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termed ‘perimyocarditis’. The clinical management and outcomes of mixed forms follow the predominant involvement. At present, patients with myopericarditis can be treated as patients with pericarditis, while patients with perimyocarditis should be managed as patients with pure myocarditis. The knowledge in the field is rapidly evolving and ongoing trials and evidence will provide more updated guidance for clinical management. A common scenario with overlapping forms of myocarditis and pericarditis is represented by systemic immune disorders, which can affect either the myocardium or pericardium, or both. Several systemic inflammatory and autoimmune diseases may present with myocarditis, pericarditis, mixed forms, or PEff without pericarditis. Main conditions include RA, SLE, Sjögren’s syndrome, SSc, polymyositis and dermatomyositis, and vasculitis (e.g. particularly EGPA).

8.1 Inflammatory myopericardial

syndrome in systemic disorders Inflammatory myopericardial syndrome may complicate several types of systemic diseases, generally immune-mediated,349,350 either with myocardial (myocarditis) or pericardial involvement (pericarditis and/ or PEff). Symptoms and signs that may raise the suspicion of a rheumatic autoimmune disease are family history, history of low-grade fever, joint symptoms, dry eyes and mouth, headache, lymphadenopathy, Raynaud phenomenon, skin rash, oral and genital aphthae, deep or superficial vein thrombosis, recurrent foetal losses, lymphopenia, and chronic increase of CK and other inflammatory markers. Targeted therapy should be applied, if a specific diagnosis is reached during the diagnostic work-up.

8.1.1 Rheumatoid arthritis

Pericarditis can be seen as part of the inflammatory activity in RA, and is often present concurrently with pleuritis. It is the most common cardiac manifestation, affecting up to 50% of patients, as detected on postmortem examination. Myocarditis may also be present, and generally resolves if the disease is controlled.351 Anakinra, already registered for the treatment of RA, can be used also for related pericardial involvement.

8.1.2 Systemic lupus erythematosus

Pericarditis is the most common cardiac manifestation of SLE, reported in approximately 25% of patients, and it is included in the American Rheumatism Association/American College of Rheumatology (ARA/ ACR) classification criteria.352 Myocardial involvement in SLE affects about 5% of patients.353,354,355 Symptoms of IMPS are often nonspecific, including elevated troponin, alterations on the ECG, LVEF abnormalities, and PEff. These manifestations are usually associated with typical symptoms of SLE, including fever, arthritis, nephritis, and skin rashes.356,357 The diagnosis can be supported by specific laboratory markers, as well as multimodality imaging, to guide the therapy.358,359 Endomyocardial biopsy could be rarely considered in selected cases.349 Cardiac tamponade occurs in less than 2% of cases, and CP is extremely rare. Cardiomyopathy is uncommon clinically, but autopsy studies found myocardial involvement in 40%–50% of patients.

8.1.3 Antiphospholipid syndrome

Antiphospholipid syndrome is associated with the presence of anti-cardiolipin and/or anti-β2-glycoprotein antibodies, and/or lupus anticoagulant, and may be the cause of myocardial dysfunction through various mechanisms. While myocarditis can manifest in this syndrome, mediated by autoimmune mechanism, myocardial damage can be also caused by microthrombotic phenomena.360,361

8.1.4 Sjögren’s syndrome

In Sjögren’s syndrome, the most common clinical manifestation of cardiac damage is pericarditis, which has been reported in less than one-third of cases. Myocarditis and myocardial fibrosis are also possible,362 and may occur associated with rhythm disturbances. Specific antibodies (anti-Ro/SSA) could also be identified in unexplained heart blocks.363

8.1.5 Systemic sclerosis

Inflammatory myopericardial syndrome is frequent in SSc,364 often clinically occult and, when symptomatic, the prognosis is poor. It may be related to ventricular dysfunction and/or structural damage of the microvascular bed, leading to repeated focal ischaemic injuries and irreversible myocardial fibrosis. It can be also due to a primary systemic myositic disease.349,365 Damage usually follows two pathways.366 The first one is linked to the production of collagen by activated fibroblasts at the myopericardial level, especially at the subepicardial level, with consequent fibrosis, valve stenosis, coronary spasms, and ischaemic events. The second pathway is caused by renal and pulmonary involvement, with severe cardiac consequences.367,368 Further research is needed to improve the understanding of IMPS in SSc.364

8.1.6 Polymyositis and dermatomyositis

The myocardium can be affected in inflammatory myopathies, such as polymyositis and dermatomyositis,369 with consequent myocardial hypertrophy, ischaemia, cardiac enlargement, and systolic and diastolic ventricular dysfunction.370 Cardiac involvement in inflammatory myopathies is clinically occult in most patients, but may be suspected by multimodality imaging371,372 and is related to poor outcome.370,372 Myocarditis occurs in up to 30% of autopsied patients, with or without concomitant coronary or vessel vasculitis. Pericarditis is rarely reported in these patients.

8.1.7 Vasculitis

Myocardial involvement is prognostically relevant in vasculitis, particularly EGPA. In EGPA, about 15%–60% of patients present with cardiac disease350 including PEff, conduction disturbances, and motion abnormalities, as well as ACS, often due to coronary spasm, and EM (see Section 9.2). While cardiac involvement in EGPA often occurs early, it can be late in the course of the disease, and mainly affects antineutrophil cytoplasmic antibody (ANCA)-negative patients with high eosinophil counts. Myocardial and pericardial involvement is also possible in other vasculitis, especially in Kawasaki’s disease, where myocarditis is a common cardiovascular complication (see Supplementary data online, Figure S3).373 Cooperation with specialists is important in all these systemic conditions to reach the correct diagnosis and to plan appropriate treatment. The diagnosis of EGPA may be particularly challenging.349

8.2 Inflammatory myopericardial

syndrome in COVID-19 disease The COVID-19 pandemic raised the awareness of IMPS, as in many patients the myocardium as well as the pericardium were affected. It is not proven whether the SARS-CoV-2 virus can directly damage cardiomyocytes or pericardiocytes, as it was suggested in cultivated human cardiomyocytes.374 Autopsy studies could only detect small amounts of virus Downloaded from academic.oup.com/eurheartj/article/46/40/3952/8234483 by guest on 07 October 2026