Extensive medium-vessel vasculitis with SLE: an unusual association

Area of Science:

• Rheumatology
• Immunology
• Vascular Medicine

Background:

• Systemic lupus erythematosus (SLE) is a complex autoimmune disease affecting multiple organ systems.
• Vasculitis commonly occurs in SLE, typically affecting small vessels (leukocytoclastic vasculitis).
• Medium- and large-vessel vasculitis are rare in SLE, with limited case reports usually involving single vessel beds.

Observation:

• A patient with SLE presented with classic symptoms, toe gangrene, and paraplegia.
• Radiological studies revealed diffuse medium-sized vessel vasculitis.
• The patient met several American College of Rheumatology criteria for SLE.

Findings:

• This case represents a rare instance of extensive medium-sized vessel vasculitis associated with SLE.
• Treatment with pulse cyclophosphamide and high-dose prednisolone led to improvement in SLE symptoms and no new vasculitis.
• The patient's paraplegia did not improve with treatment.

Implications:

• Highlights the potential for SLE-related vasculitis to affect medium-sized vessels extensively.
• Emphasizes the need to consider medium-vessel vasculitis in SLE patients presenting with severe ischemic complications.
• Suggests that while SLE symptoms may improve, severe neurological deficits like paraplegia may not be reversible.