This condition has several pulmonary-renal diseases. The patient usually gives a history of recent hemoptysis and often of malaise, anorexia, and headache. A severe acute glomerulonephritis may be accompanied by diffuse hemorrhagic inflammation of the lungs. The urine shows gross or microscopic hematuria, and laboratory findings of severely suppressed renal function are usually evident.
Biopsy shows glomerular crescents, glomerular adhesions, and inflammatory infiltration interstitially. Electron microscope examination shows an increase in basement membrane material and deposition of fibrin beneath the capillary endothelium.
In cases of anti-GBM disease, circulating antibody against GBM can be identified. IgG, C3, and, often, other components of the classic complement pathway can be demonstrated as linear deposits on the basement membranes of the glomeruli and the lung. This was formerly called Goodpasture’s disease.
Large doses of corticosteroids in combination with immunosuppressive therapy may be useful. Plasmapheresis to remove circulating antibody has been reported to be effective in some patients. Transplantation should be delayed until circulating antiglomerular basement antibodies have disappeared.
In contrast, some patients have crescenteric glomerulonephritis but do not show any immune deposits or antibody deposition by immunofluorescence studies. They have pauci-immune idiopathic rapidly progressive glomerulonephritis (RPGN). Many of these patients are serologically ANCA-positive. High-dose prednisone pulse therapy and cytotoxic agents may result in prolonged remission.