Long-Term Outcome of Patients with Kidney Damage Due to Goodpasture Disease Who Receive Very Intensive Treatment

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The summary below is from the full report titled "Long-Term Outcome of Anti–Glomerular Basement Membrane Antibody Disease Treated with Plasma Exchange and Immunosuppression." It is in the 5 June 2001 issue of Annals of Internal Medicine (volume 134, pages 1033-1042). The authors are JB Levy, AN Turner, AJ Rees, and CD Pusey.

What is the problem and what is known about it so far?

The Goodpasture syndrome (also known as anti–glomerular basement membrane, or anti-GBM, disease) causes kidney failure and, sometimes, bleeding in the lungs. It has been thought that patients who develop severe kidney failure from the Goodpasture syndrome do not usually recover their kidney function, but few large, long-term studies of this condition have been done. The Goodpasture syndrome is probably due to an autoimmune process. This means that the immune system, which should attack only foreign substances such as bacteria, attacks a person's own body tissues. Treatments for the Goodpasture syndrome include plasma exchange, which filters the blood to remove immune substances, and drugs that suppress the immune system, such as prednisone and cyclophosphamide.

Why did the researchers do this particular study?

The researchers sought to describe what happens over many years to patients with severe kidney failure from the Goodpasture syndrome, and to see whether certain factors or treatments are associated with better outcomes for these patients.

Who was studied?

The researchers studied 71 patients who had developed severe kidney failure because of the Goodpasture syndrome and who received treatment with plasma exchange, prednisone, and cyclophosphamide at a medical center in the United Kingdom.

How was the study done?

The researchers recorded information from the medical records of all 71 patients on their condition when the disease was first diagnosed, the treatments given, and how patients did over time. In particular, the researchers were interested in whether patients lived and whether they avoided end-stage kidney failure that required dialysis. Dialysis is a procedure that uses a special machine to filter the blood in the way that normal kidneys do.

What did the researchers find?

Patients were followed for 12 to 289 months (the average was about 90 months). Patients whose kidney damage was less severe when they first sought care did better than those with more severe kidney damage; 74% of the former patients did not need dialysis at last follow-up compared with 69% of the latter patients. However, even patients with severe kidney damage could recover when treated. Patients who needed dialysis when their disease was first diagnosed fared worst; only 65% survived for 1 year, and only 5% of the surviving patients did not need dialysis by the end of the study.

What were the limitations of the study?

Because all patients received the same general treatment, the study could not determine whether certain treatments result in better outcomes than others. In addition, the researchers were not able to follow all patients for similar lengths of time, so the information available on very long-term follow-up (25 years) is based on only a few patients.

What are the implications of the study?

Some patients with severe kidney disease from the Goodpasture syndrome recover kidney function when they are treated with plasma exchange and drugs that suppress the immune system.