Key result
Immunosuppressive therapy resolves myocardial inflammation and improves outcomes in suspected IgG4-related myocarditis.
Why the study?
IgG4-related disease is a systemic fibroinflammatory condition that can affect any organ, but myocarditis represents a rare manifestation.
Case Report (n=1)
IgG4-related disease is an uncommon but important differential diagnosis for unexplained myocardial inflammation and troponin-positive non-obstructive coronary arteries, as cardiac injury may be reversible with early immunosuppression.
Hypothesis-generating for immunosuppression in suspected IgG4 myocarditis; prospective trials needed before practice change.
Background: IgG-4-related disease (IgG4-RD) is a systemic fibroinflammatory disease that can affect any organ system. Myocarditis is a rare manifestation. Case summary: A 65-year-old woman with clinically diagnosed IgG4-RD, who had recently completed corticosteroid taper, presented with multiple admissions for recurrent chest pain and myocardial injury. Coronary angiography repeatedly showed preserved coronary anatomy, and initial transthoracic echocardiography showed preserved left ventricular systolic function. Subsequent transthoracic echocardiography showed rapid decline in ejection fraction, and cardiac magnetic resonance imaging (MRI) showed myocardial oedema, infarct scar, and patchy non-ischaemic fibrosis. A probable diagnosis of IgG4-RD myocarditis was made clinically based on medical history, multimodal imaging findings, and exclusion of alternative diagnoses, although tissue confirmation was not obtained. The patient improved clinically and radiographically with corticosteroid re-initiation followed by maintenance with mycophenolate mofetil (MMF). Conclusion: This case underscores the importance of considering IgG4-RD in the differential diagnosis of unexplained myocardial inflammation and highlights the potential reversibility of cardiac injury with early immunosuppression.
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Ellis et al. (2026) conducted a case report in IgG4-related disease myocarditis (n=1). Corticosteroids and mycophenolate mofetil was evaluated on Clinical and radiographic improvement. Immunosuppressive therapy with corticosteroids and mycophenolate mofetil led to clinical and radiographic improvement in a 65-year-old woman with suspected IgG4-related myocarditis.
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