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Goodpasture syndrome – Wikipedia

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” redirects here. For other uses, see Goodpasture ( disambiguation )Rare autoimmune disease
Medical condition

Goodpasture syndrome
Other names Goodpasture’s syndrome, Goodpasture disease, Goodpasture’s disease, anti–glomerular basement membrane disease, anti–glomerular basement membrane antibody disease, anti-GBM disease, anti-GBM antibody disease
Crescentic glomerulonephritis - high mag.jpg
Micrograph of a crescentic glomerulonephritis that was shown to be anti–glomerular basement membrane disease, PAS stain
Specialty Nephrology, pulmonology, immunology Edit this on Wikidata

Goodpasture syndrome (GPS), also known as anti–glomerular basement membrane disease, is a rare autoimmune disease in which antibodies attack the basement membrane in lungs and kidneys, leading to bleeding from the lungs, glomerulonephritis,[1] and kidney failure.[2] It is thought to attack the alpha-3 subunit of type IV collagen, which has therefore been referred to as Goodpasture’s antigen.[3] Goodpasture syndrome may quickly result in permanent lung and kidney damage, often leading to death. It is treated with medications that suppress the immune system such as corticosteroids and cyclophosphamide, and with plasmapheresis, in which the antibodies are removed from the blood.

The disease was first described by an American pathologist Ernest Goodpasture of Vanderbilt University in 1919 và was later named in his honor. [ bốn ] [ 5 ]

Signs và symptoms[edit]

220px Antibody Diagram of a monomeric ( one-unit ) antibodyThe anti – glomerular basement membrane ( GBM ) antibodies primarily attack the kidneys và lungs, although, generalized symptoms lượt thích malaise, weight loss, fatigue, fever, & chills are also common, as are joint aches & pains. [ 6 ] 60 mập 80 % of those with the condition experience both lung và kidney involvement ; 20-40 % have kidney involvement alone, & less than 10 % have lung involvement alone. [ 6 ] Kidney symptoms usually include blood in the urine, protein in the urine, unexplained swelling of limbs or face, high amounts of urea in the blood, và high blood pressure. [ 6 ] Lung symptoms usually antedate kidney symptoms & usually include : coughing up blood, chest pain ( in less than 50 % of cases overall ), cough, và shortness of breath. [ 7 ] Some other signs & symptoms that could be used bự identify Goodpasture syndrome during a physical exam include an increased respiratory rate, cyanosis, crackles, hepatosplenomegaly, & hypertension. [ tám ]


While the exact cause is unknown, the genetic predisposition to GPS involves the human leukocyte antigen (HLA) system, specifically HLA-DR15.[9] In addition to genetic susceptibility, an initial environmental insult to the pulmonary vasculature is needed to allow the anti-glomerular basement membrane (anti-GBM) antibodies to reach the alveolar capillaries. Examples of such an insult include: exposure to organic solvents (e.g. chloroform) or hydrocarbons, exposure to tobacco smoke, infection (such as influenza A), cocaine inhalation, metal dust inhalation, bacteremia, sepsis, high-oxygen environments, and antilymphocyte therapies (especially with monoclonal antibodies).[10] Exposure to dry cleaning chemicals and paraquat herbicide have also been implicated as potential insults. [11] In GPS, anti-GBM antibodies are produced and circulated throughout the bloodstream, damaging the membranes lining the lungs and kidneys as well as targeting their capillaries.[12]


GPS is caused by abnormal plasma cell production of anti-GBM antibodies. [ 10 ] The major target of these abnormal antibodies is the non-collagen tên miền of the alpha-3 chain of type bốn collagen, which is mostly found in the basal membranes of glomerular & alveolar capillaries, explaining the obscurely specific symptoms of this condition. [ 13 ] This preferred targeting of these alpha-3 collagen chains specifically in the basal membranes of glomerular và alveolar capillaries can be explained by the higher accessible exposure of epitopes, a larger expansion of the alpha-3 collagen units, và because these alpha-3 collagen chains structurally provide higher accessibility for the targeting antibodies. [ 14 ] Thes e antibodies bind their reactive epitopes phệ the basement membranes & activate the complement cascade, leading lớn the death of tagged cells. [ 10 ] A specific antibody và epitope binding that shows the highest affinity và is pathogenic occurs between GPA antibodies và the anti-GBM epitope region, designated EA, which is residues 17-31 of the alpha ba subunit of non-collagenous tên miền of type IV collagen. [ 15 ] T cells are also implicated, though it is generally considered a type II hypersensitivity reaction. [ 10 ]


The diagnosis of GPS is often difficult, as numerous other diseases can cause the various manifestations of the condition và the condition itself is rare. [ 16 ] The most accurate means of achieving the diagnosis is testing the affected tissues by means of a biopsy, especially the kidney, as it is the best-studied organ for obtaining a sample for the presence of anti-GBM antibodies. [ 16 ] On top of the anti-GBM antibodies implicated in the disease, about one in three of those affected also has cytoplasmic antineutrophilic antibodies in their bloodstream, which often predates the anti-GBM antibodies by about a few months or even years. [ 16 ] The later the disease is diagnosed, the worse the outcome is for the affected person. [ 10 ]In addition, if there is substantial suspicion of the disease, seralogic testing for ELISA assay is usually done by looking for alpha3 NC1 tên miền area of collagen IV in order béo avoid false positives. [ 17 ]


The major mainstay of treatment for GPS is plasmapheresis, a procedure in which the affected person’s blood is sent through a centrifuge và the various components separated based on weight. [ 18 ] The plasma contains the anti-GBM antibodies that attack the affected person’s lungs & kidneys, & is filtered out. [ 18 ] The other parts of the blood ( the red blood cells, trắng blood cells, & platelets ) are recycled & intravenously reinfused. [ 18 ] Most individuals affected by the disease also need bự be treated with immunosuppressant drugs, especially cyclophosphamide, prednisone, & rituximab, phệ prevent the formation of mới ra anti-GBM antibodies so sánh as Khủng prevent further damage lớn the kidneys & lungs. [ 18 ] Other, less toxic immunosuppressants such as azathioprine may be used mập maintain remission. [ 18 ]


With treatment, the five-year survival rate is > 80 % & fewer than 30 % of affected individuals require long-term dialysis. [ 10 ] A study performed in nước Australia và New Zealand demonstrated that in patients requiring renal replacement therapy ( including dialysis ) the median survival giây phút is 5.93 years. [ 10 ] Without treatment, virtually every affected person will die from either advanced kidney failure or lung hemorrhages. [ 10 ]


GPS is rare, affecting about 0.5 – 1.8 per million people per year in Europe & Asia. [ 10 ] It is also unusual among autoimmune diseases in that it is more common in males than in females và is also less common in blacks than whites, but more common in the Māori people of New Zealand. [ 10 ] The peak age ranges for the onset of the disease are trăng tròn – 30 and 60 – 70 years. [ 10 ]

See also[edit]


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