IgA-mediated anti-glomerular basement membrane disease: an uncommon mechanism of Goodpasture's syndrome

Moulis, Guillaume; Huart, Antoine; Guitard, Joëlle; Fortenfant, Françoise; Chauveau, Dominique

doi:10.1093/ckj/sfs087

Cited by 24 publications

(32 citation statements)

References 15 publications

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“…A proportion of patients who have demonstrable deposition of IgG on the GBM by immunofluorescence, but who are negative for circulating antibodies by these conventional techniques, may be positive when tested by highly sensitive biosensor assay (54). In anti-GBM disease, the pathogenic antibodies are usually of the IgG class, with IgG1 and IgG3 subclasses predominating (37,38), although rare cases of IgA-and IgG4-mediated disease have been described (55,56). These antibodies may not be detected on routine assays.…”

Section: Serologic Testingmentioning

confidence: 99%

Anti-Glomerular Basement Membrane Disease

McAdoo¹,

Pusey²

2017

CJASN

302

303

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Anti-glomerular basement membrane (anti-GBM) disease is a rare small vessel vasculitis that affects the capillary beds of the kidneys and lungs. 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 autoimmune response are not fully understood. The recent confirmation of spatial and temporal clustering of cases suggests that environmental factors, including infection, may trigger disease in genetically susceptible individuals. The majority of patients develop widespread glomerular crescent formation, presenting with features of rapidly progressive GN, and 40%-60% will have concurrent alveolar hemorrhage. Treatment aims to rapidly remove pathogenic autoantibody, typically with the use of plasma exchange, along with steroids and cytotoxic therapy to prevent ongoing autoantibody production and tissue inflammation. Retrospective cohort studies suggest that when this combination of treatment is started early, the majority of patients will have good renal outcome, although presentation with oligoanuria, a high proportion of glomerular crescents, or kidney failure requiring dialysis augur badly for renal prognosis. Relapse and recurrent disease after kidney transplantation are both uncommon, although de novo anti-GBM disease after transplantation for Alport syndrome is a recognized phenomenon. Copresentation with other kidney diseases such as ANCAassociated vasculitis and membranous nephropathy seems to occur at a higher frequency than would be expected by chance alone, and in addition atypical presentations of anti-GBM disease are increasingly reported. These observations highlight the need for future work to further delineate the immunopathogenic mechanisms of anti-GBM disease, and how to better refine and improve treatments, particularly for patients presenting with adverse prognostic factors.

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Section: Serologic Testingmentioning

confidence: 99%

Anti-Glomerular Basement Membrane Disease

McAdoo¹,

Pusey²

2017

CJASN

302

303

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“…Speculatively, this type of analysis may have been able to detect circulating antibodies in the cases reported by Dash et al [ 3 ] and Kussman and Gohara [ 4 ]. Alternatively, as in the case reported by Moulis et al [ 2 ] and the 11 similar cases reviewed in their discussion, ELISA testing for IgG anti-GBM autoantibodies was negative because the disease was mediated by IgA autoantibodies. Anti-GBM nephritis thus exemplifies the glomerular disease whose firm diagnosis relies on a combination of clinical, serologic and histopathologic findings.…”

mentioning

confidence: 89%

“…These unusual cases of RPGN were instances in which the diagnosis could only be made by a kidney biopsy. Moulis et al [ 2 ], Dash et al [ 3 ] and Kussman and Gohara [ 4 ] all describe classic clinical presentations of RPGN with negative serologic testing for circulating anti-GBM IgG autoantibodies (as well as negative testing for other causes of RPGN, such as ANCA and lupus) but firm diagnoses of anti-GBM nephritis made by kidney biopsies. Interestingly, the case reported by Moulis et al [ 2 ] showed on IF linear staining that was stronger for IgA than for IgG, prompting the authors to re-test their patients for anti-GBM IgA autoantibodies, which were positive.…”

mentioning

confidence: 99%

“…Moulis et al [ 2 ], Dash et al [ 3 ] and Kussman and Gohara [ 4 ] all describe classic clinical presentations of RPGN with negative serologic testing for circulating anti-GBM IgG autoantibodies (as well as negative testing for other causes of RPGN, such as ANCA and lupus) but firm diagnoses of anti-GBM nephritis made by kidney biopsies. Interestingly, the case reported by Moulis et al [ 2 ] showed on IF linear staining that was stronger for IgA than for IgG, prompting the authors to re-test their patients for anti-GBM IgA autoantibodies, which were positive. In this instance, the renal biopsy not only made the diagnosis but also informed serologic testing (as anti-GBM IgA autoantibodies are not routinely tested) that in turn guided treatment (plasma exchange until negative serologic testing for anti-GBM IgA).…”

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confidence: 99%

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Anti-glomerular basement membrane nephritis: why we still 'need' the kidney biopsy

Bomback

2012

Clinical Kidney Journal

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“…Goodpasture's syndrome (GPS) is an immuno disorder characterized by pulmonary hemorrhage, glomerulonephritis and the presence of the antiglomerular basement membrane (anti-GBM) antibody (1,2). The incidence of GPS is <1 case/1,000,000 (3).…”

Section: Introductionmentioning

confidence: 99%

Goodpasture's syndrome in early pregnancy: A case report

Qin¹,

Song²,

Liu³

2017

Exp Ther Med

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Abstract. Goodpasture's syndrome (GPS) presenting during pregnancy is extremely rare and patients exhibiting hemoptysis and renal dysfunction in early pregnancy are uncommon. The present study reports the case of a 17-year-old diagnosed with GPS during the thirteenth week of pregnancy. Prompt recognition and immediate treatment (steroids and plasma exchange) was initiated. Renal function normalized following treatment and anti-GBM antibody became negative during hospital stay. However, due to irregular follow-up, the patient eventually succumbed. The case highlights that the current treatment plan for GPS should be revised to improve the outcome of pregnancy. In addition, it determines how important it is for obstetricians to consider whether a pregnancy should be terminated to improve the chances of survival of pregnant patients with GPS. IntroductionGoodpasture's syndrome (GPS) is an immuno disorder characterized by pulmonary hemorrhage, glomerulonephritis and the presence of the antiglomerular basement membrane (anti-GBM) antibody (1,2). The incidence of GPS is <1 case/1,000,000 (3). Combination therapy is recommended for the initial management of the disease, including corticosteroid therapy, cytostatic therapy and plasmapheresis. However, patients have poor prognosis with current treatments and the mortality rate has been estimated as 11% (4). The etiology of Goodpasture's Syndrome remains unknown; however, the development of the disease following environmental exposures including hydrocarbon fumes, metallic dust or tobacco smoke has been reported (5). GPS occurs due to antibody and T-lymphocyte reactivity to the NC1 domain of the α3 chain of type IV collagen (6).Women presenting with GPS in the early period of pregnancy are uncommon and the present case study reports an unusual case of GPS in the early stage of pregnancy. In the present study, the patient received hemodialysis and methylprednisolone, prior to succumbing due to sustained, irreversible renal failure. The majority of pregnancies are terminated prior to term delivery to prevent worsening of conditions (7,8). Thus, current therapeutic strategies used to treat GPS should be revised in order to improve the survival rate of pregnant patients with GPS. Case reportA 17-year-old female was hospitalized at the Traditional Chinese Medicine Hospital of Guizhou (Guiyang, China) on 9th December 2013 due to no urination and hemoptysis for 3 days in the thirteenth week of pregnancy. The patient had history of double lower limb swelling lasting from the onset of pregnancy, which was treated with furosemide in Qianxi County Central hospital. The patient's medical history prior to pregnancy was normal; however, the patient had a 2-week history of cough prior to admission and experienced an episode of brown-colored urine 1 week later. The patient did not experience fever, dysuria or oliguria at that time. The patient visited Qianxi County Central hospital (Quianxi, China) on 21th October 2013 and was diagnosed with acute renal insufficiency. Serum was analysed ...

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IgA-mediated anti-glomerular basement membrane disease: an uncommon mechanism of Goodpasture's syndrome

Cited by 24 publications

References 15 publications

Anti-Glomerular Basement Membrane Disease

Anti-Glomerular Basement Membrane Disease

Anti-glomerular basement membrane nephritis: why we still 'need' the kidney biopsy

Goodpasture's syndrome in early pregnancy: A case report

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