Multiple recurrences of anti-glomerular basement membrane disease with variable antibody detection: can the laboratory be trusted?

Liu, Patricia; Waheed, Sana; Boujelbane, Lamya; Maursetter, Laura

doi:10.1093/ckj/sfw038

Cited by 15 publications

(10 citation statements)

References 22 publications

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“…It is usually associated with ongoing exposure to pulmonary irritants such as cigarette smoke and hydrocarbons (76,77), and avoidance of these precipitants is an essential part of long-term management of these cases. We recommend repeat kidney biopsy in cases of relapse with kidney involvement, in order to secure an accurate diagnosis and to exclude concomitant pathologies such as AAV and membranous nephropathy (discussed below).…”

Section: Outcome and Prognosismentioning

confidence: 99%

Anti-Glomerular Basement Membrane Disease

McAdoo¹,

Pusey²

2017

CJASN

304

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: Outcome and Prognosismentioning

confidence: 99%

Anti-Glomerular Basement Membrane Disease

McAdoo¹,

Pusey²

2017

CJASN

304

303

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“…Few plausible explanations for undetectable antibodies in case of seronegative autoimmune disorders are serum dilution, immunofluorescence substrate, prozone phenomenon, low immunoglobulin levels and hidden complexed antibodies deposited in the tissue [ 2 ]. Similarly, in the case of anti-GBM disease, technical limitations of the routinely available assay and low levels of antibodies from previous immunosuppressive therapy might be the reason for seronegativity [ 6 , 7 ]. Standard ELISA testing for anti-GBM is performed for the immunoglobulin G1 (Igg-1) subtype, but more recent cases have introduced an immunoglobulin G4 (Igg-4) subtype implicated in atypical presentation [ 8 ].…”

Section: Discussionmentioning

confidence: 99%

Recurrent alveolar hemorrhage: How do you treat that which you cannot see?

Philipose

Siddiqui

Jiyoung

et al. 2018

Respiratory Medicine Case Reports

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Diffuse alveolar hemorrhage (DAH) is a rare and life-threatening event which is characterized by bleeding into the alveolar spaces of the lung. Etiology of DAH can be broadly divided into immune and non-immune mediated disease. In the absence of infection or malignancy, an immunological workup is required to find the cause of alveolar bleed. Rarely, there is a failure to establish a definitive etiology in patients with DAH. In those scenarios, patients who do not respond to steroids, plasmapheresis should be considered as a rescue treatment to prevent catastrophic outcomes. Herein, we present a unique case of a 48-year-old male admitted with DAH of unknown etiology completely recovered after empirical plasmapheresis.

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“…868 Individual patients with relapses many years after the first presentation of the disease were, however, reported, [883][884][885][886] and repeated relapses may occur in patients who do not cease smoking or are exposed to lung irritants. 887,888 Treatment of patients who do not have detectable anti-GBM antibodies beyond six months is not recommended. Smoking should be strongly discouraged.…”

Section: Practice Point 1122 Plasma Exchange Should Be Performed Until Anti-gbm Titers Are No Longer Detectablementioning

confidence: 99%

Nephrotic syndrome in adults: symptoms and management

2021

Pharmaceutical Journal

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Focal segmental glomerulosclerosis in adults……………………………………………………… 190 Chapter 7. Infection-related glomerulonephritis……………………………………………………………….. 210 Chapter 8. Immunoglobulin and complement-mediated glomerular diseases with an MPGN pattern of injury……………………………………………………………………..……………………………………..

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Multiple recurrences of anti-glomerular basement membrane disease with variable antibody detection: can the laboratory be trusted?

Cited by 15 publications

References 22 publications

Anti-Glomerular Basement Membrane Disease

Anti-Glomerular Basement Membrane Disease

Recurrent alveolar hemorrhage: How do you treat that which you cannot see?

Nephrotic syndrome in adults: symptoms and management

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