[Wegener's granulomatosis in pediatric patients].
Wegener's Granulomatosis (WG) is a disease occurring rarely in childhood and adolescence. Together with the Churg-Strauss-Syndrome and the microscopic Polyangiitis it belongs to the vasculitis syndromes associated with ANCA. WG mostly affects the upper and lower respiratory tract and kidneys. It is characterized by a chronic development and high tendency to relapse. In cases of persistent disturbances of the respiratory system which do not have infectious or allergic geneses differential diagnosis should also consider WG. Although clinical course and prognosis have improved since the introduction of immunosuppressive therapy, WG, leading to renal failure in about one third of all cases, remains a disease which has to be taken seriously. Early treatment has been proven to improve prognosis. Due to the necessity of longterm treatment less toxic immunosuppressive therapy should be applied (e.g. Methotrexate).
Fiebig
B
B
Klinik und Poliklinik für Kinder- und Jugendmedizin, Universitätsklinikum Carl Gustav Carus, Technische Universität Dresden, Dresden. barbara.fiebig@uniklinikum-dresden.de
Hedrich
C M
CM
Heubner
G
G
Gahr
M
M
D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000367:Age Factors; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000032:analysis; D002648:Child; D002675:Child, Preschool; D003520:Cyclophosphamide / Q000008:administration & dosage / Q000627:therapeutic use; D003937:Diagnosis, Differential; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000453:epidemiology / Q000209:etiology / Q000503:physiopathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use; D007223:Infant; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging; D008297:Male; D008727:Methotrexate / Q000008:administration & dosage / Q000627:therapeutic use; D011379:Prognosis; D013902:Radiography, Thoracic; D012008:Recurrence; D014057:Tomography, X-Ray Computed
D000305:Adrenal Cortex Hormones; D019268:Antibodies, Antineutrophil Cytoplasmic; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D008727:Methotrexate