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At least 865 records · Page 48Linked to original sources
PubMed1986

Lung function abnormalities in different connective tissue diseases.

Lung volumes, forced expiratory flow-volume curves, diffusing capacity indexes, and arterial blood gases were measured in 72 non-smoking patients with various connective tissue diseases (13 with rheumatoid arthritis, 17 with systemic lupus erythematosus, 25 with progressive systemic sclerosis, 10 with primary Sjögren's syndrome, 4 with polymyositis, and 3 with mixed connective tissue disease). Small airways disease and a diffusion capacity impairment were the most frequent and marked functional abnormalities in the whole group, and were often present in asymptomatic patients. Different lung function defects seemed to be present in each disease group. In fact, large airway obstruction was prevalent in progressive systemic sclerosis, diffusion capacity impairment in systemic lupus erythematosus, and small airways disease in rheumatoid arthritis. In contrast, primary Sjögren's syndrome appeared to be the connective tissue disease in which lung function abnormalities were less frequent and less pronounced.

Adolescent↗
PubMed1993

Medical treatment of esophageal motility disorders.

Swallowing is a complex mechanism that is based on the coordinated interplay of tongue, pharynx, and esophagus. Disturbances of this interplay or disorders of one or several of these components lead to dysphagia, non-cardiac chest pain, or regurgitation. The major esophageal motility disorders include achalasia, diffuse esophageal spasm, hypercontractile esophagus ("nutcracker esophagus"), and hypocontractile esophagus ("scleroderma esophagus"). Other esophageal diseases such as hypopharyngeal (Zenker's) diverticula or gastroesophageal reflux disease also may be sequelae of primary esophageal motility disorder. Finally, a substantial group of patients referred for evaluation of possible esophageal motor disorders have milder degrees of dysmotility--referred to as nonspecific esophageal motor disorder--that are of unclear clinical significance. Medical treatment of esophageal motility disorders involves the uses of agents that either reduce (anti-cholinergic agents, nitrates, calcium antagonists) or enhance (prokinetic agents) esophageal contractility. Despite the beneficial effect of the various drugs on esophageal motility parameters, the clinical benefit of medical treatment is often disappointing. From clinical and epidemiological studies there is some evidence for a "psychological" component in the pathogenesis or perception of esophageal symptoms. Further understanding of esophageal pathophysiology, as well as development of new receptor selective drugs, might increase our chances of successful treatment of esophageal motility disorders.

Esophageal Motility Disorders↗
PubMed1983

Absence of positional change in pulmonary diffusing capacity in systemic sclerosis.

Patients with systemic sclerosis frequently have pulmonary function abnormalities, and recent evidence suggests that pulmonary vascular involvement is a common manifestation. To test the hypothesis that patients with systemic sclerosis have impaired ability to recruit or distend the pulmonary vascular bed, the postural change in the coefficient of carbon monoxide diffusing capacity was measured in 11 patients with systemic sclerosis, and the results were compared with results from age-, smoking-, and sex-matched control subjects with rheumatoid arthritis and with results from healthy subjects. In normal subjects and patients with rheumatoid arthritis increased, the coefficient of diffusion by 9.4 percent (p less than 0.005) and 8.4 percent (p less than 0.01), respectively, when they moved from the sitting to the supine position. In contrast, patients with systemic sclerosis did not show a significant increase in coefficient of diffusion, even those who had otherwise normal pulmonary function. Regression analyses showed that the change in coefficient of diffusion decreased with increasing age (r = -0.57) in normal subjects, and that the change in coefficient of diffusion was a function of the percent predicted forced vital capacity, both in patients with systemic sclerosis (r = 0.59) and in those with rheumatoid arthritis (r = 0.70). Thus, these findings indicate that patients with systemic sclerosis have a nondistensible pulmonary capillary bed and that the absence of positional change in the coefficient of diffusion in systemic sclerosis is a subtle indicator of pulmonary involvement.

Adult↗
PubMed2003

Synovial remodeling process induced by type V collagen immunization in rabbits.

The pathogenesis of diffuse connective tissue diseases is still unknown despite studies of the autoimmunity aspects related to extracellular matrix elements, mainly the collagens. Articulations are frequently affected by the synovitis process in these diseases. The objective of the present study was to verify the morphologic aspects of the synovial membrane of rabbits immunized with type V collagen, which has some particular characteristics 75 days after the first antigen inoculation and when compared to control animals. The synovial membrane of the animals sacrificed after 75 days of immunization presented an intense remodeling phenomenon along the connective tissue screen and interlobular septa of the adipose-muscle tissue screen compartment. The remodeling process determined type I and III collagen fiber depositions in the vascular and connective tissue compartments of the synovial membrane. The nutrient vessels of the adipose-muscle compartment showed a similar remodeling process, which resulted in small vessel occlusion. Few residual inflammatory foci consisting of monocytes and eosinophils were observed. Thus, our experimental model reproduces morphologic changes in different tissues, characterized by an extracellular matrix remodeling process similar to those observed in many diffuse connective tissue diseases such as systemic lupus erytematosus and scleroderma. Therefore, this model could be useful in understanding the pathogenesis and the treatment of these diseases.

Animals↗
PubMed2003

Interstitial lung disease in systemic sclerosis.

PURPOSE: To evaluate high-resolution CT (HRCT) parameters of inflammation and fibrosis in systemic sclerosis (SSc), for correlation with lung function, skin scores and exercise tolerance. MATERIAL AND METHODS: 45 SSc patients (40 women, 48.5+/-13.4 years), underwent thoracic HRCT, lung function assessment, and modified Rodnan skin scores. Exercise tolerance was also graded. HRCT were scored for extent of 4 HRCT patterns of interstitial lung disease (ILD): ground glass opacification (GGO), reticular, mixed and honeycomb pattern in each lobe. Total HRCT score, inflammation index (GGO and mixed score) and fibrosis index (reticular and honeycomb scores) were correlated with lung function and clinical parameters. RESULTS: ILD was present in 39/45 (86.7%) patients. Abnormal (<80% predicted) forced vital capacity (FVC), total lung capacity (TLC) and carbon monoxide diffusion factor (DLco) were detected in 30%, 22% and 46% of patients. Total HRCT score correlated with FVC (r=-0.43, p=0.008), FEV1 (forced expiratory volume) (r=-0.37, p=0.03), TLC (r=-0.47, p=0.003), and DLCO (r=-0.43, p=0.008); inflammatory index with DLCO (r=-0.43, p=0.008) and exercise tolerance (r=-0.39, p < 0.05); and fibrosis index with FVC (r=-0.31, p=0.05) and TLC (r=-0.38, p=0.02). Higher total HRCT score, and inflammation and fibrosis indices were found in patients with abnormal lung function. CONCLUSION: Qualitative HRCT is able to evaluate inflammation and fibrosis, showing important relationships with diffusion capacity and lung volume, respectively.

Exercise Tolerance↗
PubMedDate not supplied

Elevated whole blood chemiluminescence in patients with systemic sclerosis.

OBJECTIVE: Systemic sclerosis (SSc) is accompanied by oxidative stress that in turn may accelerate endothelium degeneration and thus disease progression. We tested whether phagocytes from SSc patients release more reactive oxygen species (ROS) and whether this release correlates with some clinical parameters. METHODS: ROS production by blood phagocytes was measured with the luminol enhanced whole blood chemiluminescence (CL). Resting and N-formyl-methionyl-leucyl-phenylalanine -induced CL (fMLP-induced CL) was measured in 30 patients with SSc and 30 healthy controls matched as to age, sex, and level of cigarette smoking. RESULTS: Resting CL and fMLP-induced CL calculated per 10(4) phagocytes present in the assayed blood sample were higher in patients with systemic sclerosis than in healthy controls (median; range, 0.88; 0.47-1.39 vs. 0.73; 0.13-1.07 aU/10(4)p and 621; 293-3522 vs. 411; 289-810 aUxs/10(4)p, p<0.02). Patients treated with cyclophosphamide and/or prednisone for 11; 3-168 months did not differ in respect to CL from those that never received the medications. Similarly, no significant differences were found between patients with limited and diffuse SSc. Resting CL correlated (p<0.05) with clinically manifested interstitial lung disease (r=0.59), single breath carbon monoxide diffusing capacity (r= -0.56) and serum autoantibodies titre (r= 0.43). CONCLUSIONS: Blood phagocytes from patients with systemic sclerosis, especially from those with interstitial lung disease, generate elevated amounts of ROS as assessed with CL. This confirms the presence of systemic oxidative stress in SSc patients.

Adolescent↗
PubMed1991

Failure of vasoldilator infusion to alter pulmonary diffusing capacity in systemic sclerosis.

PURPOSE: Patients with systemic sclerosis (SSc) do not exhibit a normal increase in the diffusing capacity for carbon monoxide (DLCO) on assuming the supine position. We sought to determine whether a potent prostacyclin derivative and vasodilator, iloprost, would reverse this defect. PATIENTS AND METHODS: Fourteen patients with SSc were enrolled in a randomized, double-blind, placebo-controlled study of iloprost. Patients were tested before and during 3 days of iloprost or placebo infusion with both upright and supine pulmonary function studies. RESULTS: The results of baseline pulmonary function studies including DLCO were not significantly altered by iloprost. Furthermore, iloprost did not alter the abnormal postural DLCO response. CONCLUSION: These results suggest that the pulmonary vascular defects seen in this group of patients are not a consequence of reversible pulmonary vasospasm.

Adult↗
PubMed1992

Evaluation of commercial enzyme immunoassays compared to immunofluorescence and double diffusion for autoantibodies associated with autoimmune diseases.

A commercially available enzyme immunoassay system for detecting autoantibodies to double-stranded DNA, deoxyribonucleoprotein, Smith, ribonuclearprotein, Sjögren's syndrome-associated antigens A and B, and scleroderma-associated antigen 70 was compared to the conventional immunofluorescence assay for double-stranded DNA and double diffusion assays for extractable nuclear antigens. There was excellent correlation between methods, but it appears that the enzyme immunoassays are more sensitive. Based on the results of this study, the authors recommend performing anti-nuclear antibody screening at two dilutions, with enzyme immunoassay follow-up of appropriate patient sera that are positive on anti-nuclear antibody testing. Nucleolar and centromere pattern anti-nuclear antibodies are diagnostic for variants of scleroderma and need no further evaluation. Negative anti-nuclear antibody tests performed using HEp-2 tissue culture cells require no further evaluation.

Antibodies, Antinuclear↗
PubMed1975

[Silicosis associated with scleroderma].

Such an association would not appear to be fortuitous since scleroderma is commoner in male miners than in women in general, whilst the predominance of the disease in the latter group is well known. The picture is that of generalised scleroderma without any special characteristics. Silicosis usually precedes the scleroderma. In general, the onset of scleroderma alters the chest X-ray appearance with the development of diffuse, non-cavitated macro-nodular opacities localised in the lower two-thirds of the lungs and in particular at the bases, without absolute symmetry. Several hypotheses may be advanced to explain this association. Irritation by dust in the presence of a particular underlying terrain may cause silicosis and the same terrain may be associated with the development of scleroderma. A second possibility is that of immune phenomena secondary to the pneumoconiosis which favourise the development of scleroderma.

Aged↗
PubMed1976

Scleroderma in childhood.

Scleroderma in childhood is a rare but confusing disorder. Systemic and diffuse disease do occur but are uncommon. Much more common are local lesions of different types which can be multiple; these are often associated with joint symptoms and the development of joint contractures and tendon contractures due to nodule formation. In such circumstances the condition mimics juvenile chronic polyarthritis (Still's disease). A third, rare variant, sclerodermatous fasciitis, has also been described in one young patient.

Adolescent↗
PubMed2000

Cyclophosphamide is associated with pulmonary function and survival benefit in patients with scleroderma and alveolitis.

BACKGROUND: Lung inflammation (alveolitis) may cause lung fibrosis in scleroderma. OBJECTIVE: To determine whether cyclophosphamide treatment is associated with retention of lung function and improved survival in scleroderma patients with alveolitis. DESIGN: Retrospective cohort study. SETTING: Johns Hopkins and University of Maryland Scleroderma Center. PATIENTS: 103 patients with scleroderma who had bronchoalveolar lavage or lung biopsy. INTERVENTION: Cyclophosphamide therapy. MEASUREMENTS: 1) Serial measurement of forced vital capacity (FVC) and carbon monoxide diffusing capacity and 2) survival. RESULTS: During a median follow-up of 13 months after bronchoalveolar lavage or biopsy, patients with alveolitis who did not receive cyclophosphamide therapy experienced a decrease in FVC (mean difference, -0.28 L [95% Cl, -0.41 to -0.16 L] and -7.1% of the predicted value [Cl, -10.9% to -4.0%]). Carbon monoxide diffusing capacity also decreased in these patients (mean difference, -3.3 x mmol min(-1) x kPa(-1) [Cl, -4.6 to -2.1 mmol x min(-1) x kPa(-1)] and -9.6% of the predicted value [Cl, -16.7% to -2.4%]). During a median follow-up of 16 months, patients with alveolitis who received cyclophosphamide were more likely to have a good outcome (stabilization or improvement) in FVC (relative risk, 2.5 [Cl, 1.5 to 4.1]) and diffusing capacity (relative risk, 1.5 [Cl, 1.0 to 2.2]). These patients also had improved survival; the median survival rate was 89% (25th, 75th percentiles, 84%, 94%) compared with 71% (25th, 75th percentiles, 55%, 86%) in untreated patients (P = 0.01, log-rank test). CONCLUSIONS: The presence of lung inflammation identifies patients with scleroderma who are more likely to have worsening lung function. Lung function outcomes and survival are improved in patients with alveolitis who receive cyclophosphamide.

Biopsy↗