Pulmonary medium vessel vasculitis in an 11 year old boy: Hughes Stovin syndrome as a variant of polyarteritis nodosa?
| Autor: | Hendrika Bootsma, Gerard H. Koppelman, Wineke Armbrust, Willemien de Vries, Marc T. R. Roofthooft, Martha K. Leijsma |
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| Přispěvatelé: | Groningen Research Institute for Asthma and COPD (GRIAC), Faculteit Medische Wetenschappen/UMCG, Translational Immunology Groningen (TRIGR) |
| Rok vydání: | 2011 |
| Předmět: |
Pediatrics
medicine.medical_specialty lcsh:Diseases of the musculoskeletal system CHILDREN Case Report Rheumatology medicine.artery CRITERIA Immunology and Allergy Medicine Pediatrics Perinatology and Child Health GRANULOMATOSIS BEHCETS-DISEASE business.industry Polyarteritis nodosa lcsh:RJ1-570 lcsh:Pediatrics medicine.disease Thrombosis Pulmonary hypertension medicine.anatomical_structure Pediatrics Perinatology and Child Health Dural venous sinuses Hughes–Stovin syndrome Kawasaki disease lcsh:RC925-935 business Bronchial artery Vasculitis ARTERY ANEURYSMS |
| Zdroj: | Pediatric Rheumatology Online Journal Pediatric Rheumatology Online Journal, Vol 9, Iss 1, p 19 (2011) Pediatric rheumatology, 9:19. BioMed Central Ltd. |
| ISSN: | 1546-0096 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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