Goodpasture's syndrome: Recurrence after a five-year remission

Klasa, Richard; Abboud, Raja T.; Ballon, Henry S.; Grossman, Lawrence

doi:10.1016/0002-9343(88)90114-3

Cited by 27 publications

(15 citation statements)

References 16 publications

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“…10 Consistent with this, the vast majority of reported cases of recurrent disease had recrudescence of detectable anti-GBM antibodies at clinical recurrence, despite prior immunosuppressive therapy. [10][11][12][13][14][15] Furthermore, there is no evidence that prior immunosuppressive therapy alters the ability to detect anti-GBM antibodies by changing the epitopes recognised by the antibodies or otherwise interfering with the assays used.…”

Section: Discussionmentioning

confidence: 99%

Alveolar haemorrhage in anti-glomerular basement membrane disease without detectable antibodies by conventional assays

Serisier

Wong²,

Armstrong³

2006

Thorax

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Section: Discussionmentioning

confidence: 99%

Alveolar haemorrhage in anti-glomerular basement membrane disease without detectable antibodies by conventional assays

Serisier

Wong²,

Armstrong³

2006

Thorax

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“…The anti-GBM antibodies seem to disappear spontaneously after 12-18 months (Levy et al, 1996). However, several reports demonstrated recurring cases with anti-GBM disease (Adler et al, 1981;Hind et al, 1984;Klasa et al, 1988;Levy et al, 1996). In our survey (Hirayama et al, 2008), relapse or recurrence was also rare in patients with anti-GBM disease (13.9%) in comparison with patients with ANCA-associated vasculitis, such as WG (29.4%) and MPA (29.3%).…”

Section: Relapse/recurrencementioning

confidence: 45%

“…In another study, alveolar hemorrhage was present in 100% of patients who smoked and in only 20% of nonsmokers with Goodpasture's disease (Donaghy & Rees, 1983). No significant difference in circulating anti-GBM antibody titers was found between smokers and nonsmokers, suggesting that cigarette smoking may increase the permeability of lung capillaries and thus expose alveolar basement membranes to circulating anti-GBM antibodies (Donaghy & Rees, 1983;Klasa et al, 1988). Other inhaled substances may also be associated with anti-GBM disease, including cocaine (García-Rostan y Pérez et al , 1997;Lazor et al, 2007), hard metals such as inert tungsten carbide and cobalt (Lechleitner et al, 1993), smoke inhalation (Klasa et al, 1988) and possibly volatile hydrocarbon solvents (Beirne & Brennan, 1972;Bombassei & Kaplan, 1992).…”

Section: Environmental and Genetic Factorsmentioning

confidence: 98%

Anti-Glomerular Basement Membrane Disease

Hirayama¹,

Yamagata²

2011

An Update on Glomerulopathies - Clinical and Treatment Aspects

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“…Lazor et al showed that 89% of their patients with alveolar hemorrhage were active smokers [9], and Donaghy et al demonstrated that alveolar hemorrhage was present in 100% of their patients who smoked, and only in 20% of nonsmokers with Goodpasture's disease. No significant difference in circulating anti-GBM antibody titers was found between smokers and non-smokers, suggesting that cigarette smoking may increase the permeability of lung capillaries and thus expose alveolar basement membranes to circulating anti-GBM antibodies [14,15].…”

Section: Pathobiology and Immunologymentioning

confidence: 88%

Cutting Edge Issues in Goodpasture’s Disease

Chan

Louie

Leslie

et al. 2011

Clinic Rev Allerg Immunol

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Goodpasture's disease, or anti-glomerular basement membrane (anti-GBM) disease, is a systemic autoimmune disorder defined by anti-GBM antibody-mediated damage (mainly immunoglobulin G-1) resulting in progressive crescentic glomerulonephritis and, frequently, diffuse pulmonary alveolar hemorrhage. It may be regarded as a "conformeropathy" where the quaternary structure of the α345NC1 hexamer that constitutes GBM undergoes a conformational change, exposing pathogenic epitopes on the α3 and α5 chains, eliciting a pathogenic autoantibody anti-GBM response. Goodpasture's disease accounts for 20% of all patients presenting with a pulmonary-renal syndrome and may be associated with detectable perinuclear antineutrophil cytoplasmic autoantibody positivity in up to a third of patients. Associated triggers may include tobacco smoking, hydrocarbon solvent exposure, and cocaine abuse. Cough, hemoptysis, and dyspnea with fatigue are the commonest presenting features. It is critical to rapidly distinguish Goodpasture's disease from other causes of pulmonary-renal syndromes such as Wegener's granulomatosis. Early and intensive treatment with plasmapheresis and immunosuppression with systemic corticosteroids pending results of diagnostic testing, and later cyclophosphamide, is often beneficial, with 90% of patients surviving the acute presentation of Goodpasture's disease. The need for hemodialysis on initial presentation, a serum creatinine >5 mg/dL, and 50% to 100% crescents on renal biopsy, portend the necessity of long-term hemodialysis. Further elucidation of the molecular pathobiology of Goodpasture's disease, particularly the regulation of involved antigen-specific T cells, may improve early diagnosis, treatment, and outcomes in this rare but potentially lethal autoimmune disorder.

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Goodpasture's syndrome: Recurrence after a five-year remission

Cited by 27 publications

References 16 publications

Alveolar haemorrhage in anti-glomerular basement membrane disease without detectable antibodies by conventional assays

Alveolar haemorrhage in anti-glomerular basement membrane disease without detectable antibodies by conventional assays

Anti-Glomerular Basement Membrane Disease

Cutting Edge Issues in Goodpasture’s Disease

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