Isfahan University of Medical Science*
Isfahan University of Medical Science
Granulomatosis with polyangiitis (GPA) is chronic granulomatous necrotizing C-ANCA positive systemic vasculitis, most frequently in adults and typically involves the respiratory system and the kidneys.
Few cases in children have been reported yet and CNS manifestations are also rare.
Here we discuss a case of Granulomatosis with polyangiitis (Wegner) in a 12-year-old female who presented with a cough, dyspnea, fever, and high blood pressure. Pulmonary involvement showed cavitary lesions and nodules. Lab data showed
increased serum creatinine and macroscopic hematuria. patient’s C-ANCA was positive. Considering such findings renal biopsy was considered and confirmed the diagnosis of GPA. During her hospitalization, due to headache & loss of consciousness brain MR imaging was done. Evidence of Small vessel vasculitis with confluent T2 hyper signal intensity of gray- white matter junction of both parietooccipital and frontal lobes containing hemorrhagic component suggestive of Posterior reversible encephalopathy syndrome (PRES) was seen