40. Goodpasture Syndrome
Definition
Goodpasture syndrome is an autoimmune process composed of acute glomerulonephritis in conjunction with pulmonary alveolar hemorrhage and antiglomerular basement membrane antibodies.
Incidence
The incidence of Goodpasture syndrome is approximately 1:100,000. The disease is reported in all races, but occurs most frequently among Caucasians. Males are affected more than females at a ratio of 2.9:1.
Etiology
The cause of Goodpasture syndrome is the production of antiglomerular basement membrane antibodies, frequently as the result of exposure to an environmental insult in genetically predisposed people. The environmental triggers include cigarette smoke, hydrocarbon inhalation, and viral infections.
Signs and Symptoms
• Azotemia
• Bronchial breathing
• Cough
• Cyanosis
• Dyspnea (exertional)
• Edema
• Fatigue/weakness
• Fevers/chills/diaphoresis
• Gross hematuria
• Hemoptysis
• Inspiratory crackles
• Proteinuria
• Tachypnea
Medical Management
Treatment of Goodpasture syndrome involves two simultaneous interventions: (1) removing existing pathogenic antibodies and (2) preventing subsequent production of new antibodies. Antibody removal is best achieved via plasma exchange. Current recommendations for an adult patient present two options: either daily exchanges for 14 consecutive days or every third day for 1 month. A plasma exchange session involves total volumes of 3 L to 4 L, with volume replacement consisting of albumin and fresh frozen plasma.
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