Background Polyarteritis nodosa (PAN) is a necrotizing vasculitis involving medium or small sized vessels. It is characterized by a variable clinical presentation, ranging from a benign cutaneous involvement to a more severe systemic disease with multisystem involvement
Objectives We describe clinical and laboratory features, treatment and outcome of pediatric PAN patients
Methods A retrospective case review of all children diagnosed as PAN at a single tertiary care hospital in north India between 1990 to 2014 was done. Diagnosis of PAN was based on European League against Rheumatism (EULAR)/Paediatric Rheumatology European Society (PRES)/Paediatric Rheumatology International Trials Organisation (PRINTO) classification criteria. (1)
Results A total of 16 children (12 boys and 4 girls) with PAN were identified. Median age of symptom onset and diagnosis was 8 years (range 1–11 years) and 9 years, respectively. The presenting symptoms were fever (68%), CNS complaints [headache, seizures, altered sensorium, stroke or vision loss] (50%), fatigue (44%), myalgia (44%), gangrene (31%), weight loss (31%), gastrointestinal complaints [pain abdomen, bleed] (31%) and bluish black patches over body (19%). Hypertension was present in 88% patients at admission. Various neurological manifestations were seen in 62.5% patients. Skin involvement (digital gangrene, livedo reticularis, bluish black patches, erythema nodosum or necrotic ulcer) was seen in 50% patients. Renal and gastrointestinal involvements were seen in 38% patients each.
CT angiography was done in 13 patients. Aneurysmal dilatation of vessels was seen in 6 patients, while 3 patients showed features of renal infarcts. CT angiography was normal in 4 patients. In 2 such patients, digital subtraction angiography (DSA) showed aneurysmal dilatation of vessels. ANCA was positive in 4 patients (c-ANCA in 3 and p-ANCA in 1). HBsAg was positive in 3 patients (not treated as liver function remained normal). ASO was positive in 4 patients (2 patients were given penicillin; one patient had a relapse with high ASO titres on stopping penicillin).
Two patients had only a single visit to the clinic; hence their treatment details were not available. Treatment regimen included cyclophosphamide (78%), methylprednisolone pulses (43%), oral prednisolone (93%), maintenance azathioprine (36%), and IVIg and methotrexate (14% each). Mean follow up was for 4.8 years (range 0–25 years).
Five patients experienced relapses with more than 1 relapses seen in 2 patients. Relapses were noted as early as 6 months and as late as 22 years after diagnosis
Conclusions We found a significant male preponderance in our cohort. Streptococcal infection can have a pathogenic role in the disease progression and relapse, and penicillin prophylaxis can prevent disease relapse in susceptible patients
Ozen S, Pistorio A, Iusan SM, Bakkaloglu A, Herlin T, Brik R, et al. EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria. Ann Rheum Dis. 2010 May;69(5):798–806.
Disclosure of Interest None declared