Clinical manifestation of pediatric @entity2883 with XXXX - the experience of two regions in Poland.

@entity1 @entity1244 @entity548 @entity740 @entity2883 @entity5142 @entity454
BACKGROUND: The purpose of this study was to describe clinical manifestations, laboratory findings and outcome of @entity2883 with @entity2883 (GPA) in pediatric @entity1 living in two regions (Southern and Central) of Poland. METHODS: Retrospective analysis of @entity1 hospital records from four large hospitals during a period from 1995 to 2013. @entity1 with confirmed diagnosis of GPA according to American College of Rheumatology (ACR) and EULAR/PRINTO/PRES criteria for GPA were analyzed. All @entity1 were subjected to clinical, laboratory, radiological and immunological assessment. RESULTS: During this 18-year period only 9 @entity1 with confirmed diagnosis of GPA (6 @entity1 , 3 @entity1 ) were identified. The average age of the disease onset was 12 years (range: 8-16 years). Average delay between first symptoms and diagnosis was approx. 20 months (range: 0-84 months). Organ system involvement at presentation included: kidneys 88.8% (8/9), lungs 77.7% (7/9), ear/nose/ throat 55.5% (5/9), gastrointestinal tract 55.5% (5/9), skin 44.4% (4/9), joints 22.2% (2/9), eyes 11.1% (1/9) and nervous system 11.1% (1/9). In 5 @entity1 disease course was progressive (constant progression of @entity1244 in one case, @entity454 in two, @entity740 stage IV in one and one @entity1 died due to @entity548 ). CONCLUSION: The majority of our @entity1 were females. Clinical features of pediatric GPA were similar to those described in adults. None of our @entity1 developed @entity5142 and in only 2 @entity1 saddle-nose deformity was observed. Although GPA was treated according to contemporary standards care, disease progression was observed in more than a half of @entity1 .
