Open Access Case Report

Pulmonary medium vessel vasculitis in an 11 year old boy: Hughes Stovin syndrome as a variant of polyarteritis nodosa?

Willemien de Vries1, Gerard H Koppelman2, Marc TR Roofthooft34, Hendrika Bootsma6, Martha K Leijsma6 and Wineke Armbrust5*

Author Affiliations

1 Department of Pediatrics, Beatrix Children's Hospital, University Medical Centre, University of Groningen, The Netherlands

2 Department of Pediatric Pulmonology and Pediatric Allergology, Beatrix Children's Hospital, University Medical Centre, University of Groningen, The Netherlands

3 Department of Pediatric Cardiology, Beatrix Children's Hospital, University Medical Centre, University of Groningen, The Netherlands

4 Department of National Expertise Centre for Children with Pulmonary Hypertension, Beatrix Children's Hospital, University Medical Centre, University of Groningen, The Netherlands

5 Department of Pediatric Rheumatology, Immunology and Infectious Disease, Beatrix Children's Hospital, University Medical Centre, University of Groningen, The Netherlands

6 Rheumatology and Clinical Immunology, University Medical Centre Groningen, University of Groningen, The Netherlands

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Pediatric Rheumatology 2011, 9:19  doi:10.1186/1546-0096-9-19

Published: 4 August 2011

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.