Systemic Polyarteritis Nodosa in the Young: A Single‐Center Experience Over Thirty‐Two Years

Abstract

Results. Sixty-nine children with PAN were identified; 55% were male, and their median age was 8.5 years (range 0.9-15.8 years). Their clinical features at presentation were fever (87%), myalgia (83%), skin (88%), renal (19%), severe gastrointestinal (GI) (10%), and neurologic (10%) involvement. The PVAS at presentation was 9 of 63 (range 4-24). Histopathologic analysis of the skin showed necrotizing vasculitis in biopsy samples from 40 of 50 children. Results of selective visceral arteriography suggested the presence of PAN in 96% of patients. Treatment included cyclophosphamide and corticosteroids (83%), plasma exchange (9%), and biologic agents (after 2002; 13%). The relapse rate was 35%, and the mortality rate was 4%. Severe GI involvement was associated with increased risk of relapse (P ‫؍‬ 0.031), while longer time to induce remission (P ‫؍‬ 0.022) and increased cumulative dose of cyclophosphamide (P ‫؍‬ 0.005) were associated with lower relapse risk.Conclusion. Childhood PAN is a severe inflammatory disease of insidious onset and variable clinical presentation. Relapses occurred more frequently in those with severe GI involvement. A higher cumulative dose of cyclophosphamide was associated with a lower risk of relapse.

Orbital inflammatory disease management

“…This entity affects the eye and orbit in roughly 10% of patients. The disease usually affects adults between 40 and 60 years old but can also occur in children [93,94]. Diagnosis of PAN is based on the presence of clinical criteria organized by the American College of Rheumatology [95].…”

“…In PAN not confined to the orbits, induction treatment has also been successfully carried out with rituximab [100,101] and infliximab [102] in separate, isolated case reports. With regard to maintenance therapy, azathioprine, methotrexate, and mycophenolate mofetil have been successfully utilized in smaller, non-blinded case series [94,103].…”

Diagnostic approach and current treatment options in childhood vasculitis

“…Since organ involvement is extremely variable in polyarteritis nodosa, very different complaints and findings may occur. While the skin, musculo-skeletal system, kidney, gastrointestinal system and neurological system are involved fre- (1,(17)(18)(19).…”

“…Eleftheriou et al (18) retrospectively examined a large number of patients (n=69) who were followed up in their own center for 32 years and were diagnosed with PAN in a paper related with childhood PAN published in 2013. The most common initial complaints and findings included cutaneous involvement (88%), fever (87%), myalgia (83%), arthritis/arthralgia (75%), weight loss (64%), fatigue (62%), hypertension (16%), peripheral neuropathy (4%) and the systems involved included the urinary system (19%), gastrointestinal system (10%) and nervous system (10%).…”

“…In the study conducted by Eleftheriou et al (18), it was reported that cyclophosphamide and corticosteroid were found to be the most commonly used drugs (83%) and the other therapeutical options included plasma exchange (9%) and biological drugs (after 2002; 13%). The recurrence rate was found to be 35% and the mortality rate was found to be 4%.…”