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Review Question - QID 100997

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QID 100997 (Type "100997" in App Search)
A 47-year-old-man presents with hemoptysis, RBC casts in the urine, and increased serum concentrations of BUN and creatinine. Kidney biopsy with immunofluorescence was performed, and the results are shown in Figure A. What glomerular changes would be expected if the sample were evaluated under light microscopy?
  • A

"Wire-looping" of the capillaries

7%

15/223

Crescentic changes

64%

142/223

Hypercellular glomeruli

12%

26/223

Segmental sclerosis

4%

9/223

Apple-green birefringence with congo red stain

9%

20/223

  • A

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The patient presents with signs and symptoms of Goodpasture syndrome, which is marked by crescentic changes on renal biopsy.

Goodpasture syndrome is a disease characterized by antibodies that form against the basement membrane of the glomeruli and alveoli, which manifest as nephritic syndrome and alveolar hemorrhage, respectively. Immunofluorescence of the glomerulus shows a characteristic linear pattern. Light microscopy reveals proliferation of glomerular parietal epithelium with fibrin, monocytes, and macrophages that form a crescentic pattern that expands into the Bowmans capsule. Goodpasture syndrome is one type of rapidly progressive glomerulonephritis, a group of nephritic syndromes all characterized by this crescentic formation. Granulomatosis with polyangiitis (GPA) is a systemic vasculitis that presents similarly to Goodpasture and will demonstrate crescentic glomeruli on biopsy, however GPA has no characteristic immuofluorescence staining (no immune depositions).

Cui and Zhao discuss advances in human antiglomerular basement membrane disease. Antiglomerular basement membrane (anti-GBM) disease is an autoimmune disorder that mostly presents as raised titers of antibodies against the GBM, rapidly progressive glomerulonephritis and pulmonary hemorrhage. The disease is caused by antibodies against noncollagenous domain of the a3 chain of type IV collagen, which contains the epitopes E(A) and E(B).

Agrawal and Swartz discuss acute renal failure. Acute renal failure occurs in 5% of hospitalized patients. Etiologically, this common condition can be categorized as prerenal, intrinsic, or postrenal. Most patients have prerenal acute renal failure or acute tubular necrosis (a type of intrinsic acute renal failure that is usually caused by ischemia or toxins). In certain situations, such as when a patient has glomerular disease, microvascular disease or obstructive disease, rapid diagnosis and treatment are necessary to prevent permanent renal damage.

Figure A depicts the classic appearance of anti-glomerular basement membrane antibodies as seen on immunofluorescence. Illustration A shows side by side crescentic changes on light microscopy and the immunofluorescence findings seen in Goodpasture syndrome. Illustration B shows wire-looping of capillaries in a lupus patient. Illustration C shows apple-green birefringence with congo red stain as seen in amyloidosis.

Incorrect Answers:
Answer 1: "Wire-looping" is characteristic of SLE in diffuse proliferative glomerulonephritis (Illustration B).

Answer 3: Hypercellular glomeruli is typical of acute poststreptococcal glomerulonephritis.

Answer 4: Segmental sclerosis is typical of focal segmental glomerulosclerosis, a nephrotic disorder.

Answer 5: Apple-green birefringence with congo red stain is characteristic of amyloidosis when viewed under polarized light (Illustration C).

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