چكيده لاتين :
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 Wegenerיs 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
