Small Airways in Idiopathic Pulmonary Fibrosis

Fulmer, Jack D.; Roberts, William C.; Gál, Roman; Crystal, Ronald G.

doi:10.1172/jci108811

Cited by 169 publications

(50 citation statements)

References 53 publications

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“…The association between pulmonary fibrosis and HLA genes has been examined in several studies. Association with the HLA class I genes B7, B8 and B12 and the HLA class II gene DR2 was inconsistently found and not confirmed by published studies [118][119][120][121].…”

Section: Pulmonary Fibrosismentioning

confidence: 76%

Immunogenetic basis of environmental lung disease: lessons from the berylliosis model

Saltini¹,

Amicosante²,

Franchi³

et al. 1998

Eur Respir J

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The role of genetic factors has been hypothesized in the pathogenesis of a number of chronic inflammatory lung diseases. The genes of the major histocompatibility complex (MHC) locus on human chromosome 6 have been identified as important determinants in diseases caused both by inorganic and organic compounds such as beryllium, gold, acid anhydrides, isocyanates and grass pollens. Since many environmental factors are the determinants of the immunopathogenesis of asthma, pulmonary granulomatous disorders, hypersensitivity pneumonitis and fibrotic lung disorders, an understanding of the interaction between environmental factors is crucial to epidemiology, prevention and treatment of these disorders. Berylliosis is an environmental chronic inflammatory disorder of the lung caused by inhalation of beryllium dusts. A human leukocyte antigen class II marker (HLA-DP Glu69) has been found to be strongly associated with the disease. In in vitro studies, the gene has been shown to play a direct role in the immunopathogenesis of the disease. In human studies, the gene has been shown to confer increased susceptibility to beryllium in exposed workers, thus suggesting that HLA gene markers may be used as epidemiological probes to identify population groups at higher risk of environmental lung diseases, to identify environmental levels of lung immunotoxicants that would be safe for the entire population and to prevent disease risk associated with occupation, manufactured products and the environment. Studies on the associations between human leukocyte antigens and chronic inflammatory lung disorders are reviewed in the context of the berylliosis model.

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Section: Pulmonary Fibrosismentioning

confidence: 76%

Immunogenetic basis of environmental lung disease: lessons from the berylliosis model

Saltini¹,

Amicosante²,

Franchi³

et al. 1998

Eur Respir J

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show abstract

“…Because of the very strong LD within this haplotype, it is difficult to precisely identify the particular disease-associated HLA allele among them, or distinguish the contributions of these HLA from other interspersed immunoregulatory elements, in lieu of focused, high-resolution genomic studies [21,38,39]. Almost all previous HLA characterizations of IPF patients date from before the development of precise and definitive molecular methodologies that distinguish these alleles [40][41][42][43][44][45] (Table 4). Moreover, only two of these early serologic-based determinations examined even a very restricted repertoire of the many, sincediscovered, Class II alleles [44,45].…”

Section: Discussionmentioning

confidence: 99%

“…The numbers of subjects among those investigations were also usually quite small (Table 4), severely limiting their power to detect intergroup differences. Despite these potential limitations, however, several of those earlier investigations indicated HLA allele frequency perturbations may be present in IPF [40,[43][44][45], although this finding was not invariable [41,42]. In particular, one of these earlier studies indicated that DR2, a serologic correlate of HLA-DRB1*15 and, generally much less frequently, HLA-DRB1*16 gene products, appeared to be over-represented in IPF subjects (45), a finding which may be congruent with the current results.…”

Section: Discussionmentioning

confidence: 99%

The HLA Class II Allele DRB1*1501 Is Over-Represented In Patients With Idiopathic Pulmonary Fibrosis

Xue¹,

Gochuico²,

Feghali‐Bostwick³

et al. 2011

C32. Studies of Lung Fibrosis, Copd and Airway Remodeling

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Background: Idiopathic pulmonary fibrosis (IPF) is a progressive and medically refractory lung disease with a grim prognosis. Although the etiology of IPF remains perplexing, abnormal adaptive immune responses are evident in many afflicted patients. We hypothesized that perturbations of human leukocyte antigen (HLA) allele frequencies, which are often seen among patients with immunologic diseases, may also be present in IPF patients.

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“…Moreover, among patients with RA, the development of interstitial lung disease was associated with HLA-B8 (57), HLA-Dw3 (58), HLA-DQw (59), and several specific HLA haplotypes (60). On the other hand, several studies did not identify a relationship between specific HLA alleles and pulmonary fibrosis among patients with IPF (12,61). These inconsistencies may result from ethnic differences in the distribution of HLA alleles.…”

Section: Candidate Gene Studies In Pulmonary Fibrosismentioning

confidence: 99%

Genetic Analysis of Sporadic and Familial Interstitial Pneumonia

Schwartz

2008

Proceedings of the American Thoracic Society

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Although much progress has been made in understanding the biology and clinical course of interstitial pneumonia, the etiology of this disease remains elusive. Epidemiologic studies have consistently identified cigarette smoke as an important exposure; however, most smokers do not develop interstitial pneumonia and many individuals with interstitial pneumonia do not smoke cigarettes. Moreover, interstitial pneumonias have been reported to cluster in families. Thus, a more thorough understanding of the genetic etiology of interstitial pneumonia may prove critically important in defining the biology and clinical course of this complex human disease.

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Small Airways in Idiopathic Pulmonary Fibrosis

Cited by 169 publications

References 53 publications

Immunogenetic basis of environmental lung disease: lessons from the berylliosis model

Immunogenetic basis of environmental lung disease: lessons from the berylliosis model

The HLA Class II Allele DRB1*1501 Is Over-Represented In Patients With Idiopathic Pulmonary Fibrosis

Genetic Analysis of Sporadic and Familial Interstitial Pneumonia

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