Antiglomerular Basement Membrane Disease Follow-up

Updated: May 23, 2018
  • Author: Ramesh Saxena, MD, PhD; Chief Editor: Vecihi Batuman, MD, FASN  more...
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Follow-up

Further Outpatient Care

After discharge from the hospital, patients need a detailed follow-up visit with a nephrologist. Renal function should be closely monitored for progression or recurrence of renal disease. Patients should also have their blood cell counts checked frequently while they are taking cyclophosphamide

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Further Inpatient Care

Patients presenting with respiratory failure may require intubation and mechanical ventilation. Furthermore, patients may present with hemorrhagic shock and require blood transfusion and hemodynamic monitoring. Patients presenting with advanced renal failure may require short- or long-term dialysis.

While in the hospital, patients should also receive supportive care, cindluing the following:

  • Adequate nutrition
  • Prophylaxis for deep vein thrombosis and stress ulcers
  • Good blood pressure control
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Inpatient & Outpatient Medications

See the list below:

  • Methylprednisolone at 7-17 mg/kg/d intravenously for 3 days should be administered to patients with fulminant disease.

  • The starting dose of prednisolone is 1 mg/kg/d. In patients receiving intravenous methylprednisolone, oral prednisolone should be started on the fourth day. Administration should be continued after discharge in tapering doses for one year.

  • Cyclophosphamide should be administered orally at 1 mg/kg/d. This agent should be started once the diagnosis is confirmed and should be continued in an outpatient setting for a total of one year.

  • Calcium carbonate at 500 mg orally 2-3 times/d should be administered for osteoporosis prevention.

  • Double-strength trimethoprim-sulfamethoxazole or equivalent should be administered at a dose of one tablet every other day for prophylaxis of Pneumocystis jiroveci infection.

  • Omeprazole at 20 mg or an equivalent proton pump inhibitor should be administered once or twice a day for prophylaxis of stress ulcers.

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Transfer

Anti-GBM nephritis is a rare disease with a fulminant course if left untreated. The patient should be transferred to a well-equipped tertiary care center for prompt diagnosis and treatment.

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Complications

Patients with severe pulmonary hemorrhage may present with profound hypoxia and respiratory failure. They usually require admission to an intensive care unit for intubation and ventilation support. Furthermore, patients may present with hemorrhagic shock and may require blood transfusion and hemodynamic monitoring.

Patients presenting with advanced renal failure may require short- or long-term dialysis.

Hemorrhagic shock

In addition to respiratory failure, patients with severe pulmonary hemorrhage may present with hemorrhagic shock. They may require blood transfusion and hemodynamic monitoring. They also may require transfusion of other blood products, such as fresh frozen plasma and platelets, to replenish clotting factors and to prevent further bleeding.

Because many of these patients are young and are potential candidates for kidney transplantation, every attempt should be made to use leukocyte-poor blood to prevent allosensitization.

Renal failure

Patients with anti-GBM nephritis usually present with rapidly progressive renal failure. Those with an advanced degree of renal failure may require short- or long-term dialysis.

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Prognosis

Anti-GBM disease is an aggressive disease with a rapidly progressive course. In the early years, the mortality rate was extremely high (90-95%). With the introduction of immunosuppression and plasmapheresis, the prognosis has improved considerably, with patient and renal survival rates of approximately 85% and 60%, respectively.

Both renal survival and patient survival depend on the severity of the disease at the time of presentation. The following reported survival rates underscore the importance of rapid diagnosis and prompt institution of aggressive immunosuppression therapy for patients with Goodpasture syndrome and severe renal failure:

  • Patients who present with a serum creatinine level of less than 500 µmol/L (5.7 mg/dL) have 1-year patient and renal survival rates of 100% and 95%, respectively.

  • Patients who present with a serum creatinine level of more than 500 µmol/L (5.7 mg/dL) but do not require dialysis have 1-year patient and renal survival rates of 83% and 82%, respectively.

  • Patients who present with dialysis-dependent renal failure have 1-year patient and renal survival rates of 65% and 8%, respectively.

  • Importantly, patients with advanced renal disease at the time of presentation (ie, oliguric or dialysis dependent) do not usually respond to plasmapheresis, methylprednisolone, or other immunosuppressive therapy.

Other poor prognostic factors include the following:

  • Extensive crescent formation (>50%)
  • Significant tubular atrophy
  • Interstitial fibrosis or glomerulosclerosis
  • Oliguria or anuria
  • Serum creatinine level of more than 6 mg/dL
  • HLA-DR W2 and HLA-B7 

Nasr and colleagues reported 20 cases of "atypical anti-GBM disease," characterized by bright, linear GBM immunoglobulin deposition but without a diffuse crescentic phenotype, pulmonary involvement, or detectable circulating α3NC1 antibodies. The 1-year patient and renal survival rates were 93% and 85%, respectively, indicating an indolent course for this rare variant. [19]

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Patient Education

Patient education should cover the following risks:

  • Bladder cancer
  • Osteoporosis
  • Opportunistic infections

Risk of bladder cancer

Patients with anti-GBM nephritis receive large doses of cyclophosphamide for a prolonged period. This makes them high-risk candidates for the development of hemorrhagic cystitis and bladder cancer. They should drink large quantities of water to ensure urine output of at least 2 L/d and should avoid becoming dehydrated.

They should also watch for gross hematuria and report it promptly to their physician. Patients should have regular urinalyses to screen for nonglomerular hematuria.

Cigarette smoking has been shown to increase the risk of bladder cancer in patients receiving cyclophosphamide. Therefore, patients should be encouraged to quit smoking.

Risk of osteoporosis

Patients are at a high risk for developing steroid-induced osteoporosis. They should be encouraged to take adequate calcium in their diets and to take additional calcium supplements. Postmenopausal women should also receive estrogen.

Risk of opportunistic infections

Intense immunosuppression can make patients susceptible to opportunistic infections. Therefore, patients should be advised to avoid close contact with ill people. 

They should receive prophylaxis against certain infections (eg, Pneumocystis jiroveci, yeast) and should contact their physician if they develop fever, sore throat, cough with expectoration, or any other signs of infection.

Patient education information sources

For patient education information, see Blood in the UrineKidney Transplant, and Mayo Clinic - Kidney Transplant Information.

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