Goodpasture Syndrome

Incidence of 1 case per million people per y.

Accounts for 20% of cases of RPGN or crescentic glomerulonephritis.

In terms of bimodal age distribution, more common in males 20–30 y of age and females 60–70 y of age.

Anemia from recurrent or persistent intrapulmonary hemorrhage

Hypoxia or hypoxic respiratory failure in cases of massive intrapulmonary hemorrhage

Rapidly progressive renal failure or uremia

Significant third-space fluid loss secondary to proteinuria

Pts with active pulmonary hemorrhage may require mechanical ventilation in the postop period for hypoxic respiratory failure.

Renal failure will alter drug pharmacokinetics and require adjustment of dosing or choice of anesthetic drugs.

Anemia secondary to iron deficiency from repeated pulmonary hemorrhage, as well as anemia related to chronic kidney disease.

Opportunistic infections such as pneumocystitis pneumonia in pts receiving immunosuppressive therapy.

Volume overload in pts with severe renal insufficiency.

Rare, autoimmune, renal-pulmonary syndrome caused by autoantibodies directed against the glomerular basement membrane (anti-GBM antibodies).

Major cause of RPGN, defined as a ≥50% loss of renal function (as quantified by glomerular filtration rate) over a 3-mo period.

Usually presents with constitutional symptoms (night sweats, malaise), chronic cough progressing to hemoptysis, and hematuria or foamy urine (from proteinuria).

Pulmonary symptoms may be episodic in nature, related to discreet episodes of pulmonary hemorrhage. Each episode may be severe and can lead to life-threatening respiratory failure requiring mechanical ventilation.

Pts may present for elective surgery, such as placement of dialysis access, or require kidney transplantation related to loss of renal function caused by progression of Goodpasture syndrome.

Autoimmune disease caused by anti-GBM antibodies. These antibodies also have affinity for the alveolar basement membrane, causing the renal-pulmonary syndrome.

In most pts, the anti-GBM antibodies are directed against a specific subunit within the alpha 3 chain of type IV collagen.

In a classic type II hypersensitivity reaction, anti-GBM antibodies bind to target epitopes and activate the complement system, leading to cellular damage.

Cellular damage within the glomeruli leads to glomerulonephritis with proteinuria and hematuria. In RPGN, crescentic scarring of the glomeruli can be seen on kidney biopsy.

Within the alveoli, cellular damage leads to a breakdown in the barrier between airspaces and blood vessels, leading to diffuse alveolar hemorrhage.

Evidence of a genetic predisposition. A positive association among Goodpasture syndrome, pernicious anemia, systemic lupus erythematosus, and Sjogren syndrome and HLA-DR15 has been demonstrated.

All other known risk factors are pulmonary insults, such as exposure to hydrocarbon fumes, exposure to metal dusts, inhalation of smoke or cocaine, or viral infections. These insults may lead to damage of the alveolar basement membrane and exposure of type IV collagen to the immune system, leading to the development of autoantibodies.