Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0034069 (pulmonary fibrosis)
 7,050 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Previous studies of HLA antigen frequencies in asbestos related pulmonary fibrosis have suggested some weak associations both with susceptibility to the disease (B12 and B27) and protection from the disease (B18 and Bw35). HLA typing was performed on a further series of 64 asbestos workers with no chest abnormality and 166 workers with various radiographic changes, 78 having pulmonary fibrosis. The results fail to give statistical confirmation of these associations although B27 was twice as frequently associated with pulmonary fibrosis and diffuse pleural thickening. Analysis of the combined data from this and four other studies failed to show any consistent associations.
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PMID:HLA-A and B antigen frequencies in an asbestos exposed population with normal and abnormal chest radiographs. 44 71

HLA antigens were identified in 64 patients with radiographic asbestosis and 37 matched controls with equivalent asbestos exposure but no radiographic pulmonary fibrosis. A high prevalence of HLA-B18, B27 and Cw2 was found in the controls. This result might indicate that the possessors of these HLA antigens are thus protected from the development of diffuse pulmonary fibrosis from asbestos exposure. Signs of susceptibility were not demonstrated. The radiographic severity and progression of asbestosis were not associated with any of the HLA antigens tested.
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PMID:HLA-B18 antigens and protection from pulmonary fibrosis in asbestos workers. 55 59

HLA antigens were determined in 37 patients with asbestosis and 37 matched controls with equivalent asbestos exposure but no pulmonary fibrosis. All had worked in the same textile factory. No significant differences in the prevalence of antigens were found between the two groups or between either group and controls who had not been exposed to asbestos. When the data were combined with findings from other pilot studies the previously suggested association between asbestosis and HLA-B27 was not confirmed. Subjects who were positive for HLA-B12 tended also to have advanced radiographic fibrosis. Asbestos workers without pulmonary fibrosis had an unexpectedly high frequency of HLA-BW5, which might indicate that this antigen protects against the development of pulmonary fibrosis.
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PMID:Frequency of HLA antigen in asbestos workers with and without pulmonary fibrosis. 84 30

75 systemic sclerosis patients were independently tested for pulmonary fibrosis, autoantibodies, and MHC class II genes. 24 of 42 (57%) patients with pulmonary fibrosis had either HLA DR3/DRw52a or anti-Scl-70 vs 2 of 33 (6%) patients without pulmonary fibrosis. The presence of DR3/DRw52a or anti-Scl-70 gives a relative risk of 16.7 for the development of pulmonary fibrosis in a patient with scleroderma--a risk substantial enough to require careful monitoring of these patients and treatment at an early stage of disease.
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PMID:Immunogenetic prediction of pulmonary fibrosis in systemic sclerosis. 168 77

A 20-year-old Chinese male given an HLA-identical sibling bone marrow transplant for severe aplastic anemia, who had previously had chronic graft-versus-host disease (GVHD) of the mouth, developed myasthenia gravis and widespread pulmonary infiltrates with cough and exertional dyspnea at day 820 post-transplant. There was nothing to suggest aspiration pneumonia. Lung histology at day 940 showed some areas of dense pulmonary fibrosis, some areas of normal parenchyma, and some areas of widening of the interstitium and a mild lymphocytic infiltrate. Evidence of infection was not found. Treatment with cyclosporin and prednisone resulted in slow partial resolution of the infiltrates over 5 months. The myasthenia gravis was controlled with pyridostigmine. In view of the association with myasthenia gravis, of the absence of infectious agents and the response to immunosuppression, we conclude that widespread pulmonary fibrosis can be a major clinical manifestation of chronic GVHD. Examination of lung tissue to distinguish this from infective interstitial pneumonitis is essential.
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PMID:Widespread pulmonary fibrosis as a major clinical manifestation of chronic graft-versus-host disease. 264 79

Interstitial pneumonitis and biopsy-proven pulmonary fibrosis developed in a 35-year-old man with acute myeloblastic leukemia 4 months after conditioning with total body irradiation, etoposide and cyclophosphamide and allogeneic marrow transplantation from an HLA-identical sister. The patient had no evidence of graft-versus-host disease. Under treatment with cyclosporine and prednisone the patient became asymptomatic and radiographic changes of the chest normalized. Following discontinuation of immunosuppressive treatment the patient again became dyspneic, and chest radiographs showed bilateral diffuse interstitial infiltrates. Concurrently there was a rise in serum transaminases. Treatment with prednisone again resulted in resolution of all symptoms and normalization of radiographic and hepatic function abnormalities. This case indicates that late onset interstitial pneumonitis may respond to immunosuppressive therapy. Conceivably, such pulmonary disease may represent the first or only manifestation of chronic graft-versus-host disease.
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PMID:Pulmonary fibrosis after bone marrow transplantation responsive to treatment with prednisone and cyclosporine. 265 Jul 91

We report on four members of a family with rheumatic diseases. Rheumatoid arthritis of an extremely severe and mutilating type developed in a 17-year-old boy (Patient 1). The disease showed some characteristics of juvenile chronic arthritis. 20 years later his sister (Patient 2) was affected by progressive systemic sclerosis (PSS) with arthritis, Raynaud's phenomenon, aperistalsis of the esophagus and pulmonary fibrosis, however without skin involvement. After 2 years rheumatoid arthritis developed in the mother of the family (Patient 3) and another 2 years later the second son (Patient 4) was affected by mixed connective tissue disease (MCTD) with arthritis, Raynaud's phenomenon, aperistalsis of the esophagus and a high titer of antibody to extractable nuclear antigen (ENA). Rheumatoid factor was found in Patient 1, 2 and 3. All members of the family expressed HLA-DR3 in association with HLA-B8. Earlier reports in the medical literature of the familial occurrence of rheumatoid arthritis, progressive systemic sclerosis, systemic lupus erythematosus and other collagen diseases, e.g. mixed connective tissue disease, are reviewed, with a discussion of the possible etiologic mechanisms.
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PMID:[Rheumatoid arthritis, progressive systemic sclerosis without skin involvement and mixed collagen disease in a family. Clinical description of a mother and her 3 adult children--studies of HLA antigens and review of the literature]. 370 74

Immunoglobulin heavy chain (Gm) and kappa light chain (Km) allotype and phenotype frequencies were compared in 173 patients with rheumatoid arthritis (RA) and in 798 controls. No significant differences were found between allotype or phenotype frequencies in overall RA and control groups. However, the Gm(zaxfngb) phenotype and G1m(x) allotype were increased in HLA-DR4 positive RA patients compared with DR4 negative patients and controls, suggesting that immunoglobulin heavy chain genes interact with HLA in the pathogenesis of RA. All four patients with pulmonary fibrosis were Km(1) positive suggesting a possible role for immunoglobulin kappa light chain genes in the pathogenesis of pulmonary fibrosis found in rheumatoid patients.
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PMID:Gm and Km allotypes in rheumatoid arthritis. 392 59

Patients with progressive systemic sclerosis (PSS; scleroderma) were typed for the HLA-A, -B, and -DR antigens. No significant differences in the frequencies of any HLA-A or -B antigen were found. In the subgroup of patients with PSS and diffuse scleroderma (PSS-DS), the frequency of Bw35 was increased (0.30 vs 0.17 in controls; p less than 0.005, corrected P greater than 0.2). Although patients with PSS-DS also had an increased frequency of DR1 antigen (0.27 vs 0.12 in local controls; P less than 0.005, corrected P less than 0.05), no association between Bw35 and DR1 antigens could be detected. We found no increase in the frequencies of the DR3 or DR5 antigens in patients with PSS. However, in a subset of PSS patients with pulmonary fibrosis, an increase in DR3 and a decrease in DR4 antigens (P less than 0.005) were observed. Serum antibodies to centromere occurred more frequently in DR1-positive than DR/-negative patients (0.46 vs 0.18; P less than 0.005). This study of a large number of patients with PSS failed to confirm previously reported associations of PSS with the HLA-B8/DR3 haplotype of HLA-DR5 antigen.
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PMID:Histocompatibility antigens in progressive systemic sclerosis (PSS; scleroderma). 698 3

Several autoantibodies are found specifically in myositis. Mostly this is myositis in the context of a connective tissue disease with associated features such as Raynaud's phenomenon, arthralgias, pulmonary fibrosis or scleroderma. The patterns of disease amount to overlap syndromes or subsets in which one can often predict the presence of a particular type of autoantibody. There are HLA associations, particularly with DQ alleles, related more closely to the antibody than to myositis overall. There is also a suggestion of seasonal and geographical variation within the United States distinguishing the advent of particular autoantibodies with the myositis. The myositis antigens are mainly ribonucleoprotein particles (RNA-protein complexes) functioning in RNA processing, protein translation and protein translocation into the endoplasmic reticulum. Within the cell the antigens are not accessible to antibody, with the possible exception that antibody to U(1)RNP may enter cells. If antibodies are to have a role in pathogenesis it is more likely to be through binding to cell membranes (there to activate complement or mediate T lymphocyte activity) or through immune complex mechanisms. The myositis antigen Ku has been detected on cell membranes, but generally in myositis there is scant deposition of immunoglobulin or complement in muscle except within small blood vessels. If autoantibodies really have little part to play in the pathogenesis of myositis their existence must relate to the earlier events of aetiology, like fingerprints on fragments of a terrorist's bomb.
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PMID:Autoantibodies in myositis. 751 8


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