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Goodpasture’s Syndrome: Autoimmunity Gone Wrong

Goodpasture’s Syndrome, more accurately referred to today as anti-GBM disease, is a rare autoimmune small vessel vasculitis. In this condition, the immune system produces circulating antibodies that mistakenly attack a specific antigen found in the glomerular basement membrane (GBM) of the kidneys and the alveolar basement membrane (ABM) of the lungs.1 In the kidneys, this immune attack causes rapidly progressive glomerulonephritis, leading to the leakage of blood (hematuria) and protein (proteinuria) in the urine, and may eventually result in renal failure. In the lungs, the same immune mechanism triggers alveolar hemorrhage, which can present as coughing up blood and shortness of breath

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 Goodpasture’s Syndrome: the Lung and Kidney Disease

The human body is deeply interconnected; when things go awry in one organ or system, it often means that another will be affected, too. One such example of this is Goodpasture’s Syndrome, a rare autoimmune disease that affects both the lungs and kidneys. Autoimmune diseases are characterized by being conditions in which our immune systems, which are supposed to function as a way our body fights infection, mistakenly attacks parts of our own bodies. In the case of Goodpasture’s Syndrome, the body produces antibodies (proteins made to fight infection) that attack and subsequently hurt the linings of the lungs and kidneys. Experts have not yet pinned down why it is the bodies of those with Goodpasture’s begin to make such antibodies, though they speculate that smoking, inhaling vapors from organic solvents, as well as exposure to viral lung infections could trigger the condition.

Articles of Interest

A unique way to treat Goodpasture's disease

Goodpasture's disease (GD) is rare, but knowledge about the disease and early diagnosis is key because the antiglomerular basement membrane (GBM) antibody can cause end stage renal failure if untreated. The gold standard for treating GD is plasmapheresis, corticosteroids and cyclophosphamide. Unfortunately, cyclophosphamide can cause several toxicities, so trialing less toxic agents is essential. Our case demonstrates the use of Rituximab along with plasmapheresis and steroids to treat GD with good outcomes for the patient.

Anti-GBM (Goodpasture’s) Disease

Anti-GBM disease most often affects men in their 20s and women in their 60s, but it can occur at any age. The condition can occur in children, but this is extremely rare.

Autoimmunity in Anti–Glomerular Basement Membrane Disease: A Review of Mechanisms and Prospects for Immunotherapy

Anti–glomerular basement membrane (anti-GBM) disease is an organ-specific autoimmune disorder characterized by autoantibodies against the glomerular and alveolar basement membranes, leading to rapidly progressive glomerulonephritis and severe alveolar hemorrhage. The noncollagenous domain of the α3 chain of type IV collagen, α3(IV)NC1, contains the main target autoantigen in this disease.

Goodpasture and his syndrome

Ask any medical student which disease affects the lungs and kidneys, and he or she is likely to answer Goodpasture's syndrome.

Goodpasture's syndrome: a clinical update

Goodpasture's syndrome (GS) is a rare and organ-specific autoimmune disease that is mediated by anti-glomerular basement membrane (anti-GBM) antibodies and has pathology characterized by crescentic glomerulonephritis with linear immunofluorescent staining for IgG on the GBM. It typically presents as acute renal failure caused by a rapidly progressive glomerulonephritis, accompanied by pulmonary hemorrhage that may be life-threatening.

Goodpasture’s Disease: An Uncommon Disease With an Atypical Clinical Course

Goodpasture’s disease is an uncommon composite of features including renal failure with pulmonary hemorrhage secondary to an autoimmune response that specifically targets these organ systems.

Resources

Vasculitis Foundation

Anti-GBM (anti-glomerular basement membrane) disease is a rare, but serious autoimmune disease that causes inflammation of the small vessels in the kidneys and lungs. In this disease, the immune system mistakenly makes “anti-GBM antibodies,” which attack the lungs and kidneys, causing bleeding and damage to those organs. The disease can progress rapidly to life-threatening lung and kidney failure, so early diagnosis and treatment are vital. Smoking and exposure to certain chemicals may be among the triggers for this disease.

National Institute of Diabetes and Digestive and Kidney Diseases

Ernest Goodpasture first described the syndrome during the influenza pandemic of 1919 when he reported on a patient who died from bleeding in the lungs and kidney failure. Diagnostic tools to confirm Goodpasture syndrome were not available at that time, so it is not known whether the patient had true Goodpasture syndrome or vasculitis

National Kidney Foundation

Goodpasture's Syndrome may cause life-threatening bleeding in the lungs, but does not usually cause long-term damage in that area. The harm done to your kidneys, however, can result in kidney failure. You may need either dialysis or a kidney transplant.

Vasculitis Foundation

Anti-GBM/Goodpasture’s syndrome is a rare, but serious autoimmune disease that causes inflammation of the kidneys and lungs. In this syndrome, the immune system mistakenly makes “anti-GBM antibodies” which attack the lungs and kidneys, causing bleeding within the organs. The disease can progress rapidly to life-threatening organ failure, so early diagnosis and treatment are vital. Smoking and exposure to certain chemicals may be among the triggers for this disease.

FP Notebook

Symptoms and Signs. Pulmonary Hemoptysis. Cough. Exertional Dyspnea. Rales and rhonchi on Lung Exam. Renal-Glomerulonephritis

GARD

Goodpasture syndrome is an autoimmune disease that affects the lungs and kidneys and is characterized by pulmonary alveolar hemorrhage (bleeding in the lungs) and a kidney disease known as glomerulonephritis. Some use the term "Goodpasture syndrome" for the findings of glomerulonephritis and pulmonary hemorrhage and the term "Goodpasture disease" for those patients with glomerulonephritis, pulmonary hemorrhage, and anti-GBM antibodies.

NORD

Goodpasture syndrome is a rare autoimmune disorder characterized by inflammation of the filtering structures (glomeruli) of the kidneys (glomerulonephritis) and excessive bleeding into the lungs (pulmonary hemorrhaging).

Patient

Typically presents as acute kidney injury caused by a rapidly progressive glomerulonephritis, accompanied by pulmonary haemorrhage that may be life-threatening.

StatPearls

Goodpasture syndrome, also known as anti-glomerular basement membrane (anti-GBM) disease, is a rare autoimmune disorder characterized by the production of autoantibodies targeting antigens in the glomerular and alveolar basement membranes. These antibodies, primarily directed against the non-collagenous domain of the α-3 chain of type IV collagen, trigger inflammatory responses involving local complement activation and recruitment of polymorphonuclear leukocytes.

StatPearls

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 use several terms interchangeably, including anti-glomerular basement membrane disease, Goodpasture syndrome, and Goodpasture disease.