Churg-Strauss syndrome (CSS) is a vasculitis of small to medium sized vessels. This syndrome is known by a history of bronchial asthma with systemic necrotising vasculitis and peripheral blood

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Churg-Strauss syndrome (CSS) is a vasculitis of small to medium sized vessels. This syndrome is known by a history of bronchial asthma with systemic necrotising vasculitis and peripheral blood hypereosinophilia. It is now calledeosiniphilicgranulomatosis with polyangiitis (EGPA). This disease affects both genders and all age groups but it is very rare among children. CSSdiagnosis is based on clinical findings such as asthma, eosinophilia, rhinosinusitis and signs of vasculitis in major organs. In cases which Steroids alone or in combination with other immunosuppressive agents are used as treatment, the outcome andlong-term survival is usually satisfying. in comparison with other systemic vasculitis, the mortality rate of this syndrome is low. The case that we report is a7 year old boy who had a poorly controlled bronchial asthma since he was 3. Then he developed purpuric skin lesions, sinusitis, arthritis and weakness of limbs with symptoms of mononeuritis multiplex at the age of7. After being admitted to our hospital, a series of studies including CBC-diff, chest X-Ray, paranasal sinus radiographies, brain MRI, nerve conduction study, spirometry and serological tests for autoantibodies, were performed and he was diagnosed as Churg-Strauss syndrome. Thereafter, he received regular corticosteroid therapy in combination with methotrexate and his symptoms were generally well-controlled with the beginning of the treatments. The clinical characteristics, diagnosis and management of CSS in children are also reviewed.

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تاریخ انتشار 2017