Goodpasture disease is a term used to describe glomerulonephritis, with or without pulmonary hemorrhage, and the presence of circulating antiglomerular basement membrane antigbm antibodies. Circulating antigbm antibodies circulating antigbm antibodies can be detected by elisa or ria in more than 98% of patients with goodpastures disease, and this test is highly specific with less than 1% falsepositive results. The incidence in the united states is about one per million per year. Pdf on nov 28, 2018, satyapal rangaraj and others published antigbm. Pusey hammersmith hospital and imperial college london, london, united kingdom case presentation a 47yearoldwhite manwho workedas aprofessional musician presented to his general practitioner with a 3week history of malaise, loss of appetite, slight weight loss, and dark urine. However, in some cases either of them may also get affected i. Antiglomerular basement membrane antigbm disease is an. Antiglomerular basement membrane antibody disease goodpasture syndrome antiglomerular basement membrane antibody antigbm disease, or goodpasture syndrome, is a rare autoimmune disease characterized by the presence of autoantibodies directed against the nc1domain of the alpha3 chain of basement membrane collagen type iv.
It may manifest as an isolated glomerulonephritis antigbm nephritis or as a pulmonaryrenal syndrome with. The renewed interest in antigbm disease is spurred by the detection of autoantibodies in other forms of glomerulonephritis and has led to an exciting development in studies on pathogenesis as well as diagnosis and management. Mar 28, 2017 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 antigbm antibodies. Read general information about kidney transplant here.
Diagnosis is confirmed with the presence of antigbm antibody in the blood or in the kidney. The clinical and immunologic features of patients with. Anti glomerular basement membrane anti gbm disease is a rare small vessel vasculitis that affects the capillary beds of the kidneys and lungs. Gbm breaks but without crescents or detectable serum anti gbm antibody in a patient followed over 9 years. Antiglomerular basement membrane antigbm disease encompasses tissue injury caused by autoantibodies to constituents of the gbm, most commonly the noncollagenous domain nc1 of the alpha3 subunit of type iv collagen 14. Goodpasture syndrome is sometimes called anti gbm disease. Cureus a case of antiglomerular basement disease without. 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 and kidney failure. It may occasionally be useful to confirm the specificity of the antibodies by western blotting.
However, anti gbm disease is only one cause of pulmonaryrenal syndromes, including goodpasture syndrome. History the history of what we today call anti gbm disease dates back to 1958 when stanton et al reported as series of patients with the combination of glomerulonephritis and lung. Clinical data patients were entered into the study when the diagnosis of antigbm disease was established. Antigbm disease associated with membranous nephropathy. However, several reports demonstrated recurring cases with antigbm disease 31,32,33,34. Anti gbm antibodymediated glomerulonephritis usually presents with rapidly progressive glomerulonephritis. The antigbm antibodies seem to disappear spontaneously after 1218 months. Jan 27, 2020 anti gbm antibody disease, also known as goodpastures disease, is a severe kidney disease where the immune system mistakenly develops iggantibodies, resulting in an acute immune attack on the kidneys and in some patients also on the lungs. Treatment was started with oral prednisolone, 60 mg daily. The causes of goodpasture syndrome are not fully understood. Fortunately, kidney transplant is an option for these patients. Diagnosis and classification of goodpastures disease antigbm. A very small fraction of patients with anti gbm nephritis lack detectable circulating anti gbm antibodies, and rare reports of monoclonal anti gbm nephritis exist. Once the autoantibodies attach to the basement membrane, this creates a signal to the bodys immune system to attack.
Pdf background antiglomerular basement membrane antigbm disease classically presents with aggressive necrotizing and crescentic. Ancas and anti gbm antibody is extremely rare and has high rates of fatal outcome. The target antigen is the alpha3 chain of type iv collagen. Hansa biopharma completes enrollment in antigbm biospace. Anti glomerular basement membrane disease orphanet. Antigbm antibody disease is one of the three major forms of rapidly progressive or crescentic glomerulonephritis. Atypically, anti gbm nephritis follows an indolent course. As in other autoimmune diseases, anti gbm disease has been strongly associated with the major histocompatibility complex mhc. Goodpasture syndrome is fatal unless quickly diagnosed and treated. The first peak is in the second and third decades of life with a striking male preponderance and a higher frequency of pulmonary haemorrhage. Jun 01, 2012 anti gbm disease based gn kdigo guidelines from kidney international 1. Jan 18, 20 varied presentations of antigbm disease in antigbm disease the pulmonary hemorrhage may precede, occur concurrently with, or follow the glomerular involvement some patients with antigbm antibodies and gn and hence antigbm disease never experience pulmonary involvement and thus do not have true goodpastures syndrome. Anti gbm disease has been described in siblings and two sets of identical twins, although discordant twins also have been reported.
Although some patients present with relatively mild renal insufficiency, this disorder is typically associated with severe renal injury that, if untreated, progresses quickly to endstage renal failure. Goodpastures disease, antigbm antibodies, crescentic glomerulonephritis, pulmonary. Relapses of antigbm disease are rarely observed, in contrast to most other autoimmune kidney diseases. Clinical and histopathological features of anti gbm disease in chinese. Treatment of anti gbm disease is focused on removing the anti gbm antibody from the blood. Anti glomerular basement membrane anti gbm disease classically presents with aggressive necrotizing and crescentic glomerulonephritis, often with pulmonary hemorrhage. This constellation of kidney and lung disease is called goodpasture syndrome. A model for autoimmune kidney diseases the renewed interest in anti gbm disease is spurred by the detection of autoantibodies in other forms of glomerulonephritis and has led to an exciting development in studies on pathogenesis as well as diagnosis and management. Anti glomerular basement membrane antibody disease anti gbm disease is an uncommon yet wellknown condition because of the striking way in which it can cause rapid renal failure and lung bleeding.
Antibody levels were 7184% lower after the first ias treatment and were within normal range after two to nine treatments. The pathologic hallmark is linear staining of gbms for deposited immunoglobulin g igg, usually accompanied by serum autoantibodies to the collagen iv alpha3 constituents of. Several serologic studies over the last 20 years have docu. Anti glomerular basement membrane anti gbm disease is a classic autoantibody mediated small vessel vasculitis. The study protocol was approved by the local independent ethics committee amiens, france. Antiglomerular basement membrane disease kidney international. Not graded background anti gbm gn is generally a fulminant and rapidly progressive disease that is caused by autoantibodies to the noncollagenous domain of the a3 chain of type iv collagen. Antiglomerular basement membrane antigbm antibody disease is a rare autoimmune disorder characterized by rapidly progressive glomerulonephri. Diagnostic and management challenges in goodpastures anti. Pulmonaryrenal syndrome with negative ancas and antigbm. Antiglomerular basement membrane disease principal discussant.
Pdf atypical antiglomerular basement membrane disease. Chinese patients with anti gbm disease have two peaks in age. Anti gbm antibody disease is one of the three major forms of rapidly progressive or crescentic glomerulonephritis. Antiglomerular basement membrane antibody an overview.
We therefore believe that all cases of anti gbm disease, with or without anca, should be enrolled in a structured prospective registry. Pdf a case report of double negative antigbm disease. Sheep antirat glomeruli gbm serum ptx001s for the induction of passive antigbm nephritis. Treatment of antigbm antibody goodpastures disease uptodate. Antigbm gn is caused by autoantibodies classically type g immunoglobulin directed against the. Slight precipitate may form when thawed, centrifuge x 3,000 5,000 rpm, 30 minutes before injection. Antiglomerular basement membrane disease in the elderly. When these anti gbm antibodies bind to the basement membrane, they activate the classical pathway of. As with the anca association, it is postulated that disruption of glomerular architecture by one disease reveals hidden epitopes that allow the second process to occur. Antiglomerular basement membrane disease american society of. There are several reports of antigbm disease associated with membranous nephropathy, occurring as a preceding, simultaneous, or succeeding diagnosis 89,90. Antigbm disease is thought to result from an environmental insult smoking, infections, exposure to certain drugs in a person with genetic susceptibility, such as a specific human leukocyte antigen hla type. Request pdf diagnosis and classification of goodpasture disease antigbm goodpastures disease or antiglomerular basement membrane disease. Antiglomerular basement membrane antigbm disease is a rare small vessel vasculitis that affects the capillary beds of the kidneys and lungs.
Currently, the preferred term for both conditions is antigbm antibody disease. Diagnosis and classification of goodpasture disease antigbm. Antiglomerular basement membrane glomerulonephritis antigbm gn is a rare autoimmune disease that is characterized by rapidly progressive glomerulonephritis that may be associated with pulmonary hemorrhage. Jan 25, 2019 anca and anti gbm disease there is a consistently strong association between the presence of antineutrophil cytoplasmic antibodies anca and the occurrence of anti gbm disease. Antiglomerular basement membrane gbm disease often presents with rapidly progres sive glomerulonephritis with or without lung hemorrhage. Introduction antiglomerular basement membrane antigbm disease is a classic autoimmune disease with autoantibodies directed against the gbm, rapidly progressive glomerulonephritis, and alveolar hemorrhage. Antiglomerular basement membrane disease american society. The conclusion was that the biopsy revealed crescentic glomerulonephritis due to antigbm disease. No treatment recommendations have been established for such cases. This is an uncommon disorder that involves quick worsening of lung disease and kidney failure. Aug 10, 2018 goodpastures syndrome also is known as antigbm disease and antiglomerular basement membrane disease.
Antiglomerular basement membrane antigbm disease is a rare small vessel. In the literature, the first use of rituximab for anti gbm disease was reported in 2002 by arzoo et al. It is an archetypic autoimmune disease, caused by the development of directly pathogenic autoantibodies targeting a well characterized autoantigen expressed in the basement membranes of these organs, although the inciting events that induce the. This phenomenon has been recognized since the 1980s, but compared with singlepositive antigbm disease, the underlying molecular pathway of doublepositive antigbm disease remains poorly understood.
This case highlights the challenging presentation and diagnosis of anti gbm disease, which requires a high clinical suspicion necessarily for early diagnosis and treatment to improve survival. Conclusion in conclusion, prs is a lifethreatening condition that warrants pulmonaryrenal syndrome with negative ancas and anti gbm. When anti gbm disease involves the kidneys, it can often lead to kidney failure. In some patients, the antibody crossreacts with alveolar basement membranes, and about half also have hemoptysis due to lung involvement. In antigbm disease, the body creates autoantibodies that recognize and attach to the basement membrane, which is part of the wall of capillary blood vessels in the kidneys and lungs.
1638 1376 862 956 835 1584 1152 1482 525 1449 1621 1613 507 1102 393 26 490 116 1142 588 21 314 696 1476 1183 579 895 1034 1436 1230 1220 1086 1144