Myocarditis in anti-synthetase syndrome: clinical features and diagnostic modalities

Giacomo De Luca; Corrado Campochiaro; Anna Palmisano; Elisa Bruno; Davide Vignale; Giovanni Peretto; Simone Sala; Arianna Ferlito; Maria Bernardette Cilona; Antonio Esposito; Marco Matucci-Cerinic; Lorenzo Dagna

doi:10.1093/rheumatology/kead541

Myocarditis in anti-synthetase syndrome: clinical features and diagnostic modalities

Rheumatology (Oxford). 2023 Oct 5:kead541. doi: 10.1093/rheumatology/kead541. Online ahead of print.

Authors

Giacomo De Luca^{1

2}, Corrado Campochiaro^{1

2}, Anna Palmisano^{2

3}, Elisa Bruno^{2

3}, Davide Vignale^{2

3}, Giovanni Peretto⁴, Simone Sala⁴, Arianna Ferlito^{1

2}, Maria Bernardette Cilona^{1

2}, Antonio Esposito^{2

3}, Marco Matucci-Cerinic¹, Lorenzo Dagna^{1

2}

Affiliations

¹ Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Hospital, Milan, Italy.
² School of Medicine, Vita-Salute San Raffaele University, Milan, Italy.
³ Clinical and Experimental Radiology Unit, Experimental Imaging Center, IRCCS San Raffaele Hospital, Milan, Italy.
⁴ Department of Cardiac Electrophysiology and Arrhythmology, IRCCS San Raffaele Hospital and University, Milan, Italy.

PMID: 37796832
DOI: 10.1093/rheumatology/kead541

Abstract

Objectives: Myocarditis is an overlooked manifestation of anti-synthetase syndrome (ASS). Our study describes the clinical and instrumental features of ASS-myocarditis and evaluates the diagnostic performance of cardiac magnetic resonance (CMR) with mapping techniques.

Methods: Data from ASS-patients were retrospectively analyzed. CMR data of patients diagnosed with myocarditis, including late gadolinium enhancement (LGE), T2-ratio, T1-mapping, extra-cellular volume (ECV) and T2-mapping, were reviewed. Myocarditis was defined by the presence of symptoms of heart involvement with increased high-sensitive troponin T (hs-TnT) and/or NT-proBNP and at least an instrumental abnormality. Clinical features of ASS patients with and without myocarditis were compared. A p value<0.05 was considered.

Results: Among a cohort of 43 ASS-patients (median age 58[48.0-66.0] years; females 74.4%; anti-Jo1 53.5%), 13(30%) were diagnosed with myocarditis. In 54% of patients, myocarditis was diagnosed at clinical onset. All ASS-myocarditis patients had at least one CMR abnormality: increased ECV in all cases, presence of LGE, increased T1 and T2-mapping in 91%. The 2009-Lake Louis criteria (LLC) were satisfied by 6 patients, the 2018-LLC by 10. With the updated LLC, the sensitivity for myocarditis improved from 54.6% to 91.0%. ASS-patients with myocarditis were more frequently males(53% vs 13%;p=0.009) with fever(69% vs 17%;p=0.001), and had higher hs-TnT (88.0[23.55-311.5] vs 9.80[5.0-23.0]ng/L; p < 0.001), NT-proBNP(525.5[243.5-1575.25] vs 59.0[32.0-165.5;p=0.013]pg/ml;p=0.013) and C-reactive protein(CRP)(7.0[1.7-15.75] vs 1.85[0.5-2.86]mg/L;p=0.011) compared to those without myocarditis.

Conclusion: In ASS, myocarditis is frequent, even at clinical onset. ASS-patients with myocarditis frequently presented with fever and increased CRP, suggesting the existence of an inflammatory phenotype. The use of novel CMR mapping techniques may increase the diagnostic sensitivity for myocarditis in ASS.

Keywords: Anti-synthetase syndrome; Cardiac Magnetic Resonance; Myocarditis.