A 35-year-old man is taken to the emergency depament because he is coughing up large volumes of blood. He does not have any history suggestive of exposure to tuberculosis, and a chest x-ray film does not show a mass lesion. Chest x-ray films performed on the day of admission and daily for the next several days show asymmetric densities in both lungs that vary in shape and position from film to film. Urinary screening shows hematuria and proteinuria, and the urinary sediment contains cellular and granular casts. Renal biopsy shows rapidly progressive glomerulonephritis with prominent epithelial cell crescents. The mechanism causing this patient’s disease is closest to the mechanism underlying which of the following diseases?
Correct Answer: Bullous pemphigoid
Description: This patient has Goodpasture syndrome, which is a rare, severe autoimmune disease in which antibodies directed against the basement membrane in pulmonary alveoli and renal glomeruli predispose for hemoptysis and progressive renal disease. These patients can die because of exsanguination, asphyxiation by blood, or renal failure. Aggressive immunosuppression coupled with management of complications may be lifesaving. Goodpasture disease is often cited as an example of a Type II hypersensitivity reaction, and a very similar pathologic mechanism produces the blistering disease bullous pemphigoid, in which antibodies are directed against the basement membrane region of the skin. The immunologic basis of Graves disease is stimulation of TSH receptors by anti-receptor antibodies. The immunologic basis of hereditary angioedema is inadequate C1 esterase inhibitor activity. The immunologic basis of rheumatoid ahritis is the presence of immune complexes within joints.
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