[Clinical Course of an Early Childhood-Onset Granulomatosis with Polyangiitis].

A 26-year-old male patient presented with an eight-week history of unspecific symptoms such as weight loss and fever. Besides, he also suffered from haemoptysis, cough, and arthralgia. Since the age of twelve years, the patient has been treated for Wegner's granulomatosis. At the age of 20 years he received a kidney transplant which failed only four years later.  The relapse we clinically suspected was confirmed by CT scan showing bilateral pulmonary manifestations. Moreover, we found highly positive antibodies against proteinase 3.  After an induction therapy using Glucocorticoids and Rituximab, accompanied by plasmapheresis, the patient's clinical condition showed a marked improvement. We were able to discharge him continuing the treatment in an outpatient setting.  Childhood-onset GPA is a life-threatening disease and often characterized by recurring relapses as well as a significantly reduced quality of life for the patient.