Case report: computer tomography presentation of Wegener’s Granulomatosis in a 10-year-old boy with renal syndrome

Abstract

Background: The term pulmonary-renal syndrome consists of a group of complex and often severe disorders, although rare in incidence, and includes Wegener's Granulomatosis (WG) which is a predominantly small-vessel vasculitis associated with antineutrophil cytoplasmic antibodies (ANCAs). There are few reports describing its clinical features and outcome in children. We report computed tomography (CT) findings in a 10-year-old boy referred to our Pediatric Department. Materials and Methods: A 10-year-old boy presented in April 2013 with rhinitis, fever and dry cough. He was prescribed antibiotics with moderate improvement of the general condition. His examination results were unremarkable except low hemoglobin level (9.9 g/dL) and markedly increased erythrocyte sedimentation rate (44 mm/h). A month later he had been admitted to Nephrology Unit with complaints of proteinuria, hematuria and anemia. In June he developed also arthritis. In October 2013 the child was admitted to the Pediatric Intensive Care Unit in a severe condition. Antineutrophil cytoplasmic antibodies (ANCA) were positive with antigen specificity for myeloperoxidase (anti-MPO 37 KU/L). The other laboratory results included: mild anemia and leukocytosis; proteinuria (69 mg/kg/day); increased blood urea nitrogen (BUN) and creatinine (10.4 mmol/L and 123 mmol/L, respectively). Thoracic CT revealed a solitary nodule 1.5x1 cm in the posterio-basal segment of the inferior lobe in the left lung. Renal biopsy with fine needle revealed pauci-immune crescentic glomerulonephritis. He was diagnosed as WG from the clinical, radiologic, laboratory and morphologic findings and was given treatment with methylprednisolone and cyclophosphamide. Results and discussion: The CT findings of pulmonary WG include multiple nodules or masses with or without cavitation, and are particularly helpful to identify cavities within nodules. The ANCA-associated pulmonary-renal syndrome, ANCA positive with antigen specificity for myeloperoxidase (anti-MPO), is almost always caused by microscopic polyangiitis and this association can be manifested as rapidly progressive renal failure, as happened with our patient. Conclusions: Our aim in presenting this case is to alert clinicians that, even without the definitive histological diagnosis, it is possible, based on clinical history and physical examination, and whenever possible serological tests (ANCA and anti-GBM), to start immunosuppressive therapy, that can avoid the irreversible loss of renal function and interrupt the fatal course of lung complications.