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Updated: Aug 29, 2025
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Published on: June 16, 2020
[Heart involvement in systemic lupus erythematosus and antiphospholipid syndrome]
N Morel1, V Le Guern1, L Mouthon1
1Service de médecine interne, hôpital Cochin, Centre de référence maladies auto-immunes et systémiques rares d'Île-de-France, Assistance publique-Hôpitaux de Paris (AP-HP), Paris, France.
Insights
Systemic lupus and antiphospholipid syndrome can affect the heart, causing pericarditis, myocarditis, or coronary issues. Early diagnosis and management, potentially with colchicine, can improve outcomes and prevent severe cardiac complications.
Area of Science:
- Cardiology
- Rheumatology
- Immunology
Context:
- Cardiac involvement is a significant concern in systemic lupus (SL) and antiphospholipid syndrome (APS).
- Presentations vary, including pericarditis, myocarditis, coronary artery disease, and valve disease.
- Understanding these manifestations is crucial for timely diagnosis and effective management.
Purpose:
- To review the spectrum of cardiac manifestations in SL and APS.
- To highlight the common and rare cardiac complications associated with these conditions.
- To discuss potential therapeutic strategies and their impact on patient prognosis.
Summary:
- Pericarditis is the most frequent lupus cardiac manifestation, often responsive to corticosteroids or colchicine.
- Myocarditis in SL, though rare, can lead to heart failure and dilated cardiomyopathy.
- APS commonly causes valve disease and thrombotic events, including myocardial infarction, with catastrophic APS leading to multiorgan failure.
- Coronary involvement is typically atherosclerotic or thrombotic, especially with co-existing APS.
- Iatrogenic cardiac issues can arise from treatments like cyclophosphamide or antimalarials.
Impact:
- Provides a comprehensive overview of cardiac involvement in SL and APS for clinicians.
- Emphasizes the importance of considering cardiac complications in patients with these autoimmune diseases.
- Informs therapeutic decisions, potentially reducing steroid use and preventing relapses or severe outcomes.
Abstract:
Cardiac involvement in systemic lupus (SL) and antiphospholipid syndrome (APS) can be due to variables and involve different presentations. Pericarditis is the most common lupus manifestation and occurs in 16% to 25% of patients. While corticosteroids are usually very effective, colchicine may avoid steroids and prevent relapse. Myocarditis during SL is rare and often inaugural. They may manifest as chest pain, acute heart failure, arrhythmias or conduction disturbances, and may progress to dilated cardiomyopathy and/or permanent heart failure. Their prognosis is however generally good, even in the absence of treatment with cyclophosphamide for the less serious forms. Finally, coronary involvement in SL is most often due to atherosclerotic, thrombotic origin (generally in the context of associated APS), and exceptionally explained by coronary vasculitis. During APS, valve disease is frequent and usually asymptomatic. Thrombotic damage can be (1) coronary, typically manifesting as a myocardial infarction in a young subject with healthy coronary arteries, (2) much more rarely intracardiac, or (3) microcirculatory, generally as part of a catastrophic antiphospholipid syndrome (CAPS) leading to a multiorgan failure. Finally, iatrogenic cardiac manifestations can exceptionally be seen during treatment with cyclophosphamide or antimalarials characterized by conduction disorders and/or heart failure.
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