ABSTRACT
Anti-glomerular basement membrane (Anti-GBM) nephritis is an autoimmune disorder characterized by rapidly progressive crescentic glomerulonephritis (RPGN). The treatment of anti-GBM nephritis with plasmapheresis, steroids and immunosuppressant has improved outcomes. An early diagnosis is essential for the survival of patients and a recovery of renal function. The diagnosis of anti-GBM disease has been traditionally based on the demonstration of linear deposits of immunoglobulins along the glomerular basement membrane by immunofluorescence (IF) microscopy. However, a kidney biopsy cannot always be easily performed in such ill patients. Recent development of specific enzyme immunoassays for anti-GBM antibody in the serum has made possible a provisional diagnosis without a kidney biopsy. A 46-year-old male patient with hypertension and hepatitis B presented with generalized edema and general weakness. Laboratory findings were compatible with acute renal failure and nephrotic syndrome with positive serum anti-GBM antibodies. After plasmapheresis with steroid pulse therapy, renal biopsy was performed and diagnosed as membranoproliferative glomerulonephritis (MPGN) with granular deposit of Ig G and C3. Follow-up antibody titers were negative. This case demonstrates the possibility of false-positive anti-GBM antibody in the serum. Therefore, enzyme immunoassay for anti-GBM antibody should be used only as a screening or follow-up test in patients that have been confirmed positive by IF microscopy.
Subject(s)
Humans , Male , Middle Aged , Acute Kidney Injury , Anti-Glomerular Basement Membrane Disease , Antibodies , Autoantibodies , Basement Membrane , Biopsy , Early Diagnosis , Edema , False Positive Reactions , Fluorescent Antibody Technique , Follow-Up Studies , Glomerular Basement Membrane , Glomerulonephritis , Glomerulonephritis, Membranoproliferative , Hepatitis B , Hypertension , Immunoenzyme Techniques , Immunoglobulins , Kidney , Mass Screening , Microscopy , Nephritis , Nephrotic Syndrome , Plasmapheresis , SteroidsABSTRACT
Goodpasture's syndrome is a disease that is characterized by hemoptysis, anemia, and glomerulonephritis with renal failure. Goodpasture reported a case of a young man who expired as a result of a pulmonary hemorrhage and glomerulonephritis at the recovery phase after an influenza infection in 1919. In 1958, Stanton et al. described a combined case of these two diseases as Goodpasture's syndrome. Since then, antiglomerular basement membrane antibody(anti-GBM Ab) has been confirmed to play an important role in the mechanism of this syndrome, and it was reported that this syndrome was an autoimmune disease. The triad of alveolar hemorrhage, glomerulonephritis and circulating anti-GBM Ab forms the basis of a diagnosis of Goodpasture's syndrome. When patients are affected by disease, the relief of symptoms can be accomplished by eliminating the anti-GBM Ab from the circulatory system through hemodialysis, plasmapheresis and immunoabsorption. However, the patients usually die from a massive pulmonary hemorrhage when the diagnosis or treatment is delayed. The incidence of Goodpasture's syndrome is common in the western world, but it is extremely rare in Korea with only five cases being reported. In three of these cases, pulmonary hemorrhage and renal failure was the initial manifestation. Therefore, hemodialysis or plasmapheresis were absolutely essential treatments. We report a case of Goodpasture's syndrome in Korea with a normal renal function.