 Goodpasture syndrome
Definition Alternative Names Causes, incidence, and risk factors Symptoms Signs and tests Treatment Expectations (prognosis) Complications Calling your health care provider Prevention References DefinitionGoodpasture syndrome is a rare disease that involves rapidly progressive kidney failure along with lung disease. However, some forms of the disease involve just the lung or kidney, not both. Alternative Names Anti-glomerular basement membrane antibody disease; Rapidly progressive glomerulonephritis with pulmonary hemorrhage; Pulmonary renal syndrome; Glomerulonephritis - pulmonary hemorrhage Causes, incidence, and risk factorsGoodpasture syndrome is an autoimmune disorder, a condition that occurs when the immune system mistakenly attacks and destroys healthy body tissue. Persons with this syndrome develop substances that attack a protein called collagen in the tiny air sacs in the lungs and the filtering units (glomureli) of the kidney. These substances are called anti-glomerular basement membrane antibodies. Glomerular basement membrane is a part of the kidneys that helps filter waste and extra fluid from the blood. Anti-glomerular basement membrane are antibodies against this membrane. They can lead to kidney damage. Sometimes the disorder is triggered by a viral respiratory infection or by breathing in hydrocarbon solvents. In such cases, the immune system may attack organs or tissues because it mistakes them for these viruses or foreign chemicals. The immune system's faulty response causes bleeding in the air sacs and inflammation in the kidney's filtering units. Men are eight times more likely to be affected than women. The disease most commonly occurs in early adulthood. SymptomsSigns and testsA physical examination may reveal signs of high blood pressure and fluid overload. The health care provider may hear abnormal heart and lung sounds when listening to the chest with a stethoscope. Urinalysis results are usually abnormal, and shows blood and protein in the urine. Abnormal red blood cells may be seen. The following tests may also be done: TreatmentThe main goal is to remove the antibodies from the blood. A treatment called plasmapheresis removes blood from the body and replaces it with fluid, protein, or donated plasma. Powerful anti-inflammatory and cytotoxic medicines (such as prednisone or cyclophosphamide) may be needed. If kidney failure becomes severe, dialysis may be needed. Kidney transplantation may be need if you have permanent loss of kidney function. The transplant is usually not done until the level of antibodies drop. Expectations (prognosis)An early diagnosis is very important. The patient's outlook is much worse if the kidneys are already severely damaged when treatment begins. ComplicationsCalling your health care providerCall for an appointment with your health care provider if the amount of urine you produce drops, or if you have any other symptoms of Goodpasture syndrome. PreventionNever sniff glue or siphon gasoline with your mouth, which expose the lungs to hydrocarbon solvents and can cause the disease. ReferencesAppel GB, Radhakrishnan J, D'Agatis V. Secondary Glomerular Disease. In: Brenner BM, ed. Brenner: Brenner and Rector's The Kidney. 8th ed. Philadelphia, Pa: Saunders Elsevier; 2007: chap.31. Physician ReferenceInternational Classification of Diseases, 9th Revision (ICD9)446.21
Review Date: 7/11/2008
Reviewed By: Parul Patel, MD, Private Practice specializing in Nephrology and Kidney and Pancreas Transplantation, Affiliated with California Pacific Medical Center, Department of Transplantation, San Francisco, CA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
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