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Anti-Glomerular Basement Membrane Disease: Unexpected Pulmonary Hemorrhage in a Patient Under Immunosuppression and Plasma Exchange-A Case Report
摘要
Anti-glomerular basement membrane disease (Anti-GBM disease) is a rare and life-threatening autoimmune disorder. It usually presents with rapidly progressive glomerulonephritis with or without pulmonary involvement. Early diagnosis and treatment with immunosuppression and plasma exchange may improve patient outcome. In general, it is recommended to monitor the disease activity and response to treatment by detection of serum antibodies. We reported the case of a 67-year-old woman who was admitted for rapidly deteriorating renal function that required urgent hemodialysis therapy. An urgent renal biopsy revealed extensive crescentic glomerulonephritis. Immunofluorescence staining showed linear deposits along the basement membrane compatible with anti-GBM disease, and serum anti-GBM antibody also confirmed the diagnosis. We immediately initiated immunosuppressants and plasma exchange therapy. However, despite the above measures and decreasing antibody titers, the patient developed respiratory failure and pulmonary hemorrhage. With further therapy, the pulmonary hemorrhage resolved, but the patient still required maintenance hemodialysis. This case demonstrated clinical dissociation between disease activity and serum anti-GBM antibody. Therapeutic effect should be assessed not only by serum antibody but also by clinical response of the patient. Initiation of treatment at an early stage of disease may improve the patient's renal outcome.
延伸閱讀
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