Alveolar hemorrhage with anti-neutrophil cytoplasmic antibody (ANCA) is a rare disease in children.
ANCA is associated with certain diseases such as granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EPGA). This group of disease, characterized by necrotizing small-vessel vasculitis show autoantibodies in patients serum, directed against neutrophil cytoplasmic constituents, especially proteinase 3 (PR3) and myeloperoxidase (MPO). In these diseases, affected vessels have changed with focal necrosis in pathologic findings and patients suffer from pulmonary, cerebral, gastrointestinal, other hemorrhagic complication and also renal dysfunction represented by crescentic glomerulonephritis.
A 8-year-old previous healthy girl presented with acute onset dyspnea, cough, hemoptysis, chest discomfort, fever and hypoxemia. Her chest CT scan showed diffuse consolidation and ground glass opacity in both lungs, hemoglobin was 3.9g/dL, hematocrit 12%, so she was diagnosed as diffuse alveolar hemorrhage.Bleeding diatheses were excluded by laboratory testing, and Anti nuclear antibody(ANA) and P-ANCA were positive. Myeloperoxidase-ANCA (MPO-ANCA) was positive but proteinase 3-ANCA was negative. Antiglomerular basement membrane antibodies(Anti-GBM) was negative. urinalysis showed RBC 21-30/HPF, creatinine 0.48mg/mL but there were no RBC casts or protein.
This patient had been diagnosed for microscopic hematuria, but her kidney function was normal and other symptoms of ANCA associated vasculitis had never been appeared. The patient's alveolar hemorrhage recovered on immunosuppresive therapy with cyclophopamide and corticosteroids. We report 8-year old girl case, treated for severe alveolar hemorrhage and microscopic hematuria with positive ANCA.
Key words: Diffuse alveolar hemorrhage, microscopic hematuria, ANCA