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Antiglomerular Basement Membrane Disease Clinical Presentation

  • Author: Ramesh Saxena, MD, PhD; Chief Editor: Vecihi Batuman, MD, FACP, FASN  more...
 
Updated: Dec 04, 2015
 

History

Patients with anti-GBM nephritis can present with glomerulonephritis alone or with accompanying pulmonary hemorrhage.[8] Although pulmonary hemorrhage may be minor, it is often severe and life threatening. Pulmonary hemorrhage occurs more frequently in young adult males, whereas anti-GBM nephritis without lung involvement tends to occur more frequently in women in their seventh decade of life.[9]

The disease may begin with either renal or pulmonary manifestations. Usually, both organs are involved more or less simultaneously.[10] However, in several cases, the interval to the second organ's involvement may be prolonged up to a year.

  • Prodromal period
    • In 25-30% of patients, a prodromal period of flulike illness occurs.
    • In approximately 5% of patients, arthralgia, myalgia, and arthritis are prominent features.
  • Pulmonary manifestations
    • The onset of pulmonary hemorrhage may be insidious, with symptoms such as anemia, pallor, weakness, lethargy, dyspnea upon exertion, and, sometimes, dry cough.
    • In some cases, onset is acute and includes fever, massive hemoptysis, acute respiratory failure, asphyxia, and death; however, in many cases, the symptoms, including hemoptysis, dyspnea, cough, fever, tachycardia, and fatigue, may be present intermittently for weeks to months before the diagnosis is established.
  • Renal manifestations
    • The patient usually presents with an abrupt onset of oliguria or anuria. Hematuria or the passage of tea-colored urine is usually observed.
    • Rarely, the patient's renal involvement is more insidious in onset and he or she remains asymptomatic, progressing slowly until the development of uremic symptoms.
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Physical

See the list below:

  • Physical examination in the acute stage of the disease reveals respiratory distress, tachycardia, and cyanosis.
  • The patient usually appears pale because of anemia.
  • In severe cases, the patient may be in hemorrhagic shock and in respiratory failure, thus requiring volume resuscitation and ventilatory support, respectively.
  • Chest examination may reveal fine rales and dullness to percussion over the affected lung areas.
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Causes

The disease is caused by autoantibodies directed against the NC1 domain of the alpha-3 chain of type IV collagen.

  • Genetic susceptibility
    • Anti-GBM disease shows a strong association with HLA-DR2.
    • Further molecular genetics studies of HLA-DR2 reveal that the association of anti-GBM nephritis is with HLA-DRB1 alleles (HLA-DRB1 1501 and 1502 alleles), HLA-DQA1 01 alleles, and HLA-DQB1 06 alleles.
    • Anti-GBM nephritis is major histocompatibility complex–restricted. HLA-DRB1*1501 and 1502 alleles increase the susceptibility, while HLA-DR1 and HLA-DR7 are protective.
  • Environmental factors
    • A number of studies suggest a strong association between pulmonary hemorrhage and smoking.
    • Pulmonary hemorrhage may also be associated with exposure to hydrocarbons or other agents (eg, respiratory pathogens).
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Contributor Information and Disclosures
Author

Ramesh Saxena, MD, PhD Professor, Department of Internal Medicine, Division of Nephrology, University of Texas Southwestern Medical Center

Ramesh Saxena, MD, PhD is a member of the following medical societies: International Society for Peritoneal Dialysis, National Kidney Foundation, Texas Medical Association, American Society of Nephrology, International Society of Nephrology

Disclosure: Received honoraria from e-medicine for authoring review articles.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Christie P Thomas, MBBS, FRCP, FASN, FAHA Professor, Department of Internal Medicine, Division of Nephrology, Departments of Pediatrics and Obstetrics and Gynecology, Medical Director, Kidney and Kidney/Pancreas Transplant Program, University of Iowa Hospitals and Clinics

Christie P Thomas, MBBS, FRCP, FASN, FAHA is a member of the following medical societies: American College of Physicians, American Heart Association, American Society of Nephrology, Royal College of Physicians

Disclosure: Nothing to disclose.

Chief Editor

Vecihi Batuman, MD, FACP, FASN Huberwald Professor of Medicine, Section of Nephrology-Hypertension, Tulane University School of Medicine; Chief, Renal Section, Southeast Louisiana Veterans Health Care System

Vecihi Batuman, MD, FACP, FASN is a member of the following medical societies: American College of Physicians, American Society of Hypertension, American Society of Nephrology, International Society of Nephrology

Disclosure: Nothing to disclose.

Additional Contributors

Chike Magnus Nzerue, MD, FACP Professor of Medicine, Associate Dean for Clinical Affairs, Meharry Medical College

Chike Magnus Nzerue, MD, FACP is a member of the following medical societies: American Association for the Advancement of Science, American College of Physicians, American College of Physicians-American Society of Internal Medicine, American Society of Nephrology, National Kidney Foundation

Disclosure: Nothing to disclose.

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Light microscopy of kidney biopsy specimen from a patient with antiglomerular basement membrane nephritis showing extensive crescent formation and the collapse of glomerular tuft.
Immunofluorescent examination of a kidney biopsy specimen from a patient with antiglomerular basement membrane nephritis showing a linear deposition of immunoglobulin G along the glomerular basement membrane.
 
 
 
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