Childhood Polyarteritis Nodosa: a prospective multicentre study from eastern India.

Area of Science:

• Pediatric Rheumatology
• Vasculitis Research
• Systemic Autoimmune Diseases

Background:

• Childhood Polyarteritis Nodosa (PAN) is a rare systemic vasculitis affecting medium-sized arteries.
• Understanding its clinical spectrum in diverse populations is crucial for timely diagnosis and management.
• Previous studies on childhood PAN have primarily focused on Western populations.

Purpose of the Study:

• To describe the clinical features and organ involvement in Indian children diagnosed with Polyarteritis Nodosa.
• To identify unique presentation patterns and diagnostic parameters in this cohort.
• To provide insights into treatment responses and outcomes in childhood PAN.

Main Methods:

• A prospective observational multicenter study was conducted over 8 years.
• Included children under 12 years diagnosed with childhood PAN based on established criteria.
• Excluded cases of microscopic polyangiitis and cutaneous PAN; diagnosis confirmed by skin biopsy.

Main Results:

• Fifteen children with childhood PAN were identified, with a mean age of 8.6 years.
• Common manifestations included prolonged fever, myalgia, skin involvement, hypertension (93%), and peripheral neuropathy (46%).
• Peripheral gangrene occurred in nine patients; renal, pulmonary, and testicular involvement were absent. Skin biopsy confirmed necrotizing vasculitis.

Conclusions:

• Childhood PAN in India exhibits a distinct clinical pattern with significant skin and neurological involvement but minimal visceral organ compromise.
• This multicentric study provides valuable data on the presentation and management of childhood PAN in a South Asian context.
• The findings underscore the importance of considering PAN in children with unexplained prolonged fever, myalgia, and skin lesions.