Goodpasture's Syndrome with Negative Anti-glomerular Basement Membrane Antibodies - PubMed (original) (raw)

Goodpasture's Syndrome with Negative Anti-glomerular Basement Membrane Antibodies

Tjitske Berends-De Vries et al. Eur J Case Rep Intern Med. 2017.

Abstract

A young male patient with rapidly progressive and life-threatening pulmonary haemorrhage due to anti-glomerular basement membrane (anti-GBM) antibody disease without renal involvement repeatedly tested negative for serum anti-GBM antibodies. Although rare, anti-GBM antibody disease should be considered in the differential diagnosis in patients with life-threatening pulmonary haemorrhage due to isolated diffuse alveolar haemorrhage. Enzyme-linked-immunosorbent assay (ELISA) testing for anti-GBM antibodies in anti-GBM antibody disease can give false-negative results. A negative serum anti-GBM antibody test is therefore insufficient to exclude the diagnosis. Thus, a kidney or lung biopsy should be considered in any case with a high clinical suspicion but negative anti-GBM antibody test to confirm or rule out the diagnosis.

Learning points: Diffuse alveolar haemorrhage (DAH) is a life-threatening disorder caused by severe damage due to injury or inflammation of the alveolar-capillary basement membrane.Anti-GBM antibody disease is a rare autoimmune disorder with circulating autoantibodies directed against the alpha-3 chain[Q2] of type VI collagen of the glomerular and/or alveolar basement membrane which may result in oliguric acute kidney failure due to rapidly progressive glomerulonephritis with or without DAH (commonly referred to as Goodpasture's syndrome).A kidney or lung biopsy should be considered to confirm or rule out the diagnosis if there is a high clinical suspicion but the anti-GBM antibody test is negative; prompt diagnosis and initiation of plasmapheresis, cyclophosphamide and prednisone therapy is essential.

Keywords: Goodpasture; anti-GBM antibodies; diffuse alveolar hemorrhage.

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Conflict of interest statement

Conflicts of Interests: The Authors declare that there are no competing interests.

Figures

Figure 1

Figure 1

Postero-anterior chest radiograph obtained on the day of admission showing bilateral consolidations and air bronchogram

Figure 2

Figure 2

Chest CT scan showing extensive bilateral ground glass opacities

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References

    1. Jung C, Karpouzas G, Stringer WW. Dyspnea, hemoptysis, and perihilar infiltrates in a 35-year-old man. Chest. 2005;127:1437–1441. - PubMed
    1. Greco A, Rizzo MI, De Vergilio A, et al. Goodpasture’s syndrome: a clinical update. Autoimmun Rev. 2015;14:246–253. - PubMed
    1. Serisier DJ, Wong RCW, Armstrong JG. Alveolar haemorrhage in anti-glomerular basement membrane disease without detectable antibodies by conventional assays. Thorax. 2006;61:636–639. - PMC - PubMed
    1. Silvarino R, Noboa O, Cervera R. Anti-glomerular basement membrane antibodies. Isr Med Assoc J. 2014;16:727–732. - PubMed
    1. Ohlsson S, Herlitz H, Lundberg S, et al. Circulating anti-glomerular basement membrane antibodies with predominance of subclass IgG4 and false negative immunoassay test results in anti-glomerular basement membrane disease. Am J Kidney Dis. 2014;63:289–293. - PubMed

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