ANCA-associated vasculitides in childhood

Methods and result 14-year-old patient diagnosed with TGA at birth, at the age of 14 she presented a sudden hepatosplenomegaly. The systematic examination confirmed WG. According to our knowledge this is the first case of association of these two infrequent diseases. Furthermore hepatosplenomegaly as initial symptom of WG is especially rare feature. The second patient suffering from asthma for years became non-responder for adequate bronchodilator therapy. Laboratory tests revealed eosinophilic leukocytosis. After excluding the most frequent causes of eosinophilia, the biopsy of maculopapular and nodular skin lesions showed vasculitis, and ANCA antibodies were found, we diagnosed Churg-Strauss syndrome.