Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
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.
 ...
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.
 ...
PMID:Pulmonary hypertension in the CREST syndrome variant of progressive systemic sclerosis (scleroderma). 84

Late effects after radiotherapy for breast cancer include radiation induced malignancy and changes in irradiated tissues leading to e.g. edema of the arm, decreased mobility of the shoulder joint, brachial plexus neuropathy, pulmonary fibrosis, telangiectasia or atrophic ulceration of the skin. While radiation-induced malignancy depends on the volume of tissue irradiated and the total dose, other late effects are also fractionation dependent. Several reports have shown increased rates of such late effects after changes of the fractionation schedule which should be isoeffective according to the mathematical models commonly used to predict early effects. Although knowledge of the relation between total dose, number of fractions and radiation effects in late responding tissues has increased, extrapolations from the models should be used cautiously. The dose-response curve seems to be steeper for late effects than for tumour control. The possibility of late effects should be included in the decision as to when and how to treat breast cancer with radiotherapy.
 ...
PMID:Late effects of radiotherapy in the treatment of breast cancer. 162 40

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.
 ...
PMID:Pulmonary hypertension in the CREST syndrome: variant of systemic sclerosis (scleroderma)--a case report. 275 66

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).
 ...
PMID:Progressive systemic sclerosis in a child: case report. 824 4

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.
 ...
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.
 ...
PMID:Clinical features and serum antinuclear antibodies in 230 Danish patients with systemic sclerosis. 948 49

Adjuvant radiotherapy to the breast or chest wall is given to some patients with breast cancer, to reduce the risk of local recurrence. It is known to be associated with various late sequelae, including subcutaneous fibrosis, telangiectasia and pulmonary fibrosis. Delivering radiotherapy to the chest wall and nodal drainage areas presents the technical problem of matching the glancing and anterior supraclavicular fields. Overlap between these fields will result in underlying tissues receiving a larger dose than intended; similarly, a gap results in an inadequate dose. We present the case history of a patient with subcutaneous calcification occurring as a late sequela of radiotherapy to the chest wall and anterior supraclavicular field. This has not been previously reported and is thought to have arisen from a high dose region in an area of overlap between fields.
 ...
PMID:Subcutaneous calcification as a late effect of orthovoltage chest wall irradiation. 1046 77

Systemic sclerosis is a multisystem disorder characterized by the presence of connective tissue sclerosis, vascular abnormalities concerning small-sized arteries and the microcirculation, and autoimmune changes. This disease is usually preceded by Raynaud's phenomenon. Cutaneous changes are useful for diagnosis. Sclerosis is initially proeminent on hands with frequently pitted scars. Telangiectases and calcium deposits are mainly observed in a good prognosis subset of the disease (CREST) characterized by the association of limited skin involvement and anti-centromere antibodies with possible late onset of pulmonary hypertension. The bad prognosis subset is associated with diffuse cutaneous sclerosis, pulmonary fibrosis, gastrointestinal disease, myocardial involvement and anti-topo-isomerase I antibodies. Histological skin changes are similar to those observed in morphea. In morphea, there is only localized sclerosis of the skin with highly variable aesthetic disability. The condition may be subdivided clinically into the following types: circumscribed plaques, linear morphea and frontoparietal lesions. The last two forms may be associated with atrophy of the underlying anatomic structures. Many other diseases may have a scleroderma-like appearance. In these diseases, nail folds abnormalities, visceral involvement and immunological changes are usually different from those observed in systemic scleroderma.
 ...
PMID:[How to recognize scleroderma]. 1253 64

Members of two generations of a South African family have a unique syndrome comprising poikiloderma, tendon contractures and progressive pulmonary fibrosis. The condition is clinically important as the skin changes, which involve the face, have considerable cosmetic impact, while lung involvement is potentially lethal in adulthood. Skin manifestations which facilitate diagnosis include facial telangiectasia, mottled hypo- and hyperpigmentation, papules and epidermal atrophy. The scalp, facial and body hair are fine and scanty. The tendon contractures lead to progressive digital flexion deformities and abnormalities of the ankles and feet, with disturbance of gait. Pulmonary involvement manifests as progressive dyspnoea. Pedigree data are compatible with an autosomal dominant mode of transmission. Poikiloderma of Weary is characterized by linear sclerotic and fibrous bands and not tendon contractures and is not associated with potentially lethal pulmonary fibrosis. Rather than name this disorder a variant of Weary syndrome, it might be prudent to use as an umbrella title one composed by Weary himself: 'hereditary sclerosing poikiloderma' (HSP), under which variants such as HSP Weary type, HSP with cardiac involvement (aortic stenosis described as inconsistently associated with Weary syndrome) and HSP with tendon/pulmonary involvement (current family) may be classified. The manifestations in this family differ from other poikilodermata and, to the best of our knowledge, have not been previously documented.
 ...
PMID:Poikiloderma, tendon contracture and pulmonary fibrosis: a new autosomal dominant syndrome? 1703 42


1 2 Next >>