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Review Question - QID 101027

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QID 101027 (Type "101027" in App Search)
A 25-year-old male visits his primary care physician with complaints of hemoptysis. Serum blood urea nitrogen and creatinine are elevated, blood pressure is 160/100 mm Hg, and urinalysis shows hematuria and RBC casts. A 24-hour urine excretion yields 1 gm/day protein. A kidney biopsy is obtained, and immunofluorescence shows linear IgG staining in the glomeruli. Which of the following antibodies is likely pathogenic for this patient’s disease?

Anti-DNA antibody



Anti-neutrophil cytoplasmic antibody (C-ANCA)



Anti-neutrophil perinuclear antibody (P-ANCA)



Anti-glomerular basement membrane antibody (Anti-GBM)



Anti-phospholipid antibody



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The patient’s clinical picture, laboratory data, urinalysis, and light microscopy are consistent with Goodpasture syndrome. Goodpasture syndrome is a type II hypersensitivity reaction characterized by autoantibodies to the glomerular basement membrane (anti-GBM).

Goodpasture syndrome is a type of rapidly progressive crescentic glomerulonephritis (RPGN) in which patients present with acute renal failure as well as hemoptysis. Pulmonary symptoms result from antibodies to the alveolar basement membrane. The linear IgG staining is the classic pattern seen when viewing immunoflouresence of glomeruli in affected patients as a result of antibodies to the glomerular basement membrane.

Agrawal et al. review causes of renal failure, including glomerulonephritis. They characterize glomerulonephritis as the combination of hypertension, proteinuria and hematuria, and report that most types are associated with chronic renal disease. However, the two types of glomerulonephritis that can cause acute renal failure are RPGN and acute proliferative glomerulonephritis (APGN). APGN occurs in individuals with bacterial endocarditis or other postinfectious conditions.

Sanders et al. review pulmonary-renal syndrome, the combination of pulmonary hemorrhage and acute renal failure. The authors note that while the overall incidences of diseases causing pulmonary-renal syndrome are very low in the total population (10 to 20 per million per year), as many as 60% of individuals presenting with the symptoms of pulmonary hemorrhage and acute renal failure will be diagnosed with Goodpasture syndrome or anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (Wegener).

Illustration A compares the findings of a normal patient with one affected by Goodpasture syndrome. The top portion shows alveolar light microscopy. The bottom shows immunofluorescence of the glomerular basement membrane.
Illustration B shows a histologic image of rapidly progressive cresentic glomerulonephritis.

Incorrect Answers:
Answer 1: Anti-DNA antibody is associated with the diffuse proliferative GN of SLE.
Answer 2: Anti-neutrophil cytoplasmic antibody (C-ANCA) is associated with Wegener granulomatosis.
Answer 3: Anti-neutrophil perinuclear antibody (P-ANCA) is associated with microscopic polyarteritis.
Answer 5: Anti-phospholipid antibody is associated with an autoimmune, hypercoagulable state.

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