Clinicopathological and morbidity profile of childhood polyarteritis nodosa in a tertiary referral center

ABSTRACT

Background: Polyarteritis nodosa (PAN) is a primary systemic necrotizing vasculitis predominantly targeting medium-sized arteries defined as the main visceral arteries and their branches. The objective of this study was to analyze the epidemiology, clinico-pathological and point outcome of PAN in children at a tertiary care centre in Karnataka.Methods: This prospective observational study was conducted among eighteen children diagnosed with PAN from December 2015 to May 2017.' All the children fulfilling EULAR/PRES/PRINTO classification criteria for childhood PAN were included in this study. Clinico-pathological profile of these patients was studied and documented.Results: This study had shown that there are two peaks of age at onset of symptoms at 5 to 7 years and from 11 to 13 years. No sex predilection was noted. Most children present with nonspecific symptoms like myalgia (94%), fever (84%), weight loss (50%) and joint pain (50%). Skin manifestations were seen in all the children. Neurological involvement (61%) in the form of mononeuritis multiplex (38.8%), hypertensive encephalopathy (11%) and stroke (11%) was observed. Four children (22%) presented with pain abdomen while two boys had testicular pain. Only one child had renal manifestation in the form of glomerulonephritis. Most children had neutrophilic leukocytosis, elevated ESR and thrombocytosis. Skin biopsy was performed in ten children and out of these, 9 reports were suggestive of PAN. Angiography was done in four children revealing multiple aneurysms or stenosis in the systemic arteries. All the children received corticosteroids. Cyclophosphamide was used as an induction agent in about 50% of the patients. Corticosteroid treatment alone was sufficient in only one patient. Steroid sparing agent like azathioprine (38%), mycophenolate mofetil (33%) and hydroxychloroquine (5%) were used with low dose steroids as maintenance therapy. Seven children (39%) achieved remission off drugs while 8 children are still on treatment, one child relapsed and three children were lost to follow up.Conclusions: Childhood vasculitis leads to considerable delay in diagnosis the initiation of treatment. High index of suspicion and early referral is required in all cases of vasculitis to reduce morbidity and mortality.