed as independent predictors of heart involvement in IIM patients, regardless of sex and age at diagnosis.

Conclusion: Lung involvement and anti-SRP antibodies were independent predictors of heart involvement in our cohort of IIM patients. We suggest considering a closer screening for heart involvement in these patients.Table 1 – Clinical and serological features of patients with and without heart involvement

Figure 1 – Types of heart involvement in our cohort. Some patients had more than one manifestation
Disclosures: M. Bandeira, None; E. Dourado, None; A. Melo, None; P. Martins, None; V. Fraga, None; J. Ferraro, None; A. Saraiva, None; M. Sousa, None; H. Parente, None; C. Soares, None; A. Correia, None; D. Esperança Almeida, None; S. Paiva Dinis, None; A. Pinto, None; F. Pinheiro, None; M. Rato, None; T. Beirão, None; B. Samões, None; B. Santos, None; C. Mazeda, None; A. Teodósio Chícharo, None; M. Faria, None; A. Neto, None; H. Lourenço, None; L. Brites, None; M. Rodrigues, None; J. Silva-Dinis, None; J. Madruga-Dias, None; F. Araújo, None; N. Martins, None; M. Couto, None; A. Valido, None; M. Santos, AbbVie/Abbott, AstraZeneca, pfizer, Novartis, Eli Lilly; S. Barreira, None; J. Fonseca, AbbVie/Abbott, Biogen, Bristol-Myers Squibb(BMS), Janssen, Eli Lilly, Medac, Merck/MSD, Novartis, Pfizer, UCB; R. Campanilho-Marques, None.