Goodpasture's syndrome

Goodpasture's syndrome: Excerpt from Professional Guide to Diseases (Eighth Edition)

In Goodpasture’s syndrome, hemoptysis and rapidly progressive glomerulonephritis follow the deposition of antibody against the alveolar and glomerular basement membrane (GBM). The prognosis improves with aggressive immunosuppressive and antibiotic therapy along with dialysis or renal transplantation.

Causes and incidence

The cause of Goodpasture’s syndrome is unknown. Although some cases have been associated with exposure to hydrocarbons or type 2 influenza, many have no precipitating events. The high incidence of human leukocyte antigen DRW2 in these patients suggests a genetic predisposition. Abnormal production and deposition of antibody against GBM and alveolar basement membrane activate the complement and inflammatory responses, resulting in glomerular and alveolar tissue damage.

This syndrome may occur at any age but is most common in males between ages 20 and 30. A second peak incidence occurs between ages 50 and 70, with males and females in this age group affected equally.

Signs and symptoms

Goodpasture’s syndrome may initially cause malaise, fatigue, and pallor associated with severe iron deficiency anemia. Pulmonary findings range from slight dyspnea and cough with blood-tinged sputum to hemoptysis and frank pulmonary hemorrhage. Subclinical pulmonary bleeding may precede overt hemorrhage and renal disease by months or years. Usually, renal findings are subtler, although some patients note hematuria and peripheral edema.

Diagnosis

Confirmation of Goodpasture’s syndrome requires measurement of circulating anti-GBM antibody by radioimmunoassay and linear staining of GBM and alveolar basement membrane by immunofluorescence.

Immunofluorescence of alveolar basement membrane shows linear deposition of immunoglobulin as well as complement 3 and fibrinogen. Immunofluorescence of GBM also shows linear deposition of immunoglobulin combined with detection of circulating anti-GBM antibody. This finding distinguishes Goodpasture’s from other pulmonary-renal syndromes, such as Wegener’s granulomatosis, polyarteritis, and systemic lupus erythematosus.

A lung biopsy reveals interstitial and intra-alveolar hemorrhage with hemosiderin-laden macrophages. Chest X-ray reveals pulmonary infiltrates in a diffuse, nodular pattern, and renal biopsy commonly shows focal necrotic lesions and cellular crescents.

Creatinine and blood urea nitrogen (BUN) levels typically increase two to three times normal. Urinalysis may reveal red blood cells and cellular casts, which typify glomerular inflammation. Granular casts and proteinuria may also be observed.

Treatment

Treatment aims to remove antibody by plasmapheresis and to suppress antibody production with immunosuppressive drugs, such as cyclophosphamide, to stop attacks by immune cells on the kidneys and lungs. Patients with renal failure may benefit from dialysis or transplantation. Aggressive ultrafiltration helps relieve pulmonary edema that may aggravate pulmonary hemorrhage. High-dose I.V. steroids also help control pulmonary hemorrhage.

Special considerations

❑ Promote adequate oxygenation by elevating the bed’s head and administering humidified oxygen. Encourage the patient to conserve his energy. Assess respirations and breath sounds regularly; note sputum quantity and quality.

❑ Monitor vital signs, arterial blood gases, hematocrit, and coagulation studies.

❑ Transfuse blood and administer steroids as ordered. Observe closely for drug adverse effects.

❑ Assess renal function by monitoring symptoms, intake and output, daily weights, creatinine clearance, and BUN and creatinine levels.

❑ Tell the patient and his family what signs and symptoms to expect and how to relieve them. Carefully describe other treatment measures such as dialysis.

Book Source Details

• Book Title: Professional Guide to Diseases (Eighth Edition)
• Author(s): Springhouse
• Year of Publication: 2005
• Copyright Details: Professional Guide to Diseases (Eighth Edition), Copyright © 2005 Lippincott Williams & Wilkins.

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• Back to disease: Goodpasture syndrome: Introduction
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.

More About This Book: Title: Professional Guide to Diseases (Eighth Edition) Authors: Springhouse Publisher: Lippincott Williams & Wilkins Copyright: 2005 ISBN: 1-58255-370-X

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