Antiglomerular Basement Membrane Disease Clinical Presentation

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

History

Patients with anti–glomeurlar basement membrane (anti-GBM) disease can present with glomerulonephritis alone or with accompanying pulmonary hemorrhage. [11] Although pulmonary hemorrhage may be minor, it is often severe and life threatening. Pulmonary hemorrhage occurs more frequently in young men, whereas anti-GBM nephritis without lung involvement tends to occur more frequently in women in their seventh decade of life. [12]

The disease may begin with either renal or pulmonary manifestations. Usually, both organs are involved more or less simultaneously. [13] However, in some cases, involvement of the second organ may not occur until as much as a year later.

Prodromal features are as follows:

  • 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 are as follows:

  • The onset of pulmonary hemorrhage may be insidious, with signs 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 are as follows:

  • Patients usually present with abrupt onset of oliguria or anuria. Hematuria or the passage of tea-colored urine is usually observed.

  • Rarely, the renal involvement progresses more insidiously and the patient remains asymptomatic initially; the glomerulonephritis progresses slowly until the development of uremic symptoms.

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Physical

Findings are as follows:

  • 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. Both genetic susceptibility and environmental features are typically involved.

Genetic susceptibility includes the following:

  • 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 include the following:

  • 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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