Email updates

Keep up to date with the latest news and content from Pediatric Rheumatology and BioMed Central.

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

For all author emails, please log on.

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.