Goodpasture Syndrome (Anti-GBM Disease)

Two organs, one antibody - The picture is rapidly progressive glomerulonephritis plus hemoptysis. Younger patients tend to present with both at once and critically ill; patients over 50 more often have glomerulonephritis alone and follow a less severe course. Know which phenotype is in front of you before you build a plan.

Quantify the pulmonary bleed - Hemoptysis ranges from life-threatening bleeding to a diffuse hemorrhage only found on careful evaluation. KCO is raised in profuse pulmonary hemorrhage because free red cells lining the alveoli take up carbon monoxide, so a high DLCO in this patient is not reassuring, it means blood in the alveoli. Pair that with chest imaging and a hematocrit trend.

Renal failure drives drug handling - Crescentic glomerulonephritis progresses to acute renal failure and urgent hemodialysis is often needed for standard indications. Get a current creatinine, potassium and the dialysis schedule. Check the potassium before giving succinylcholine, and lean on agents that don't depend on renal clearance.

Fluid balance is a knife edge - The kidney can't offload a volume load and the lung is already bleeding, so a liberal crystalloid plan buys pulmonary edema on top of alveolar hemorrhage. Run even-to-dry, guide replacement by stroke volume or arterial waveform rather than a fixed maintenance rate, and coordinate ultrafiltration timing with nephrology.

Ventilating a bleeding lung - Intubation for respiratory failure is often necessary. Blood in the airway plugs the tube and behaves like a shunt, so plan for repeated suctioning, choose a tube large enough for bronchoscopic access, and expect hypoxemia that FiO2 alone won't fix. If bleeding localizes to one side, lung isolation protects the other.

Heavy immunosuppression - Treatment is prednisone plus cyclophosphamide plus daily plasmapheresis. Acute life-threatening alveolar hemorrhage gets IV methylprednisolone pulse 1 g/day for 3 days, then 1 to 1.5 mg/kg orally tapering. Cyclophosphamide starts at 2 mg/kg orally, adjusted to hold the white count near 5,000. Treat this as a profoundly immunosuppressed patient: strict asepsis for every line and block, and take a fever seriously.

Time the case around plasmapheresis - Daily plasmapheresis runs until anti-GBM antibodies are undetectable, with steroid and cyclophosphamide continuing 3 to 6 months to remission. Place elective surgery off a pheresis day where you can. If a case must follow one, send a coagulation panel, platelet count and fibrinogen before neuraxial or a large incision.

Protect the access - These patients need vascular access for hemodialysis and plasmapheresis, sometimes placed surgically. Keep the NIBP cuff, IVs and arterial line off a limb with a fistula or tunneled catheter, and don't consume the last good vein for a case-day peripheral.

Watch the double-positive patient - A subgroup carries both ANCA and anti-GBM antibodies, mostly men aged 60-70, and a purpuric rash on exam should send you looking for coexisting ANCA-associated vasculitis. That patient may also carry the destructive upper- and central-airway lesions of granulomatosis with polyangiitis (GPA), so examine the airway rather than assuming a pure pulmonary-renal picture.

Transplant and recurrence - Patients who stay dialysis-dependent may come for renal transplant, which requires anti-GBM titers negative for at least six months beforehand. Recurrence after transplant is rare in the current era, and recurrent disease generally responds to the same treatment as the initial episode.

Goodpasture syndrome is anti-glomerular basement membrane (anti-GBM) disease involving both kidney and lung. Circulating IgG autoantibodies, predominantly IgG-1, target the alpha-3 NC1 domain of type IV collagen in basement membrane, activate complement, and produce rapidly progressive crescentic glomerulonephritis - immunofluorescence shows a bright linear band of IgG and C3 along the glomerular basement membrane. The alveolar basement membrane carries the same collagen target, but healthy alveolar endothelium keeps antibody out; an insult that increases alveolar-capillary permeability lets antibody through and produces diffuse alveolar hemorrhage.

Age distribution is bimodal, 20-30 and 60-70, with younger patients more often presenting with simultaneous renal and pulmonary disease and critically ill. Perioperatively you are managing two failing organs at once: a kidney losing function quickly and a lung bleeding into its own alveoli.