Systemic lupus erythematosus with myocardial dysfunction due to microvasculopathy

Area of Science:

• Cardiology
• Rheumatology
• Immunology

Background:

• Systemic lupus erythematosus (SLE) is a complex autoimmune disease with potential multi-organ involvement.
• Cardiac manifestations in SLE, though known, often present with diverse pathologies.
• Myocardial dysfunction in SLE patients requires precise etiological diagnosis for effective management.

Observation:

• A 25-year-old Japanese woman with SLE presented with significant myocardial dysfunction.
• Diagnostic workup including heart catheterization and left ventricular cineangiography revealed normal coronary arteries but regional hypokinesis.
• Myocardial scintigraphy showed patchy defects unrelated to coronary artery disease.

Findings:

• The observed myocardial dysfunction was attributed to microvasculopathy, a condition affecting small blood vessels.
• Evidence suggested vasculitis, rather than thrombotic events, as the cause of microvasculopathy, supported by the absence of antiphospholipid syndrome.
• The patient's positive response to corticosteroid therapy further supported the vasculitic etiology.

Implications:

• This case highlights the potential for SLE-induced microvasculopathy to cause myocardial dysfunction.
• It underscores the importance of considering non-atherosclerotic causes of cardiac dysfunction in SLE patients.
• The findings suggest that targeted immunosuppressive therapy, like corticosteroids, can be effective in managing SLE-related cardiac microvasculopathy.