Clinical and Pathological Features of Microscopic Polyangiitis in 20 Children

Abstract

Both primary and ATD-associated MPA showed a female predisposition. Renal involvement was the most frequently observed condition, followed by involvement of lungs. CNS involvement was not rare in these pediatric patients. The efficacy of steroid plus CTX as induction therapy was evident in these patients.

Comparing Presenting Clinical Features in 48 Children With Microscopic Polyangiitis to 183 Children Who Have Granulomatosis With Polyangiitis (Wegener's): An ARChiVe Cohort Study

“…The frequency of general constitutional symptoms (85%) was in the low end of the ranges reported. Renal disease, present in 75% of the patients in the present study, was also in the low end of the range of frequencies, and is in contrast to that reported previously from Departments of Nephrology, in which renal disease has been reported to be present in 100% of patients . Skin manifestations, predominantly palpable purpura or petechiae, occurred in a majority of our patients (52%) and was consistent with the reported frequencies (15–100%) in other studies (13–17); it should be noted that the 100% frequency of skin manifestations that was reported in the Serbian study was observed in a cohort of only 7 patients.…”

“…The mean age at disease onset in patients with MPA in our study (mean age 10.8 years) falls within the range of ages (mean ages 9–12 years) from other pediatric series . The marked predominance of female patients in our study, a reported frequency of 73%, was also characteristic of that in other case series, in which frequencies of up to 90% have been described; only the Turkish case series had a lower frequency of female patients, at 53%.…”

“…The frequencies of involvement of different organ systems (and specific presenting clinical features) in our MPA cohort are listed in Table . The frequencies of the more commonly involved systems fell within the wide range of frequencies described in other pediatric case series . The frequency of general constitutional symptoms (85%) was in the low end of the ranges reported.…”

“…The subcohort of 183 patients with GPA in the current study is the largest reported cohort of pediatric patients with that disease, and includes 65 patients previously recruited to the ARChiVe cohort up to November 2008 (5). The subcohort of 48 pediatric patients with MPA is larger than the 5 largest previous pediatric case series that contained retrospective data on patients with MPA from Turkey (n = 26), Japan (n = 21), Serbia (n = 7), Beijing, China (n = 19), and Guangzhou, China (n = 16) . The earliest of these studies, from Turkey , described 26 children with PAN, among whom 24 might now be considered to have MPA if diagnosed according to contemporary definitions.…”

“…The multicenter, contemporary inception cohort known as ARChiVe (A Registry for Childhood Vasculitis: e‐entry) has previously reported the largest cohort of children with GPA studied to date (5). Conversely, descriptions of childhood MPA remain limited to only a few studies, in which fewer than 26 patients have been assessed and in which the included patients have not always been uniquely classified .…”