Anti-GBM Antibody Disease Clinical Presentation

Updated: Jun 06, 2022
  • Author: Agnieszka Swiatecka-Urban, MD, FASN, FAAP; Chief Editor: Craig B Langman, MD  more...
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Presentation

History

Anti–glomerular basement membrane (anti-GBM) antibody disease can occur year-round, but the incidence increases in the spring and in early summer. Most patients present with features of systemic illness and either acute nephritis or pulmonary involvement; a subset may have all 3 findings. Pulmonary involvement can precede the onset of glomerulonephritis by several years, or it can develop after renal disease is evident.

  • Symptoms of systemic illness include low-grade fever, malaise, headache, anorexia, nausea, vomiting, weight loss, and fatigue.

  • Symptoms of renal involvement include hematuria, oliguria, and edema.

  • Symptoms of pulmonary involvement include shortness of breath, cough, and expectoration of material that ranges from blood-streaked sputum to massive hemoptysis. A feeling of warmth inside the chest may precede hemoptysis.

  • Approximately 30% of patients with Goodpasture syndrome are antineutrophilic cytoplasmic antibody (ANCA) positive some time during the illness. Such patients may present with pruritic skin rashes and arthralgia.

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Physical Examination

Physical findings depend on the organ system involved and on the severity of the disease. No abnormalities may be evident in the absence of renal or pulmonary involvement.

  • Signs of renal involvement include the following:

    • When renal involvement is severe, volume overload of the intracellular and extracellular fluid may result in tachycardia, tachypnea, hypertension, pulmonary rales, and pitting edema.

    • Patients with uremia may have a specific breath odor, bruises, pallor, tremor, myoclonus, asterixis, focal neurologic signs, mental status changes, and seizures.

  • Signs of pulmonary involvement include the following:

    • Respiratory distress ranging from mild distress to respiratory failure

    • Pulmonary hemorrhage that may result in pallor, tachycardia, and shock

  • Pulmonary manifestations can precede or follow signs of nephritis.

  • Patients with high ANCA and low anti-GBM antibody titers may present with various vasculitic skin rashes.

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