title: Pulmonary medium vessel vasculitis in an 11 year old boy: Hughes Stovin syndrome as a variant of polyarteritis nodosa?
abstract: We present the case of an 11-year-old boy presenting with haemoptysis, dyspnoea and weight loss as a manifestation of isolated pulmonary vasculitis, leading to pulmonary hypertension. He also appeared to have a longstanding dural venous sinus thrombosis. This rare presentation, especially in childhood, might represent a case of the seldomly reported Hughes-Stovin syndrome. The patient achieved remission after therapy with cyclophosphamide pulses and high-dose steroids. Based on the presented case and review of the literature, we propose that this syndrome might be a variant of polyarteritis nodosa. This report highlights diagnostic issues and describes a successful treatment regimen.
authors.affiliation: Department of Pediatric Rheumatology, Immunology and Infectious Disease, Beatrix Children's Hospital, University Medical Centre, University of Groningen, The Netherlands. w.armbrust@umcg.nl.
authors.forename: Willemien
authors.initials: W
authors.lastname: de Vries
authors.forename: Gerard H
authors.initials: GH
authors.lastname: Koppelman
authors.forename: Marc Tr
authors.initials: MT
authors.lastname: Roofthooft
authors.forename: Hendrika
authors.initials: H
authors.lastname: Bootsma
authors.forename: Martha K
authors.initials: MK
authors.lastname: Leijsma
authors.forename: Wineke
authors.initials: W
authors.lastname: Armbrust