Goodpasture syndrome is a rare and potentially life-threatening autoimmune disease. It causes buildup of autoimmune proteins in the kidneys and lungs that leads to damage of these organs. The disorder is named after Dr. Ernest Goodpasture, who first identified the syndrome in 1919.
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Why is it named Goodpasture syndrome?

Goodpasture syndrome (or anti-GBM disease) is a rare, life-threatening autoimmune disease that affects the lungs and the kidneys. It happens when the immune system mistakenly attacks a protein called collagen because it recognizes it as a foreign substance.

How was Goodpasture syndrome discovered?

In the 1950s, Krakower and Greenspon identified GBM as the antigen. In 1967, Lerner, Glassock, and Dixon confirmed that the antibodies taken from the diseased kidneys produced nephritis in experimental animals. The discovery of anti-GBM antibodies led to the understanding of the pathogenesis of Goodpasture syndrome.

Is anti-GBM the same as Goodpasture?

Does anti-GBM disease have another name? Anti-GBM disease is sometimes also called Goodpasture’s disease. Another related term is Goodpasture syndrome, a condition that also affects the kidneys and lungs but is not caused by anti-GBM antibodies.

How can you tell the difference between Wegener's and Goodpasture's?

The typical lesion in Goodpasture’s syndrome is hæmorrhage into the lungs giving rise eventually to pulmonary siderosis, whereas in Wegener’s syndrome there is replacement of the lining of bronchi and of accessory nasal sinuses by necrotizing granulomatous tissue which may simulate carcinoma or tuberculosis.

Can you survive Goodpasture syndrome?

In the past, Goodpasture syndrome was usually fatal. Aggressive therapy with plasmapheresis, corticosteroids, and immunosuppressive agents has dramatically improved prognosis. With this approach, the 5-year survival rate exceeds 80% and fewer than 30% of patients require long-term dialysis.

Is Goodpasture syndrome fatal?

Goodpasture syndrome is a rare disorder in which your body mistakenly makes antibodies that attack the lungs and kidneys. It most often occurs in people ages 20 to 30 or older than age 60. It is more common in men. It can be fatal if not quickly diagnosed and treated.

Can Covid cause Goodpasture syndrome?

In addition, reports from London showed that the pulmonary-renal syndrome that occurs during the COVID-19 pandemic was in part due to anti-glomerular basement membrane (anti-GBM) disease [5]. Anti-GBM disease — referred to as anti-GBM syndrome or Goodpasture’s disease — is a rare small vessel vasculitis.

What is GN Crescentic?

Proliferative extracapillary glomerulonephritis (GN) or crescentic GN is not a specific disease, but a histologic manifestation of severe glomerular damage. The term “extracapillary proliferation” is used to designate the cellular and/or fibrous proliferation that occupies the Bowman’s space, arising from its capsule.

What type of hypersensitivity is Goodpasture syndrome?

It is believed to be a type II hypersensitivity reaction to Goodpasture’s antigens on the basement membrane of the glomerulus of the kidneys and the pulmonary alveolus.

What is RPGN?

Rapidly progressive glomerulonephritis (RPGN) is a clinical syndrome manifested by features of glomerular disease in the urinalysis and by progressive loss of kidney function over a comparatively short period of time (days, weeks, or a few months).

Is Goodpasture a vasculitis?

Using strict criteria (pulmonary hemorrhage, glomerulonephritis, and antiglomerular basement membrane antibody), we found histological evidence of vasculitis in two of 18 patients with Goodpasture’s syndrome. The vasculitis was found in kidney biopsy specimens.

What are the symptoms of Goodpasture's disease?

Symptoms of Goodpasture syndrome include recurrent episodes of coughing up of blood (hemoptysis), difficulty breathing (dyspnea), fatigue, chest pain, and/or abnormally low levels of circulating red blood cells (anemia).

What is anti GBM disease?

Anti-glomerular basement membrane (anti-GBM) disease is a rare small vessel vasculitis that affects glomerular capillaries, pulmonary capillaries, or both. Most patients present with rapidly progressive (crescentic) glomerulonephritis, although some patients may present with relatively mild kidney impairment.

What is microscopic polyarteritis?

Microscopic polyangiitis (MPA) is a condition that causes small blood vessels to be inflamed. It’s a rare type of vasculitis. The disease can damage the blood vessels and cause problems in organs around the body. MPA most often affects people in their 50s and 60s, but it can happen at any age.

Which of the following distinguishes Goodpasture syndrome from Alport's Syndrome?

The GBM in Alport kidney is characterized by irregular thinning and thickening, splitting, and multi-laminations, which lead to progressive renal failure. In Goodpasture (GP) syndrome, the GBM is targeted by autoantibodies, leading to an inflammatory response and loss of filtration function.

What is Alport disease?

Alport syndrome is a disease that damages the tiny blood vessels in your kidneys. It can lead to kidney disease and kidney failure. It can also cause hearing loss and problems within the eyes. Alport syndrome causes damage to your kidneys by attacking the glomeruli.

What is it called when your immune system attacks your kidneys?

Lupus nephritis is a frequent complication in people who have systemic lupus erythematosus — more commonly known as lupus. Lupus is an autoimmune disease. It causes your immune system to produce proteins called autoantibodies that attack your own tissues and organs, including the kidneys.

What color is urine when your kidneys are failing?

Brown, red, or purple urine Kidneys make urine, so when the kidneys are failing, the urine may change. How? You may urinate less often, or in smaller amounts than usual, with dark-colored urine. Your urine may contain blood.

What are the signs of dying from kidney failure?

  • Water retention/swelling of legs and feet.
  • Loss of appetite, nausea, and vomiting.
  • Confusion.
  • Shortness of breath.
  • Insomnia and sleep issues.
  • Itchiness, cramps, and muscle twitches.
  • Passing very little or no urine.
  • Drowsiness and fatigue.
How many people have Goodpasture?

The disorder is named after Dr. Ernest Goodpasture, who first identified the syndrome in 1919. It’s estimated to occur in 1 out of 1 million people per year.

How does Sjogren's affect the kidneys?

Sjögren’s syndrome is typically associated with a lymphocytic and plasmacytic infiltrate in the salivary, parotid, and lacrimal glands, leading to a sicca syndrome. This immune process can also affect nonexocrine organs, including the kidneys, producing an interstitial nephritis and defects in tubular function.

Is ANCA positive in Goodpasture?

Perinuclear antineutrophilic cytoplasmic antibodies (p-ANCA), which can appear in Goodpasture syndrome, are also observed in Churg-Strauss vasculitis and occasionally in Wegener granulomatosis. In the majority of double-positive patients, the ANCAs have specificity for myeloperoxidase (MPO-ANCA).

What is the glomerular tuft?

The glomerular capillary tuft is a highly intricate and specialized microvascular bed that filters plasma water and solute to form urine. … Filtration begins only after the influx and organization of endothelial and mesangial cells in the developing glomerulus.

What is Pauci-immune Crescentic glomerulonephritis?

Pauci-immune crescentic glomerulonephritis (PICGN) is a rapidly progressive condition leading to renal failure within days or weeks and is potentially life threatening. Majority of these patients have clinical or pathological evidence of systemic vasculitis.

What are kimmelstiel Wilson nodules?

This is nodular glomerulosclerosis (the Kimmelstiel-Wilson lesion) of diabetes mellitus. Nodules of pink hyaline material form in regions of glomerular capillary loops in the glomerulus. This is due to a marked increase in mesangial matrix from damage as a result of non-enzymatic glycosylation of proteins.

Who is Goodpasture syndrome named after?

The spectrum of disease may present classically or as glomerulonephritis alone. 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.

What is Type 2 allergy?

Excerpt. Type II hypersensitivity reaction refers to an antibody-mediated immune reaction in which antibodies (IgG or IgM) are directed against cellular or extracellular matrix antigens with the resultant cellular destruction, functional loss, or damage to tissues.

What is Intrarenal failure?

Intrinsic or intrarenal acute kidney injury (AKI) , which used to be called acute renal failure, occurs when direct damage to the kidneys causes a sudden loss in kidney function. The treatment of intrinsic acute kidney injury includes identifying and correcting the cause of the kidney injury.

What is nephritic syndrome?

The nephritic syndrome is a clinical syndrome that presents as hematuria, elevated blood pressure, decreased urine output, and edema. The major underlying pathology is inflammation of the glomerulus that results in nephritic syndrome.

Is RPGN an Aki?

Various causes of rapidly progressive glomerulonephritis are described in the etiology section. Before considering the diagnosis of RPGN, other etiology of reversible acute kidney injury (AKI), proteinuria, and hematuria other than RPGN are excluded.

What is a Vasculitic screen?

These tests look for signs of inflammation, such as a high level of C-reactive protein. A complete blood cell count can tell whether you have enough red blood cells. Blood tests that look for certain antibodies — such as the anti-neutrophil cytoplasmic antibody (ANCA) test — can help diagnose vasculitis.

How is anti-GBM treated?

Standard treatment for anti-GBM disease includes plasmapheresis, to rapidly remove pathogenic autoantibody, along with cyclophosphamide and corticosteroids, to inhibit further autoantibody production and to ameliorate end-organ inflammation.

Is glomerulonephritis an emergency?

The emergency physician must consider acute glomerulonephritis in the differential diagnosis for patients that present with hypertension, hematuria, proteinuria, peripheral edema, and/or acute pulmonary edema.