Lupus vasculopathy: Diagnostic, pathogenetic and therapeutic considerations

Area of Science:

• Rheumatology
• Nephrology
• Vascular Medicine

Background:

• Systemic lupus erythematosus (SLE) can manifest with rare vascular complications like lupus vasculopathy.
• Lupus vasculopathy, characterized by arterial necrosis and immunoglobulin/complement deposition, often accompanies lupus nephritis and indicates a poor prognosis.
• Effective treatment strategies for lupus vasculopathy remain undefined, unlike the established protocols for lupus nephritis.

Observation:

• A 20-year-old woman with SLE presented with seizures, hypertension, renal disease, and neurological deficits.
• Despite treatment with mycophenolate mofetil, prednisone, and hydroxychloroquine, her condition, including renal function, did not improve.
• Diagnostic workup revealed lupus anticoagulant, cerebral hemorrhage, reduced ejection fraction, and severe mitral regurgitation, with kidney biopsy confirming lupus vasculopathy and glomerulonephritis.

Findings:

• Plasma exchange therapy combined with intravenous cyclophosphamide was initiated for the patient.
• Following treatment, significant improvement was observed in renal function, complement levels, and autoantibody titers.
• The lupus anticoagulant also resolved, suggesting a potential role for antiphospholipid antibodies in lupus vasculopathy pathogenesis.

Implications:

• Lupus vasculopathy can present even with aggressive immunosuppressive therapy in SLE patients.
• The combination of plasma exchange and intravenous cyclophosphamide shows promise as an effective treatment for severe lupus vasculopathy.
• This case highlights the potential involvement of antiphospholipid antibodies in the development of lupus vasculopathy and suggests a viable therapeutic approach.