Pediatric Anti-GBM Disease (Goodpasture Syndrome) Clinical Presentation

Updated: Mar 05, 2015
  • Author: Rudolph P Valentini, MD; Chief Editor: Michael R Bye, MD  more...
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Presentation

History and Physical Examination

Hemoptysis is the most common presenting symptom in anti–glomerular basement membrane (anti-GBM) disease (Goodpasture disease), followed by dyspnea, fatigue or weakness or both, and cough. In approximately two thirds of cases, hemoptysis precedes the onset of renal disease by 8-12 months. This interval can be as long as 12 years before nephritis develops.

Physical examination findings in patients with anti-GBM disease are those related to pulmonary hemorrhage, renal failure, and anemia and include pallor (the most common clinical sign), crackles and rhonchi, heart murmur, hepatomegaly, and edema.

Prompt diagnosis of pulmonary hemorrhage is imperative, because it is the principal cause of early death in patients with anti-GBM disease.

Go to Goodpasture Syndrome for complete information on this topic.

Renal assessment

Renal disease may be present; it can then be isolated or accompanied by pulmonary hemorrhage. When significant renal disease is present, it usually progresses rapidly. Signs and symptoms of disease can vary from hematuria and proteinuria with normal renal function to severe oligoanuric renal failure, such as the following:

  • Gross hematuria (10-41% of adults)

  • Edema (in as many as 25% of patients)

Hypertension can be present but is an uncommon manifestation of glomerulonephritis, reported in 4-17% of adult patients. Similarly, hypertension is unusual in children but has been reported.

Pulmonary assessment

Pulmonary hemorrhage can range from mild to life threatening, including the following:

  • Hemoptysis (82-90% of adults): This feature can vary from blood-tinged sputum to profound bleeding; mild hemoptysis may resolve spontaneously or progress to massive hemorrhage in a short period, resulting in fulminant respiratory failure

  • Cough (40-60% of adults)

  • Exertional dyspnea (57-72% of adults): This likely reflects both lung parenchymal involvement and anemia from pulmonary hemorrhage; in severe cases, clinical signs of pulmonary hemorrhage include tachypnea, inspiratory crackles and rhonchi, and cyanosis

  • Fatigue and weakness (38-66% of adults)

  • Fevers, chills, diaphoresis (15-24% of adults)

  • Tachypnea

  • Cyanosis

  • Inspiratory crackles

  • Bronchial breathing

Generalized vasculitis assessment

The general features listed below are more prominent in patients with systemic vasculitis than in others. The following can be the initial signs or symptoms in patients with anti-GBM disease:

  • Malaise

  • Weight loss

  • Arthralgias

  • Fever

  • Pallor: This is correlated with the degree of anemia present; approximately 51-90% of adults have pallor

Approximately 20-25% of patients have a functional heart murmur because of anemia.

Unlike patients with antineutrophil cytoplasmic autoantibodies (ANCA) disease, patients with anti-GBM disease typically lack symptoms of generalized vasculitis.