Goodpasture syndrome

Contents • • • • • • • • What is Anti-Glomerular Basement Membrane Disease (also known as Goodpasture’s Syndrome)? Anti-Glomerular Basement Membrane disease (anti-GBM disease) is a disease that occurs as a result of injury to small blood vessels (capillaries) in the kidneys and/or lungs. Autoantibodies are antibodies directed toward the body itself (rather than towards something foreign such as bacteria or viruses). In anti-GBM disease, these autoantibodies are targeted to the basement membrane, which is part of the wall of these capillary blood vessels in the kidneys and lungs. The name anti-GBM disease reflects the fact that this disease is caused by autoantibodies targeting and causing damage to (anti-) the glomerular basement membrane (GBM). Anti-GBM disease can affect just the kidneys or both the lungs and kidneys (it is uncommon for the only the lungs to be affected). The reason these organs are affected is because the basement membrane that is targeted or attacked in this disease is only accessible to antibodies in the kidneys and lungs (not in other organs). About half of people with anti-GBM disease have lung involvement. Anti-GBM disease that only affects the kidneys is called anti-GBM glomerulonephritis. This is because there is inflammation (-itis), occurring in the glomeruli (filters) of the kidney. Anti-GBM disease affecting both the kidneys and lungs is often called Goodpasture’s syndrome or Goodpasture’s disease (though anti-GBM disease is the preferred ter...

What is Goodpasture syndrome? Goodpasture syndrome is a rare autoimmune disorder that affects both the lungs and kidneys. The immune system typically makes antibodies to fight off infection. But with Goodpasture syndrome, the immune system mistakenly makes antibodies that attack the lungs and kidneys. This condition can quickly progress to an inflammation of the kidneys (glomerulonephritis) and kidney failure. It can be fatal if not quickly diagnosed and treated. This disease most often occurs in people ages 20 to 30 or those older than age 60. It is more common in men. In some cases, bleeding in the lungs may occur. In most cases, this disease doesn't cause lasting damage to the lungs. But kidney damage may be long-lasting. If the kidneys fail, kidney transplant or dialysis may be needed. What causes Goodpasture syndrome? Medical experts are not certain what causes this autoimmune disorder. It can run in families, so genetics may play a role. Or the disease may occur because of a combination of other factors. These include exposure to certain chemicals, such as dry cleaning chemicals or the weed killer Paraquat. Viral infections, smoking, or medicines may also play a role. What are the symptoms of Goodpasture syndrome? These are the most common symptoms of Goodpasture syndrome: • Lack of energy or tiring easily (fatigue) • Nausea • Trouble breathing • Pale skin As the condition progresses, other symptoms may occur, including: • Coughing up blood • Burning feeling when uri...

• Overview   • • Theory   • Epidemiology • Aetiology • Case history • Diagnosis   • Approach • History and exam • Investigations • Differentials • Management   • Approach • Treatment algorithm • Emerging • Prevention • Patient discussions • Follow up   • Monitoring • Complications • Prognosis • Resources   • Guidelines • Images and videos • Goodpasture's disease is defined by the presence of autoantibodies to the alpha-3 chain of type IV collagen. It presents with rapidly progressive renal dysfunction often combined with lung haemorrhage. Simultaneous haemoptysis and renal failure (the pulmonary renal syndrome) has many causes, but where the renal disease is a rapidly progressive glomerulonephritis, about 15% of cases will be Goodpasture's disease, with the remainder mostly antineutrophil cytoplasmic antibody vasculitis. Rapid diagnosis by early renal biopsy and serological testing is essential, because appropriate treatment given before renal damage is advanced can result in an excellent prognosis. Treatment is by removal of circulating antibodies using plasma exchange combined with agents to prevent their continued production. Definition Goodpasture’s disease, also known as anti-glomerular basement membrane antibody (anti-GBM) disease, is an important cause of pulmonary-renal syndrome (Goodpasture’s syndrome). Pusey CD. Anti-glomerular basement membrane disease. Kidney Int. 2003 Jul 7;64(7):1535-50. http://www.ncbi.nlm.nih.gov/pubmed/12969182?tool=bestpractic...

Continuing Education Activity Goodpasture syndrome also referred to as anti-glomerular basement membrane (anti-GBM) disease, is an autoimmune disease that affects both the kidneys and lungs by the formation of autoantibodies that attack their basement membranes. This activity outlines the evaluation and management of Goodpasture syndrome and explains the interprofessional team's role in improving care for patients with this condition. Objectives: • Identify the role of autoantibodies against glomerular basement membranes in the pathophysiology of Goodpasture syndrome. • Explain the use of kidney biopsy in the evaluation of Goodpasture syndrome. • Review the management of Goodpasture syndrome. • Summarize the importance of improving care coordination among the interprofessional team to enhance the delivery of care for patients affected by Goodpasture syndrome. Introduction Goodpasture syndrome refers to an anti-glomerular basement membrane (anti-GBM) disease that involves both the lungs and kidneys, often presenting as pulmonary hemorrhage and glomerulonephritis. However, some clinicians may interchangeably use several terms, includinganti-glomerular basement membranedisease, Goodpasture syndrome, and Goodpasture disease. Goodpasture syndrome is an eponym named after Ernest Goodpasture, who described this disorder for the first time in 1919. Later the discovery of anti-GBM antibodies led to a better understanding of the pathogenesis of Goodpasture syndrome. Etiology Goodpas...

Goodpasture syndrome is a rare but serious The disease occurs when the body's immune system mistakenly produces antibodies against Goodpasture syndrome initially causes vague symptoms such as Causes of Goodpasture Syndrome Researchers do not fully understand why the immune system attacks collagen in the Other factors that may increase the risk of Goodpasture syndrome include: • Exposure to certain chemicals, such as hydrocarbon solvents and the weed killer paraquat • Exposure to metallic dust • Use of certain drugs, such as • • Goodpasture syndrome usually affects young men. It more often occurs among whites, and it most commonly affects people who are: • Between ages 20 and 30 • Older than age 60 Symptoms of Goodpasture Syndrome The first signs of Goodpasture syndrome may include: • • • Difficulty breathing • Pale Because Goodpasture syndrome may rapidly involve the lungs, initial symptoms such as shortness of breath can quickly progress to a persistent When Goodpasture syndrome affects the kidneys, symptoms may include: • • • Foamy urine • Swelling in the legs • • Burning or difficulty when urinating • • Swelling of the hands and feet Although Goodpasture syndrome may cause life-threatening bleeding in the lungs, it usually does not result in long-term lung damage. The most serious consequence of Goodpasture syndrome is Diagnosis of Goodpasture Syndrome See your doctor at once if you develop the characteristic symptoms of Goodpasture syndrome. They may order the followin...