Goodpasture syndrome: pulmonary-renal syndrome
Goodpasture syndrome is an anti-glomerular basement membrane (anti-GBM) disease. It is an autoimmune disease characterized by damage to the alveolar and renal glomerular basement membranes by the anti-GMB antibody. It is a pulmonary-renal syndrome.
Goodpasture syndrome characteristically has -
- Pulmonary hemorrhage - hemoptysis
- Glomerulonephritis-hematuria
- circulating anti-glomerular basement membrane antibodies
Clinical presentation
The patient may have -
- cough
- dyspnea
- hemoptysis
- hematuria
- advanced cases might present with oliguria and symptoms of uremia
In most cases, Pulmonary manifestations are usually the cause of presentation.
Pathology
It is a Type II hypersensitivity reaction where antibodies are directed primarily against renal glomerular basement membrane’s alpha-3 chain of collagen type IV. This autoantibody cross-reacts with alveolar basement membrane also.
The antibodies bind to the glomerular basement membrane and lead crescent formation. Rapidly progressive glomerulonephritis (crescentic GN) and necrotizing hemorrhagic interstitial pneumonitis are characteristic of this disorder.
The alveolar damage causes alveolar hemorrhage, dyspnea and hemoptysis (coughing blood).
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Diagnosis
Blood test – anti-GBM antibodies are seen.
Urine analysis – microscopic hematuria (dysmorphic RBCs in urine microscopy), RBC casts or Gross hematuria
Renal biopsy – proliferative, crescentic glomerulonephritis, renal biopsy is preferred because it is more yielding.
Pulmonary biopsy – alveolar necrosis, alveolar hemorrhage and hemosiderin-laden macrophages
Immunofluorescence - the immunofluorescent studies of renal or lung tissue show linear deposits of immunoglobulins at glomerular basement membrane and alveolar septa
Treatment and prognosis
The overall prognosis is poor
- Plasmapheresis removes anti-GBM antibodies and improves survival
- Immunosuppressants such as corticosteroids and, Cyclophosphamide reduce antibody formation.
Case scenario
A 36-year-old man presents to the physician complaining of chronic cough, which is accompanied by hemoptysis for past two months. He has no history of smoking or any significant past medical illness blood tests show elevated blood urea nitrogen and creatinine levels. Urine analysis reveals microscopic hematuria and +++ proteinuria.
Which of the following is most likely to be associated with his condition?
- Anti-mitochondrial antibodies
- Antibodies against alpha-3 chain of collagen type IV
- Anti-smooth muscle antibodies
- Defect in alpha-5 chain of collagen IV
Revision for today Polyhydramnios: Excessive Amniotic Fluid - Creative Med Doses
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