String-of-pearls: Extensive medium vessel vasculitis associated with childhood lupus.

Area of Science:

• Pediatric Rheumatology
• Systemic Autoimmune Diseases
• Vasculitis

Background:

• Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with diverse clinical manifestations.
• Vasculitis, an inflammation of blood vessels, can be a rare but severe complication of SLE.
• Early recognition and aggressive management are crucial for favorable outcomes in pediatric SLE.

Purpose of the Study:

• To report a unique case of severe systemic vasculitis secondary to SLE in a pediatric patient.
• To illustrate the diagnostic challenges and treatment response in a rare manifestation of SLE.

Main Methods:

• A comprehensive review of the patient's clinical history, symptoms, and diagnostic workup.
• Imaging studies including CT aortogram to visualize arterial involvement.
• Treatment assessment based on clinical response to immunosuppressive therapy.

Main Results:

• The patient presented with a constellation of symptoms including rash, oral ulcers, alopecia, and neurological deficits (facial and lateral rectus palsy).
• CT aortogram revealed a characteristic 'string-of-pearls' appearance in multiple arteries, indicative of severe vasculitis.
• The patient showed significant improvement following treatment with methylprednisolone and cyclophosphamide.

Conclusions:

• This case underscores the potential for SLE to present with extensive systemic vasculitis, even with initially minimal systemic symptoms.
• It emphasizes the importance of considering vasculitis in the differential diagnosis of pediatric patients with lupus and neurological or vascular abnormalities.
• Aggressive immunosuppressive therapy can effectively manage severe lupus vasculitis.