A 10-Year-Old Boy with Pulmonary-Renal Syndrome and Positive C-ANCA


Department of Pediatrics, Dr. Sheikh Children Hospital, Mashhad University of Medical Sciences and Health Services, MASHHAD-IRAN.


The term pulmonary-renal syndrome has been frequently used to describe the clinical manifestations of a great number of diseases in which pulmonary hemorrhage and glomerulonephritis coexist. Antineutrophil Cryoplasmic Autoantibody (ANCA) associated vasculitides include Wegener′s Granulomatosis (WG), microscopic polyangiitis (MPA), renal limited pauci-immune glomerulonephritis and Churg-Strauss syndrome. Causes of pulmonary-renal syndrome which are more frequently associated with antineutrophil cytoplasmic autoantibodies are included MPA and WG. These diseases involve kidney as pauci-immune rapidly progressive glomerulonephritis. For MPA the estimated prevalence is 3-37 cases per million; and for WG It is approximately 3 per 100.000 persons that only 0.1 percent of patients are younger than 19 years. Pathologically, the major finding in renal biopsy is necrotizing glomerulonephritis accompanied by crescent formation in MPA and WG. A 10-year-old boy admitted to our hospital because of respiratory distress, hemoptysis, evidence of renal failure and glomerulonephritis (pulmonary- renal syndrome). Laboratory findings revealed biochemical evidence of renal failure and positive cytosolic anti-neutrophil cytoplasmic antibody (C-ANCA). Chest x-ray showed multiple round opacities with well defined margins in the right lung. Open renal biopsy showed necrotizing vasculitis with involvement of small and medium sized vessels. (Tanaffos 2007; 6(1): 75-79)