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)

Sixteen patients had biopsy-proved inflammatory sclerosis of the panniculus or fascia, which usually was seen in a generalized distribution. Although the sclerosis involved the digits of five patients, it was the result of centrifugal spread of the sclerosis and was not accompanied by ulcers, calcinosis, distal phalangeal resorption or telangiectasia. Four patients may have had digital vasopasm in the extremities involved in the sclerotic process. Five patients also had cutaneous lesions of dermal morphoea or lichen sclerosus et atrophicus. Four patients had a reduced carbon monoxide diffusing capacity, and one of these had roentgenographic evidence of pulmonary fibrosis. In three patients, oesophageal motility or barium swallow studies showed evidence of sclerodermatous changes. The sclerotic process was a contributory cause of the death of one patient. Five patients had peripheral eosinophilia. Six patients seemed to have responded to anti-inflammatory agents. We believe that subcutaneous morphoea is generally more inflammatory than generalized morphoea of the dermal type and may be more likely to progress to mild systemic sclerosis. We suggest that response of subcutaneous morphoea to anti-inflammatory agents is simply a reflection of the degree of inflammation present.
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PMID:Subcutaneous morphoea: a clinical study of sixteen cases. 45 64

Severe pulmonary hypertension without pulmonary fibrosis occurred in 10 patients with the CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, telangiectasia), reputedly a benign variant of progressive systemic sclerosis. Time from the initial symptom, Raynaud's phenomenon, to the recognition of pulmonary hypertension was as long as 40 years. Pulmonary hypertension and increased pulmonary vascular resistance was shown in all patients. Autopsy examination in three of six deaths attributable to pulmonary hypertension showed intimal proliferation with myxomatous change in the small- and medium-sized pulmonary arteries similar to changes in the digital arteries of patients with scleroderma and Raynaud's phenomenon, and interlobular renal arteries of those with "scleroderma kidney." It is concluded that the CREST syndrome is not entirely benign but may be complicated, after a long clinical course, by progressive pulmonary vascular obliteration, pulmonary hypertension, and death in the absence of significant pulmonary fibrosis.
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PMID:Pulmonary hypertension in the CREST syndrome variant of progressive systemic sclerosis (scleroderma). 84

The clinical and laboratory features of 29 patients who had one of three anti-aminoacyl-tRNA synthetase autoantibodies, anti-Jo1 (histidyl-tRNA synthetase), anti-PL12 (alanyl-tRNA synthetase) or anti-PL7 (threonyl-tRNA synthetase) were analysed and compared with the findings of other published reports. These autoantibodies were found to be associated with a syndrome delineated by inflammatory myositis (24 patients) and pulmonary fibrosis (23 of 29), but also including inflammatory arthritis (26/29), keratoconjunctivitis sicca (17/29), sclerodactyly (21/29), Raynaud's phenomenon (27/29), hepatitis (8/29) and subcutaneous calcinosis (7/29). The most important clinical determinant of outcome in this group of patients was the severity of the interstitial pulmonary disease. No patient fulfilled the classification criteria for systemic lupus erythematosus, although 10 had autoantibodies to extractable nuclear antigens including Ro, La, RNP, and Sm, and two patients had anti-dsDNA antibodies. Although it seems unlikely that anti-aminoacyl-tRNA synthetase antibodies are directly responsible for causing disease, they may provide an important clue to the aetiology of this unusual syndrome.
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PMID:Polymyositis, pulmonary fibrosis and autoantibodies to aminoacyl-tRNA synthetase enzymes. 226 80

The CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia) is a variant but not a benign form of scleroderma. Pulmonary hypertension without pulmonary fibrosis appears to be more prevalent in the CREST than in scleroderma patients. Moreover, pulmonary hypertension in CREST may be rapidly progressive and a cause of sudden death from severe cor pulmonale.
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PMID:Pulmonary hypertension in the CREST syndrome: variant of systemic sclerosis (scleroderma)--a case report. 275 66

20 cases of generalized scleroderma with calcinosis, i.e. Thibierge-Weissenbach syndrome, were reviewed concerning the clinical and radiological findings. 60% of the patients complained of esophageal dysfunction, and in 85% radiological abnormalities of the esophagus were found. Gastro-intestinal complaints, with or without radiological changes, were found in accordance with other reports. Decreased pulmonary function was a frequent feature, but only half of these cases showed pulmonary fibrosis on chest X-ray. Calcinosis primarily affects areas of sclerosis, but can be found elsewhere. Six of our patients had intervertebral calcifications in the thoracic and/or lumbar spine. This seems to be a new observation in patients with scleroderma.
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PMID:Calcosis in generalized scleroderma. 616 74

Side effects of bleomycin therapy are most frequent in the skin and as pulmonary fibrosis because of the nearly selective concentration in these organs. The cutaneous manifestations include a variety of clinical symptoms, most frequently erythema and infiltrations with marked hyperpigmentation. A 38-year-old patient is reported who received 615 mg bleomycin for therapy of a malignant testicular teratoma and in the following time developed numerous morphoea-like lesions, acrocyanosis, Raynaud's phenomenon, acrosclerosis, and cutaneous calcinosis. Histologically, the alterations were similar to those of scleroderma. In contrast to progressive systemic sclerosis the symptomatic character of the lesions is evident, for the skin changes resolved several months after the drug was discontinued.
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PMID:[Symptomatic scleroderma caused by bleomycin]. 618 16

A case of pediatric progressive systemic sclerosis is reported and a literature review concerning medical and dental aspects of this condition is provided. Systemic features include sclerodactyly, Raynaud's phenomenon, telangiectasia, calcinosis, myositis, arthritis, tenosynovitis, renal failure, esophageal hypomotility, pulmonary fibrosis and heart failure. Oral manifestations include reduced interincisal distance, xerostomia, telangiectasia, increased periodontal ligament width, osseous resorption of the mandible, periodontal disease, and increased decayed, missing, and filled teeth (DMFT). The prognosis is difficult to predict because spontaneous remission has been documented, but death may result from extensive visceral involvement (heart, kidney, and lung).
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PMID:Progressive systemic sclerosis in a child: case report. 824 4

Over 100 cases of disorders closely resembling classic autoimmune diseases have been reported among patients who were injected or implanted with a diverse group of chemicals including paraffins, vegetable oils or silicone. Most cases have occurred in silicone breast implant recipients, especially those who received their prostheses 2-10 years prior to onset of symptoms. A high proportion of patients exhibit classic signs and symptoms of Sjogren's syndrome or scleroderma. Affected patients typically experience some combination of fatigue, myalgia, joint pain, sicca syndrome (dry eyes and mouth), synovitis, rash, alopecia, muscular weakness or lymphadenopathy, and autoantibody formation. Less commonly, patients may have the CREST syndrome (calcinosis, Raynaud's phenomena, esophageal hypomotility, sclerodactyly and telangiectasias), hypertension, pulmonary fibrosis, or central nervous system pathology.
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PMID:Silicone-reactive disorder: a new autoimmune disease caused by immunostimulation and superantigens. 828 1

This review discusses the current concepts of immunological tolerance, physiological vs. pathological autoimmunity, autoimmune diseases, and laboratory tests helpful in diagnosis. The autoantibodies in organ-specific autoimmune diseases are directed against antigens of the injured organs, whereas the antinuclear antibodies (ANA) detected in systemic autoimmune diseases are detected against a vast array of nuclear and intracellular antigens and peptides necessary for DNA/RNA synthesis, repair, splicing, and transcription. Knowledge of the mean titer and presence or absence of specific ANA types will help predict the nature of the disease and the response to therapy. Noteworthy features of these "ANA profiles" are (1) patients with systemic lupus erythematosus frequently have multiple types of ANA but anti-dsDNA and anti-SM are diagnostic, (2) patients with drug-induced lupus have ANA restricted to antihistone, (3) patients with mixed connective tissue disease have ANA restricted to anti-RNP, (4) patients with CREST (calcinosis, Raynaud's, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome have ANA restricted to anticentromere, (5) ANA with anti-SS-A/Ro specificity is associated with vasculitis and nephritis, (6) ANA with anti-SS-B/La and anti-nRNP specificities is associated with milder clinical disease, (7) ANAs with anti-Jo-1 and PM-Scl specificities are associated with pulmonary fibrosis and poor prognosis. Technological advances in the fields of molecular immunogenetics are guiding the studies of autoimmune diseases from serological and histopathological evaluations toward search for subcellular risk factors such as chemical and biological agents and susceptibility genes. Knowledge of these factors will help (1) to identify disease susceptibility genes prior to clinical onset and irreversible tissue damage, (2) to avoid environmental risk factors, and (3) to devise specific immunosuppressive strategies.
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PMID:Current concepts and advances in clinical laboratory testing for autoimmune diseases. 922 6

The objective was to investigate the relationship between the presence of different types of antinuclear antibodies (ANA) in patients with systemic sclerosis (SSc) and the presence of clinical features. Sera from 230 patients with SSc were tested for the presence of ANA, including anticentromere antibodies (ab), antitopoisomerase I ab, anti-U1 RNP ab and antinucleolar ab, including anti-Th RNP, anti-U3 RNP and anti-U17 RNP. Clinical features were registered prospectively in a clinical database. Eighty-two per cent of the patients were women. The median age was 58 yr (45-67, quartiles) and median age at disease onset was 44 (30-55) yr. ANA were found in 86% of the patients (anticentromere: 34%; antitopoisomerase I: 14%; anti-U1 RNP: 6.5%; antinucleolar total: 16%; anti-Th RNP: 2.2%; anti-U3 RNP: 3.5%; anti-U17 RNP: 0%). Anticentromere ab were found to be related to a high prevalence of calcinosis, telangiectasia, digital ulcers, acrosclerosis, primary biliary cirrhosis, isolated reduction of pulmonary diffusing capacity, and a low prevalence of radiological evidence of pulmonary fibrosis. Antitopoisomerase I ab were associated with a high prevalence of digital joint deformity, distal osteolysis, radiological signs of pulmonary fibrosis, a low prevalence of calcinosis and late onset of disease. Anti-U1 RNP ab were related to a high prevalence of arthritis and myositis, a low prevalence of calcinosis, and early disease onset. The presence of antinucleolar ab, including anti-U3 RNP and anti-Th RNP, was not significantly related to any particular clinical features in this study; possibly due to the small number of patients with these ab. The presence of anticentromere, antitopoisomerase I and anti-U1 RNP ab in the serum was also found to have previously described clinical correlations in a group of Danish SSc patients.
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PMID:Clinical features and serum antinuclear antibodies in 230 Danish patients with systemic sclerosis. 948 49


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