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

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. © 2011 de vries et al; licensee biomed central ltd.