Anti gbm antibody disease is one of the three major forms of rapidly progressive or crescentic glomerulonephritis. Anti glomerular basement membrane disease orphanet. 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. Antiglomerular basement membrane antigbm disease is an. 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. When these anti gbm antibodies bind to the basement membrane, they activate the classical pathway of. As in other autoimmune diseases, anti gbm disease has been strongly associated with the major histocompatibility complex mhc. Aug 10, 2018 goodpastures syndrome also is known as antigbm disease and antiglomerular basement membrane disease. 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.
Sheep antirat glomeruli gbm serum ptx001s for the induction of passive antigbm nephritis. However, several reports demonstrated recurring cases with antigbm disease 31,32,33,34. 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. Cureus a case of antiglomerular basement disease without. Goodpastures disease, antigbm antibodies, crescentic glomerulonephritis, pulmonary.
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. Treatment of anti gbm disease is focused on removing the anti gbm antibody from the blood. Anti gbm antibodymediated glomerulonephritis usually presents with rapidly progressive glomerulonephritis. Pdf on nov 28, 2018, satyapal rangaraj and others published antigbm. 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. 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. However, in some cases either of them may also get affected i. Dec 19, 2019 antiglomerular basement membrane anti gbm antibody disease is a rare autoimmune disorder in which circulating antibodies are directed against an antigen normally present in the gbm and alveolar basement membrane.
Despite starting plasmapheresis and corticosteroids treatment, her renal functions continued to deteriorate and she was started on regular hemodialysis. The study protocol was approved by the local independent ethics committee amiens, france. Goodpasture syndrome is sometimes called anti gbm disease. Read general information about kidney transplant here. Chinese patients with anti gbm disease have two peaks in age. Antiglomerular basement membrane antigbm antibody disease is a rare autoimmune disorder characterized by rapidly progressive glomerulonephri.
Request pdf diagnosis and classification of goodpasture disease antigbm goodpastures disease or antiglomerular basement membrane disease. Antiglomerular basement membrane gbm disease often presents with rapidly progres sive glomerulonephritis with or without lung hemorrhage. Anti glomerular basement membrane anti gbm disease classically presents with aggressive necrotizing and crescentic glomerulonephritis, often with pulmonary hemorrhage. There are several reports of antigbm disease associated with membranous nephropathy, occurring as a preceding, simultaneous, or succeeding diagnosis 89,90. Ancas and anti gbm antibody is extremely rare and has high rates of fatal outcome. 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. 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. Jun 01, 2012 anti gbm disease based gn kdigo guidelines from kidney international 1. Hansa biopharma completes enrollment in antigbm biospace.
Gbm breaks but without crescents or detectable serum anti gbm antibody in a patient followed over 9 years. Antigbm gn is caused by autoantibodies classically type g immunoglobulin directed against the. 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. Antiglomerular basement membrane disease american society. In the literature, the first use of rituximab for anti gbm disease was reported in 2002 by arzoo et al. Antigbm antibody disease is one of the three major forms of rapidly progressive or crescentic glomerulonephritis. Slight precipitate may form when thawed, centrifuge x 3,000 5,000 rpm, 30 minutes before injection. Antiglomerular basement membrane antigbm disease is a rare small vessel vasculitis that affects the capillary beds of the kidneys and lungs.
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. The definitions of these terms have not been consistent, however. Antiglomerular basement membrane disease kidney international. Antiglomerular basement membrane disease in the elderly. In some patients, the antibody crossreacts with alveolar basement membranes, and about half also have hemoptysis due to lung involvement. The conclusion was that the biopsy revealed crescentic glomerulonephritis due to antigbm disease. Introduction antiglomerular basement membrane antigbm disease is a classic autoimmune disease with autoantibodies directed against the gbm, rapidly progressive glomerulonephritis, and alveolar hemorrhage. Once the autoantibodies attach to the basement membrane, this creates a signal to the bodys immune system to attack. Anti glomerular basement membrane anti gbm disease is a classic autoantibody mediated small vessel vasculitis. Clinical and histopathological features of anti gbm disease in chinese.
Fortunately, kidney transplant is an option for these patients. Anti glomerular basement membrane anti gbm disease is a rare small vessel vasculitis that affects the capillary beds of the kidneys and lungs. Diagnosis is confirmed with the presence of antigbm antibody in the blood or in the kidney. The antigbm antibodies seem to disappear spontaneously after 1218 months. Diagnosis and classification of goodpasture disease antigbm. Goodpasture disease is a term used to describe glomerulonephritis, with or without pulmonary hemorrhage, and the presence of circulating antiglomerular basement membrane antigbm antibodies. Anti gbm disease is an immune complex, small vessel vasculitis. Treatment was started with oral prednisolone, 60 mg daily. Antigbm disease associated with membranous nephropathy. 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. 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. Pdf atypical antiglomerular basement membrane disease.
Atypically, anti gbm nephritis follows an indolent course. 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. Relapses of antigbm disease are rarely observed, in contrast to most other autoimmune kidney diseases. Goodpasture syndrome is fatal unless quickly diagnosed and treated. 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. 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. Clinical data patients were entered into the study when the diagnosis of antigbm disease was established. 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. Treatment of antigbm antibody goodpastures disease uptodate. Although a clinical trial in anti gbm disease may be aspirational, mcadoo et al.
It may occasionally be useful to confirm the specificity of the antibodies by western blotting. The incidence in the united states is about one per million per year. When anti gbm disease involves the kidneys, it can often lead to kidney failure. No treatment recommendations have been established for such cases. 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. Several serologic studies over the last 20 years have docu. Anti gbm disease has been described in siblings and two sets of identical twins, although discordant twins also have been reported. Pdf background antiglomerular basement membrane antigbm disease classically presents with aggressive necrotizing and crescentic. Antiglomerular basement membrane antibody an overview. Diagnosis and classification of goodpastures disease antigbm. Pdf a case report of double negative antigbm disease.
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. The causes of goodpasture syndrome are not fully 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. Antiglomerular basement membrane antigbm disease is a rare small vessel. This is an uncommon disorder that involves quick worsening of lung disease and kidney failure. The circulating antibodies involved in anti gbm disease are almost exclusively igg and they are mainly directed against the alpha three noncollagenous domain nc1 of typefour collagen. Conclusion in conclusion, prs is a lifethreatening condition that warrants pulmonaryrenal syndrome with negative ancas and anti gbm. 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. Antiglomerular basement membrane disease american society of. 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 syndrome or antigbm diseasecausessymptoms. 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 principal discussant. We therefore believe that all cases of anti gbm disease, with or without anca, should be enrolled in a structured prospective registry. Pulmonaryrenal syndrome with negative ancas and antigbm. The first peak is in the second and third decades of life with a striking male preponderance and a higher frequency of pulmonary haemorrhage. Severe anti gbm antibody disease may progress to renal failure or death. Severe infection in antiglomerular basement membrane disease. 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.
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