Introduction: Systemic Lupus Erythematosus (SLE) is a connective tissue autoimmune disease with mul-tiple organ involvement. The cardiovascular system is frequently affected and is associated with significant morbidity and mortality. Endothelial damage in SLE is believed to be the main cause of accelerated cardio-vascular disease (CVD) in lupus population, leading to premature atherosclerosis with subsequent cardiac events. The endocardium, myocardium, and pericar-dium are less frequently involved, nevertheless, they are associated with fatal outcomes. Lupus myocarditis (LM) has a scarce occurrence, with a reported preva-lence of less than 10% during the course of the disease, hence, the aim of our paper is to increase awareness of this clinical manifestation.

Methods: A 48 y/o woman presented to an outpatient cardiology practice with palpitations, mild dyspnoea, and non-exertional chest pain, and a 12 months histo-ry of arthralgia of the small hand joints. Cardiac ultrasound and late gadolinium enhancement cardiac mag-netic resonance supported the diagnosis of mild pe-ricardial effusion and myocarditis. Laboratory results were positive for antithyroid peroxidase antibodies (Ab), antinuclear Ab (ANA), anti SSA Ab, anti SSB Ab, anti Ro52 Ab, rheumatoid factor, increased inflamma-tory markers, thrombocytopenia, microscopic haema-turia and normal thyroid hormones. Furthermore, a family history for autoimmune disease was present.

Results: The patient was diagnosed with systemic lu-pus erythematosus secondary Sjogren syndrome (SjS), lupus myocarditis and autoimmune thyroiditis, with an increased disease activity score at presentation.

Conclusions: LM is a rare entity and usually requires extended tests for diagnosis. It develops early in the SLE course and clinical data reported a 40% mortality rate; early diagnosis and aggressive treatment are man-datory. Due to limited evidence, a link between specific SLE or SjS oantibodies and myocarditis has not been established. Anti-SS-A and anti-SS-B are associated with valvular lesions in adult SLE patients. Furthermo-re, Anti-SSA Ab have long been documented to lead to heart involvement in children born to mothers bearing these antibodies, late-onset cardiomyopathy being one of the manifestations. Antibodies most frequently re-ported in association with LM are anti Smith Ab and anti nRNP. Disease activity has been the only risk fac-tor clearly associated with LM occurrence in SLE po-pulation.