Goodpasture syndrome

Defined as a triad of glomerulonephritis, pulmonary hemorrhage and antiglomerular membrane antibodies.

Also known as ((Anti-GBM disease))

Organ specific autoimmune disorder characteized by rapidly progressive glomerulonephritis, pulmonary hemorrhage and linear deposits of antibodies along the basement membrane.

Capillaries become inflamed as a result of damage to the basement membrane by antibodies to alpha 3 NC1 domain of type IV collagen.

Only 15% have a positive antineutrophilic cytoplasmic antibody (ANCA), but anti-glomerular basement membrane antibodies may be present.

Goodpasture disease is diagnosed in 1 per million persons each year.

Autoimmune process produced when the patient’s immune system attacks cells presenting the Goodpasture antigen, a type II hypersensitivity reaction, found in the kidney and lung.

First indication of the process may be associated with vague symptoms such as fatigue, nausea, dyspnea or pallor.

Often present with abrupt onset of all oliguria, hematuria and acute renal insufficiency.

May present with hemoptysis, dysuria, hematuria or proteinuria.

Some patients have both lung and kidney symptoms, some may present with one organ system abnormality alone.

Lung symptoms usually present before kidney problems by days to months.

Lung involvement occurs in patients who smoke.

Circulating autoantibodies bind to the noncollagenous-1 (NC1) domain of type IV collagen n the glomerular basment membrane.

Age distribution is bimodal.

Young men who smoke present with pulmonary-renal syndrome.

Women in the sixth and seventh decades present with disease limited to the kidney.

1-year survival rate of 79-93% and 1-years renal survival rates of only 20-40%

Patients who present with severe renal failure, in general, do not have renal recovery with modern treatment.

Most patients present with rapidly progressive glomerulonephritis.

Kidney involvement in Goodpasture’s syndrome is usually not diagnosed until a rapid deterioration of the disease occurs with impairment of renal function can be lost in a matter of days with rapidly progressive crescentic glomerulonephritis.

Early diagnosis important as treatment can preserve renal function and prevent death.

Lung manifestations may be minimal with cough, and minimal dyspnea and may be present for years before becoming more serious.

Lung involvement can lead to respiratory insufficiency which may be of rapid onset and require intensive management.

Hemoptysis may be the initial presentation.

Chronic loss of blood via the lung may lead to iron deficiency anemia.

Causes include inherited predisposition, hydrocarbon solvents, weed killer Paraquat and viral infections.

Diagnosis rests on the detection of the antibody in the blood and the deposition of IgG on the GBM on renal biopsy.

Treatment in severe disease includes high dose steroids, cyclophosphamide and plasmapheresis.

Mortality with present day treatment is 10-20%.

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