Goodpasture’s Syndrome
-A rapidly progressive syndrome of
glomerulonephritis and pulmonary hemorrhage, in association with antibodies to
glomerular basement membrane (anti-GBM) type IV collagen, detectable in plasma
by radioimmunoassay and by immunofluorescence techniques on muscle biopsy.
-These antibodies cross-react with the alveolar basement membrane, although those individuals with lung hemorrhage are
usually smokers, and those with isolated anti-GBM nephritis are non-smokers.
-The term is often applied more
loosely, to any disease with pulmonary hemorrhage and glomerulonephritis,
whether or not anti-GBM antibodies are present.
-Systemic vasculitides such as PAN
and Wegener’s granulomatosis cause lung hemorrhage and renal failure, but
without the antibodies.
Preoperative Findings:
1. Patient presents with cough, dyspnea,
hemoptysis (that can be massive), and anemia.
2. The pulmonary lesions proceed to
interstitial fibrosis, with hemosiderin deposits. Lung function tests show a
restrictive type of abnormality.
3. Glomerulonephritis, which usually
follows or coincides with pulmonary lesions, may produce proteinuria, hematuria,
and casts. The end result is renal failure.
4. Treatment may be with corticosteroids (prednisone) for pulmonary hemorrhage, cytotoxic drugs (cyclophosphamide) to stop renal damage, and
plasmapheresis to remove antibodies.
Anesthetic Problems:
1. Poor respiratory function with
hypoxemia and respiratory alkalosis.
2. Pulmonary hemorrhage that may, on
occasions, be life-threatening.
3. Impaired renal function and
sometimes renal failure.
4. Hypochromic anemia and a high
ESR.
5. Patients may be receiving
immunosuppressives or steroids, or undergoing plasma exchange, to reduce the antibody titer. Plasma exchange may reduce levels of plasma
cholinesterase.
Anesthetic Management:
1. Preoperative assessment of lung
function and, in particular, blood gases. Elective pulmonary surgery should not
be undertaken during periods of active hemorrhage.
2. Assessment of renal function and
appropriate management.
3. The management of a successful pregnancy has been described. It requires regular assessment of pulmonary and renal function. The use of steroids was associated with hyperglycemia, which needed treatment with insulin. Plasmapheresis was required.
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