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At least 937 records · Page 52Linked to original sources
PubMed1998

Abnormal uptake of Tc-99m MIBI, a novel myocardial imaging agent, in the lungs of patients with systemic sclerosis.

OBJECTIVE: Fibrosing alveolitis is a prominent feature of systemic sclerosis (SSc), and accounts for much of the morbidity and mortality of this disease. Sensitive techniques for the detection and monitoring of fibrosing alveolitis could target patients for early therapeutic intervention. The objective of this small pilot study was to assess the frequency and clinical significance of abnormal lung uptake of Tc-99m MIBI, a novel radionuclide imaging agent that selectively accumulates in cells rich in mitochondria. METHODS: Sixteen patients with SSc and evidence of pulmonary involvement were studied. The uptake of radionuclide in the lungs, and the ratio of pulmonary to cardiac uptake were evaluated after intravenous injection of Tc-99m MIBI. Results were correlated with clinical and laboratory parameters. RESULTS: Lung uptake of Tc-99m MIBI was increased in all 16 SSc patients compared to control patients with coronary heart disease but no evidence of pulmonary abnormality. The degree of isotope uptake in the lungs was correlated with the extent of maximal skin induration and with radiologic evidence of interstitial lung disease, but not with other clinical or laboratory parameters of disease activity or extent of pulmonary involvement. The ratio of pulmonary to cardiac uptake of isotope was also increased in patients with SSc compared to controls. CONCLUSION: Accumulation of Tc-99m MIBI is abnormally elevated in the lungs of SSc patients with pulmonary involvement. Isotope accumulation in the lungs may be related to activation of fibroblasts or endothelial cells. The specificity and sensitivity of Tc-99m MIBI scanning in the detection and monitoring of pulmonary involvement, and its potential role in the management of SSc, deserve further investigation.

Coronary Disease↗
PubMed1984

Clinical and demographic predictors of loss of pulmonary function in systemic sclerosis.

The course of functional pulmonary involvement in systemic sclerosis remains controversial; and it is not known if specific clinical or demographic features are predictive of subsequent changes in pulmonary function. To address these questions, we conducted a non-concurrent prospective study of serial pulmonary function in 24 patients with systemic sclerosis unselected for pulmonary involvement over a mean follow-up interval of 59.7 months. Initial values for the entire group demonstrated a mild restrictive defect with a mild reduction in gas exchange. Although a restrictive pattern was most common, normal and obstructed pulmonary function were seen. Mean rates of change of FVC, TLC, FEV1/FVC, and Dco for the entire group were not different from normal, but substantial variability in the course of pulmonary functional involvement was seen among individuals. Changes in gas transfer, lung volumes, and airflow can occur independent of each other. Rates of change in pulmonary function were not predicted by initial pulmonary function, race, sex, duration of disease, cardiac involvement, roentgenographic fibrosis, or regression of skin disease. Patients with severe Raynaud' phenomenon exhibited the greatest fall in Dco over time, suggesting an association between peripheral and pulmonary vascular involvement. A correlation between exertional dyspnea and rapid loss of Dco was noted. Former smokers had significantly greater rates of loss of FVC and Dco than either non-smokers or current smokers, suggesting that cessation of smoking was a response to rapidly declining function in a subgroup of susceptible smokers. We detected a wide spectrum of severity of pulmonary prognosis in systemic sclerosis, ranging from normal pulmonary function to rapidly progressive disease leading to death. This study indicates that patients with prolonged survival do not necessarily have a rapidly progressive pulmonary component, but those with severe Raynaud's phenomenon and susceptible smokers are at very high risk for rapid deterioration of pulmonary function.

Adult↗
PubMed1984

Lung function tests in connective tissue diseases associated with Raynaud's phenomenon.

13 patients with various connective tissue diseases associated with Raynaud's phenomenon were studied with pulmonary physiologic techniques to see the alterations of lung functions and also whether spasm of pulmonary circulation occurs in these patients. We found that an increase in the dead space ventilation was common and associated with normal tidal volume. We interpreted this finding as evidence of redistribution of blood flow in the lung by spasm of blood vessels going to well-ventilated lung units generating a high dead space ventilation. We also found commonly that the distribution of inspired air in the lung was uneven, the diffusing capacity was reduced and the dynamic compliance decreased with increasing frequency of breathing suggestive of disease in small airways. The restrictive defect, the obstructive defect, the reduction of lung compliance and the arterial hypoxemia were relatively uncommon and probably occurred when the diseases were more advanced.

Adult↗
PubMed1989

Pulmonary disease in progressive systemic sclerosis. A complication of gastroesophageal reflux and occult aspiration?

Thirteen patients with progressive systemic sclerosis were studied to evaluate the possible role of gastroesophageal reflux as a contributing pathogenic factor in the pulmonary disease of the patients. The evaluation of all patients included fiberoptic esophagogastroduodenoscopy with biopsies of the esophagus, otolaryngologic evaluation, technetium Tc 99m sulfur colloid aspiration scan, pulmonary function testing, including the diffusing capacity for carbon monoxide (DLCO) test, and 24-hour intraesophageal pH monitoring with probes placed 5 and 15 cm above the lower esophageal sphincter. Eleven patients had microscopic and macroscopic evidence of proximal esophagitis, 12 patients had laryngeal changes suggestive of aspiration, and 12 patients had abnormal DLCO values. Using multiple regression analysis, the degree of DLCO impairment correlated with the proximal and distal reflux episodes and scores recorded by pH monitoring. There was direct and indirect evidence for proximal gastroesophageal reflux and aspiration in the majority of patients, and a distinct correlation between the severity of reflux and the severity of pulmonary disease. Aggressive antireflux therapy may be helpful in reducing the pulmonary damage due to aspiration in these patients.

Adult↗
PubMed1994

Inverse relation between plasma concentration of von Willebrand factor and CrEDTA clearance in systemic sclerosis.

OBJECTIVE: Microvascular abnormalities involving endothelial cell dysfunction occur as an early event in systemic sclerosis. We studied plasma concentrations of von Willebrand factor (vWf), a substance released from injured endothelial cells, and its relation to pulmonary and renal dysfunction. METHODS: vWf was determined by immunoelectrophoresis. Renal function was assessed by CrEDTA clearance, an accurate measure of glomerular filtration rate and pulmonary function by spirometry and carbon monoxide diffusing capacity. Pulmonary pressure was measured by Doppler cardiography. RESULTS: In 22 patients with scleroderma, vWf concentrations related inversely to CrEDTA clearance. In contrast, no relationship between plasma vWf and pulmonary function was found; 9/10 patients with pulmonary hypertension and 9/12 patients with normal pulmonary pressure showed elevated levels of vWf. However in 5 patients with pulmonary hypertension without radiographic evidence of pulmonary fibrosis there was a strong correlation between plasma vWf and pulmonary pressure (rho = 0.90) which fell short of statistical significance due most likely to small population size. CONCLUSION: Renal but not pulmonary dysfunction was associated with elevated plasma levels of vWf.

Adult↗
PubMed1984

[Systemic scleroderma].

The etiology of systemic scleroderma is still unknown. This disease is well individualized by its usual forms, i.e. acrosclerosis and diffuse form, whose features and prognosis differ. The description of new entities has generated further interest, particularly through a different diagnostic and therapeutic approach. No specific biologic or immunologic criteria of the disease have as yet been discovered. Prognosis depends mainly upon the severity of visceral involvement. There have been little improvements in therapy which rests upon presumptive physiopathological hypotheses.

Bone Diseases↗
PubMed1997

[A case report of typical scleroderma accompanied with serum abnormalities characteristic of SLE during the course].

A 40-year-old woman had complained of cyanosis induced by cold exposure from the age of 26. When she was 32 years old, Raynaud's phenomenon occurred. She developed diffuse cutaneous sclerosis affecting the upper limbs, face and trunk, digital pitting scar, flexion contractures of hands, dilatation of lower esophagus and pulmonary fibrosis, and she was diagnosed as scleroderma. Laboratory findings revealed positive anti-topoisomerase I antibody and hypergammaglobulinemia (IgG 2,782, IgA 632, IgM 146 mg/dl). However, serum complement levels were normal and anti-DNA antibodies measured by radioimmunoassay (RIA) were negative. Initial dose of oral prednisolone was 30 mg/day and afterwards 5 mg/day of prednisolone was maintained. At the age of 36, scleroderma and contraction of hands were progressed, and telangiectasias appeared on her chest at the age of 36. Laboratory tests revealed hypocomplementemia (C3 27, C4 9 mg/dl, CH50 16 U/ml) and high titers, more than 100 U/ml, of anti-DNA antibodies measured by RIA. Clinical evidence suggestive of SLE could not be found. Reexamination of previous sera by enzyme immunoassay, in which anti-DNA antibody could not be detected by RIA, clarified the presence of IgG anti-dsDNA antibodies. It was considered that there existed low avidity/affinity of anti-dsDNA antibodies at first, and afterwards high avidity/affinity of anti-dsDNA antibodies appeared. Increasing of oral prednisolone up to 30 mg/day normalized serum complements and decreased titers of anti-DNA antibodies. She had not developed any clinical evidence that suspected SLE throughout the course.

Adult↗
PubMed1977

The systemic involvement in scleroderma.

A survey was made of the systemic involvement in 38 patients with scleroderma: 33 with the acrosclerotic form (Type 1, 18, Type 2, 15) and five with the diffuse form. The study comprised inquiry about symptoms, physical examination, and the laboratory tests, such as radiological examination of chest and hands, barium swallow and meal X-ray examination, electrocardiography, pulmonary function tests, haematology tests, examination for autoantibodies, and a battery of biochemical tests. Evidence of some systemic involvement (that is, in addition to skin) was almost universal. Similar disturbances occurred both in the acrosclerotic and in diffuse forms. The most common clinical involvement was that of the joints and gastrointestinal tract. The most common confirmatory signs were a positive "neck test" (tethering of the skin of the root of the neck and upper part of the chest on extending the head) and telangiectasia. The most common abnormalities in test results were those found in X-ray films of the hands (about 80%), and in pulmonary function, and barium swallow and meal X-ray studies (each about 70%). The most frequent abnormalities in the biochemical scan were increased levels of immunoglobulin M (IgM), and decreased creatine clearance.

Adult↗
PubMed1991

Long-term complex treatment of children with diffuse diseases of connective tissue.

Long-term stage-by stage care of 580 patients with systemic lupus erythematosus, dermatomyositis, scleroderma, including individual regimes of medicamentous therapy, hemosorption, water- and -mud baths, decrease the hospital letality, number of relapses and disability more than 3 times, and envisage the maintenance of stable long-year remission.

Adolescent↗
PubMed1992

The many faces of scleroderma.

This review integrates the clinical aspects of systemic sclerosis (SSc; scleroderma) and scleroderma-like conditions with new knowledge of the control of blood vessel tone and the role of anoxia in the activation of connective tissues leading to fibrosis. Serologic tests, high resolution computed tomographic scanning, bronchoalveolar lavage, and physiologic assessment of pulmonary gas diffusion are compared as diagnostic tools and as means of quantitating internal organ involvement. Treatment of Raynaud's disease and phenomenon, management of scleroderma renal crisis, and new means for improving gastrointestinal function with octreotide, the somatostatin analogue, also are discussed. The relationship between idiopathic forms of SSc and eosinophilic fasciitis/eosinophilia-myalgia syndrome caused by L-tryptophan ingestion and the scleroderma-like disease associated with silicone breast implants also is discussed.

Adult↗
PubMed1993

Diffuse interstitial lung disease--evaluation with high-resolution computed tomography.

We evaluated patterns of normal and abnormal lung parenchyma on standard 8-mm computed tomography (CT) scans, and 1-mm or 2-mm high-resolution CT (HRCT) scans in 22 subjects. There were five control subjects with no lung symptoms, and 17 patients with proved diagnosis of diffuse interstitial diseases consisting of sarcoidosis (n = 7), bronchioloalveolar carcinoma (n = 3), extrinsic allergic alveolitis (n = 2), asbestosis (n = 2), scleroderma (n = 2), and drug toxicity (n = 1). CT and HRCT scans were evaluated for specific parenchymal features particularly the distribution in secondary pulmonary lobule; conditions of pleura, mediastinum, and thoracic wall were appreciated. CT and HRCT findings were described in individual disease. We believe that CT and HRCT are useful investigations in patients with suspected or known diffuse interstitial lung diseases. At present time CT, and particularly HRCT seem to be the best available method to image the lung parenchyma.

Adult↗
PubMed1987

Frequency, levels, and significance of blood eosinophilia in systemic sclerosis, localized scleroderma, and eosinophilic fasciitis.

Blood eosinophilia is a common feature of eosinophilic fasciitis and is variably reported in systemic sclerosis and localized scleroderma. Since these diseases share cutaneous fibrosis as the final outcome and have other clinical and pathologic features that are difficult to differentiate, the presence of blood eosinophilia may be a further source of confusion. In this study, we examined the frequency and level of blood eosinophilia in 715 patients with systemic sclerosis, 72 patients with localized scleroderma, and 22 patients with clinically active eosinophilic fasciitis. When defined as greater than 400 cells/mm3, eosinophilia was present in 7% of patients with systemic sclerosis, 31% of patients with localized scleroderma, and 83% of patients with eosinophilic fasciitis. Greater than 1000 eosinophils/mm3 were present less frequently in systemic sclerosis (1%) and localized scleroderma (8%) than in eosinophilic fasciitis (61%). No difference in the frequency of eosinophilia was present in patients with the limited cutaneous CREST syndrome or the diffuse cutaneous variety of systemic sclerosis, and in these patients the presence of eosinophilia did not correlate with the extent of cutaneous or internal organ involvement or with other laboratory abnormalities. Among patients with localized scleroderma, eosinophilia was more common in those with linear scleroderma and generalized morphea than in those with morphea, and both the frequency and level of eosinophilia were greater in individuals with clinically active disease (p less than 0.02). Eosinophilia was a persistent feature in untreated patients with active eosinophilic fasciitis, even up to 30 months of disease duration.

Eosinophilia↗