Cryptogenic Fibrosing Alveolitis and Epstein-Barr Virus: An Association?

Vergnon, Jean‐Michel; Weynants, P.; Vincent, Michel; Mornex, Jf; Brune, J

doi:10.1016/s0140-6736(84)90702-5

Cited by 99 publications

(83 citation statements)

References 13 publications

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“…More reeently, antibodies against human B lymphotropie virus (HBLV) were detected in more than 50% of a group of sareoidosis patients [54]. In CFA, most patients have elevated serum IgA and IgG antibody titres against EpsteinBarr virus-specific antigens [14]. The role of viruses in the aetiology of these diseases needs further investigation.…”

Section: Discussionmentioning

confidence: 99%

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In vivo levels and in vitro production of interferon-gamma in fibrosing interstitial lung diseases

C¹,

Haslam²

1992

Clinical and Experimental Immunology

108

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SUMMARY The in vivo role of interferons in the development of fibrosis is not fully understood but it is known that interferons can suppress fibroblast proliferation and collagen synthesis in vitro. We have recently demonstrated that in a group of patients with sarcoidosis having predominant pulmonary involvement, patients with the highest levels of circulating interferon‐gamma (IFN‐y) more frequently resolved on corticosteroids, suggesting that they had a less‘fibrotic’ component to their disease. We now report that in two other diseases, where the tendency to develop pulmonary fibrosis is greater than in sarcoidosis, namely cryptogenic fibrosing alveolitis (CFA) and fibrosing alveolitis associated with the systemic connective tissue disease progressive systemic sclerosis (FA + PSS), very few patients have elevations in IFN‐γ in their serum. However, as in sarcoidosis, those with the highest levels responded to corticosteroids (P < 0·05). Attempts to measure IFN‐γ levels in the lungs, using cell‐free bronchoalveolar lavage (BAL) fluid supernatants, were negative in all the study groups, suggesting that these samples may be inadequate for such studies. To investigate whether there might be an intrinsic defect in T lymphocyte function associated with predisposition to fibrosing lung diseases, we then investigated the in vitro production of IFN‐y by lymphocytes separated from the blood of 18 untreated patients (six with CFA, six with FA + PSS and six with sarcoidosis). IFN‐γ production was impaired in 10 (56%) (two with CFA, four with FA + PSS and four with sarcoidosis). A higher proportion of the fibrosing alveolitis patients (CFA or FA + PSS) with impaired IFN‐γ production have subsequently shown spontaneous lung functional deterioration. These findings suggest that impaired IFN‐γ release might be a potentiating factor in the pathogenesis of these fibrosing lung diseases.

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Section: Discussionmentioning

confidence: 99%

“…A further aim of this study was to study levels of interferonalpha (IFN-a) in the sera ofpatients with these disorders, in view of evidence that other classes of interferons also inhibit fibroblast proliferation [10] and reports that viruses may be involved in the pathogenesis of sareoidosis [13], CFA [14] and PSS [15].…”

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confidence: 99%

In vivo levels and in vitro production of interferon-gamma in fibrosing interstitial lung diseases

C¹,

Haslam²

1992

Clinical and Experimental Immunology

108

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“…The human herpes viruses (HHVs) are a large family of ubiquitous DNA viruses that include the common pathogens herpes simplex virus type 1 (HSV-1), Epstein-Barr virus (EBV), cytomegalovirus (CMV) and HHV-7 and -8. VERGNON et al [14], in the first study to suggest an association between HHV and IPF, reported that 12 out of 13 IPF patients were seropositive for EBV compared to none of the 12 patients with other forms of interstitial lung disease. Several other studies have reported similar findings of an increased incidence of EBV in lung biopsy and bronchoalveolar lavage (BAL) specimens from IPF TABLE 1 A synopsis of studies linking viruses with the pathogenesis, progression and (acute) exacerbation of idiopathic pulmonary fibrosis (IPF)…”

Section: Infection and The Pathogenesis Of Ipfmentioning

confidence: 99%

The role of infection in the pathogenesis of idiopathic pulmonary fibrosis

Molyneaux

Maher

2013

European Respiratory Review

147

112

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Idiopathic pulmonary fibrosis (IPF) is a progressive, and invariably fatal, condition that is believed to arise in genetically susceptible individuals as a consequence of an aberrant wound-healing response following repetitive alveolar injury. The exact triggers, which initiate the fibrotic process, remain unknown. Infectious agents, including both viruses and bacteria, have the capacity to cause alveolarepithelial cell injury and apoptosis. Relatively few studies have examined the role of infection in IPF. Those that have, point to viruses playing a key role as cofactors in the initiation and progression of IPF. There is also some evidence to suggest that viral infection may be responsible for a proportion of acute exacerbations of IPF. The role played by bacteria in the pathogenesis of IPF is less clear cut. Studies from other respiratory diseases suggest that alterations in the lung microbiome are associated with disease and that these changes influence disease behaviour. Emerging molecular microbiological techniques are making the study of microbial communities in the lung easier. It is hoped that by combining such techniques with the careful longitudinal phenotyping of patients with IPF, it will be possible to elucidate the role played by bacteria and viruses in the pathogenesis of the disease. If infection plays a causal role in IPF then it is possible that therapeutic strategies, utilising currently available antiviral or antibiotic drugs, may be effective in modifying the course of this devastating condition.@ERSpublications Understanding viral and bacterial involvement in IPF could help development of novel therapeutic approaches

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“…Patients with this disorder have elevated levels of anti-EBV antibodies 33 and decreased EBV-specific T cell cytotoxicity. 34 Other autoimmune disorders, such as autoimmune thyroid disease, 35 scleroderma, 36 autoimmune liver disease (primary biliary cirrhosis and autoimmune hepatitis), 37 inflammatory bowel disease (ulcerative colitis and Crohn's disease), 38 as well as cryptogenic fibrosing alveolitis 39 are also associated with EBV. Patients with any of these disorders all have increased EBV DNA loads and increased serum levels of anti-EBV antibodies.…”

Section: Ebv-associated Autoimmune Diseasesmentioning

confidence: 99%