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Results for “Scleroderma, Diffuse”

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PubMed1991

Variability in the retraction of collagen lattices by scleroderma fibroblasts--relationship to protein synthesis and clinical data.

Skin fibroblasts from 18 scleroderma patients were seeded into collagen lattices and their ability to retract their substratum was compared with that of control fibroblasts from healthy donors. When considered as a whole, scleroderma fibroblasts retracted lattices earlier and more intensely than controls. Analysis of individual results demonstrated that morphea and diffuse systemic sclerosis (dSSc) fibroblasts had different kinetics of lattice retraction. Fibroblasts which contracted lattices more intensely than controls were found to produce increased levels of fibronectin. A comparison of the retraction of collagen lattices by fibroblasts from involved (IS) and uninvolved skin (US) of the same patients (n = 4) showed that those from IS retracted the lattices more than fibroblasts from normal donors, whereas a high variability was found with fibroblasts from US. The increased retraction of collagen lattices seems to be a feature of the more severe forms of scleroderma.

Adult↗
PubMed1995

[Myocardial scintigraphy with thallium-201 in the diagnosis of cardiac involvement in systemic scleroderma].

201Tl scintigraphy of the myocardium is a highly sensitive method of diagnosis of cardiac damage in SS. More severe lesions occur in patients with diffuse skin affections and subacute SS. Improvement of perfusion in dipiridamol test indicates the contribution of the spasm of microcirculation coronary vessels to the onset of perfusion defects. Further investigations are needed for evaluation of the role various vasodilators have in prevention of perfusion lesions and progressive myocardial fibrosis in SS patients.

Adult↗
PubMed1986

[Video-microscopic studies of telangiectases in Osler's disease and scleroderma].

4 patients with hereditary hemorrhagic telangiectasia (Osler's disease) have been studied by clinical means and by fluorescence videomicroscopy. The capillaroscopic image was characterized by convolutes of giant capillaries (diameter up to 150 micron, normal value up to 15 micron) between capillaries of normal shape and size. After intravenous injection of Na-fluorescein the enlarged capillaries filled rapidly and in some loops flow direction changed at irregular intervals. These findings are in contrast to those observed in systemic sclerosis, where generalized microangiopathy with avascular fields, microbleedings, enlarged capillaries and increased transcapillary and interstitial diffusion of the fluorescent dye are typical. In borderline cases capillaroscopy contributes to differential diagnosis of the telangiectatic form of scleroderma and Osler's disease.

Capillaries↗
PubMed2003

Scleroderma patients with combined pulmonary hypertension and interstitial lung disease.

OBJECTIVE: Most studies differentiate scleroderma associated pulmonary hypertension and interstitial lung disease (ILD) as 2 separate pathological processes, concentrating on one or the other; however, many patients have both conditions. We studied the demographics, clinical features, and prognosis of individuals with both vascular and interstitial lung disease. METHODS: A retrospective cross-sectional study of 619 patients with scleroderma who had echocardiograph and pulmonary function testing performed within 6 months of one another. Echocardiography determined the presence of pulmonary hypertension, and pulmonary function testing documented restrictive ventilatory defect (RVD) as a marker of ILD. RESULTS: Among the study group, 139 (22.5%) patients had isolated RVD; 119 (19.2%) isolated pulmonary hypertension; and 112 (18.1%) patients had combined RVD and pulmonary hypertension. The individuals with combined RVD and pulmonary hypertension resembled patients with isolated RVD in that they had a high prevalence of diffuse skin involvement and antitopoisomerase positivity, but they were older at diagnosis and at disease onset (p < 0.01). Among those with mild RVD, 39.2% had pulmonary hypertension compared to those with severe RVD, in whom 51.4% had pulmonary hypertension. Compared to those without pulmonary disease, the mortality risk ratio for patients with isolated pulmonary hypertension, combined RVD and pulmonary hypertension, and isolated RVD was 2.9, 2.4, and 1.61, respectively. CONCLUSION: Patients with combined scleroderma lung disease features are more likely to have diffuse disease, represent older patients, and have a prognosis similar to individuals with isolated pulmonary hypertension, and may represent a distinct subpopulation of scleroderma.

Adult↗
PubMed1980

Improvement of lung function in a scleroderma patient treated for 12 months with cyclofenil.

A 48 year old male scleroderma patient with typical impairment of respiratory function, including low vital capacity, low static lung compliance and low diffusing capacity for carbon monoxide, was treated with cyclofenil for one year. Assessment of lung function after 3, 6 and 12 months treatment showed marked improvement of vital capacity, increased physical working capacity, less hypoxia at maximum work, increase of static lung compliance and decreases of maximal transpulmonary pressure. Closing capacity decreased, and an increase in difussing capacity for carbon monoxide was seen.

Cresols↗
PubMed1984

[Vital microscopy of nailfold capillaries in progressive scleroderma in relation to the clinical picture].

Capillary microscopic investigations of the nailfold were performed in 38 patients presenting Raynaud's phenomenon (systemic sclerosis: diffuse type n = 6; systemic sclerosis: CREST syndrome n = 21; Raynaud's disease n = 7; mixed connective tissue disease (MCTD) n = 4). A control group of 15 healthy persons was investigated for comparison. 3 stages of capillary alteration were established in patients with systemic sclerosis by recording number (per 2 mm microscopic field diameter), morphology, colour and size of nailfold capillaries. Additional criteria like pericapillary microhemorrhages and disordered capillary blood flow were considered as well. Significant correlations of capillary microscopie findings to different subsets of systemic sclerosis including MCTD could not be proven. Cases provisionally classified as Raynaud's disease could not be assigned to systemic sclerosis on account of capillary microscopy. A clear correlation was found, however, between the grade of capillary alteration and both severity of clinical features and duration of the disease. All cases with capillary changes of stage 3 showed clinical correlation to esophageal dysmotility , also proven in 7 out of 13 patients with stage 2 capillary alteration.

Adult↗
PubMed2005

Isolated pulmonary hypertension in scleroderma.

BACKGROUND: Isolated pulmonary hypertension (PHT) is now the most frequent cause of disease-related death in limited cutaneous scleroderma, the commonest disease variant of this disabling connective tissue disorder. Endothelin-1 receptor antagonists provide symptomatic benefit but to date have not been shown to prolong survival. AIM: To determine the frequency, disease characteristics and survival of symptomatic patients with isolated PHT in our cohort of scleroderma patients. METHODS: Systematic review of the clinical course of all patients registered on the South Australian Scleroderma Register, a population-based register of 374 living and 234 deceased patients with scleroderma. RESULTS: Thirty-four patients were identified with isolated PHT, the majority with limited scleroderma. From our deceased register, we estimate that >11% of patients with this limited variant will develop this complication. Isolated PHT occurs as a late-stage complication approximately 20 years after the first symptoms of scleroderma. Patients with isolated PHT were characterized by the presence of multiple telangiectasia, reduced nailfold capillary density, digital ulceration, gross reduction of diffusing capacity for carbon monoxide and echocardiographic evidence of elevated pulmonary artery pressure. Survival was significantly shortened as compared with those patients without this complication (P=0.002), with a mean survival of 2.5 years from symptomatic onset of PHT. CONCLUSION: Isolated PHT occurs as a late-stage complication in > or =11% of patients with limited cutaneous scleroderma and leads to rapid death from right heart failure. The early use of endothelin-1 receptor antagonists may change the natural history of this fatal complication.

Aged↗
PubMed1976

Single-breath pulmonary diffusing capacity. Reference values and application in connective tissue diseases and in various lung diseases.

1.1. Diffusing capacity of the lungs was measured by using a Mark 4 Resparameter. The single-breath diffusing capacity (D) for carbon monoxide was found to be dependent on the lung volume (VA) during the breath-hold. The same applied to D/VA and to the time constant (tau) for carbon monoxide uptake. This confirms the theoretical considerations and the results of other investigators. This effect should be taken into account especially when making serial determinations of D on the same subject. 1.2. The repeatability of the method was found to be reasonably good; the coefficient of variation was about 4% in normal subjects, as well as in a series of patients with rheumatoid arthritis. 1.3. The effect of the correction for haemoglobin concentration was calculated and tabulated for a set of possible values of DM/Vc and haemoglobin. This correction did not reduce the scatter of D values in normal subjects, but it was adopted for clinical use for theoretical reasons. Caution in its use and interpretation of the correction is emphasized. 2.1. Healthy males and females, 20-69 years of age, were examined to establish reference values for the D measurement. Multiple linear regression equations were calculated stepwise, and equations based on age and height were then used in the clinical prediction equations. Similarly, prediction equations were calculated for clinical spirometry. 2.2. The prediction equations were compared with some published reference values. The equations provide a basis for the evaluation of the effect of various factors, such as age and smoking, in different populations. 3.1. Respiratory function, clinical symptoms and chest x-ray findings were examined in patients with connective tissue diseases. Three different investigations were thus formed: a) consecutive patients (free of lung disease other than that possibly due to a connective tissue disease) with definite rheumatoid arthritis (RA, 21 cases), systemic lupus erythematosus (SLE, 18 cases) or scleroderma (SCL, 6 cases) were subjected to detailed tests. b) 129 patients from the Rheumatism Foundation Hospital, Heinola, Finland, were subjected to measurement of diffusing capacity and vital capacity in addition to chest radiography and routine clinical assessment c) the histological findings on 12 patients subjected to a needle biopsy of the lung in order to exclude other conditions were compared with the results of diffusing capacity and x-ray examinations. 3.2. Restrictive impairment of the respiratory function was the general finding in all of these groups. The reduction in diffusing capacity was out of proportion to the reduction in lung volume, however, in most cases with abnormally low values. Low D values were encountered in about half of the RA and SLE and in all of the SCL patients in group a, and in 13% of the "average" RA patients studied in Heinola. The typical histological finding in patients with reduced D was a thickening of the alveolar wall...

Adult↗
PubMed1994

Autoantibodies to HMG-17 nucleosomal protein in patients with scleroderma.

Autoantibodies to HMG-17, a non-histone nucleosomal protein, were found in systemic lupus erythematosus (SLE), mixed connective tissue disease (MCTD) and ANA positive pauciarticular juvenile rheumatoid arthritis (JRA), but not in rheumatoid arthritis (RA). Using highly purified HMG-17 derived from porcine thymus, we tested sera from 50 patients with scleroderma for antibodies to HMG-17 by enzyme-linked immunosorbent assay (ELISA). There were 16 patients with diffuse cutaneous systemic sclerosis (dSSc) and 34 with limited cutaneous systemic sclerosis (1SSc) with age at disease onset 40.25 +/- 11.68 and 39.94 +/- 15.68 years, respectively, and disease duration 6.03 +/- 4.98 and 13.34 +/- 11.80 years, respectively (P < 0.0001). Anticentromere antibodies (ACA) were found in 65% of 1SSc patients but not in dSSc (P < 0.0001) while the prevalence of antinuclear antibodies (ANA) with other than ACA patterns did not differ in the two groups. Anti-HMG-17 antibodies occurred in 20 patients (40%), five with dSSc (31%) and 15 with 1SSc (41%). Twelve of the 20 HMG-17 positive patients were also positive for ACA (60%) but this association was not significant. No association was found between anti-HMG-17 and other antibody patterns. In conclusion, anti-HMG-17 antibodies occur in one third of scleroderma patients, do not discriminate scleroderma variants and are not associated with other autoantibodies.

Adult↗
PubMed2006

Does cyclically iloprost infusion prevent severe isolated pulmonary hypertension in systemic sclerosis? Preliminary results.

The objective of this study was to present our clinical experience about scleroderma-related pulmonary hypertension in the patients treated with intermittent iloprost infusions. Eighty-one patients affected by systemic sclerosis (12 men, 69 women; 30 with diffuse pattern and 51 with limited pattern; mean age 55.1 years; mean duration of disease 105.3 months) have been treated with cyclic iloprost infusions for at least 15 months (range 15-126 months). During the last 4 months all patients underwent Doppler echocardiography in order to estimate the value of systolic pulmonary artery pressure. In 14 subjects (17.2%) systolic pulmonary artery pressure was = or > 35 mmHg. Four patients presented high systolic pulmonary artery pressure associated with pulmonary fibrosis (mean value 40.5 +/- 4.5 mmHg). Ten women (one with diffuse pattern of disease and nine with limited form) showed isolated high systolic pulmonary artery pressure; one of these patients underwent right heart catheterization which resulted normal. The remaining nine patients (mean age of 67.1 years; age at the onset of scleroderma 52.2 years) showed estimated systolic pulmonary artery pressure values between 35 and 50 mmHg. Among these patients affected by isolated pulmonary hypertension only one has been receiving bosentan in association with iloprost infusions. None of our scleroderma patients treated with cyclic iloprost infusions developed severe isolated pulmonary hypertension. In systemic sclerosis the multiple effects of iloprost on endothelium, platelets and cytokine network may counteract the vasospastic profile of lung microvasculature in pulmonary arterial hypertension and the consequent vascular wall remodelling, thus preventing the development of severe illness.

Adult↗
PubMed2006

Improvement of lung function in patients with systemic sclerosis after 6 months cyclophosphamide pulse therapy.

This study aims to analyze the effects of cyclophosphamide pulse therapy on parameters of lung function in patients with systemic sclerosis. Nineteen patients with systemic sclerosis (15 women and four men, aged 25-67 years, mean disease duration 5 years and 9 months) were included in this study. The main reason for the beginning of cyclophosphamide therapy was the decrease of transfer-factor (DLCO) or diffusing coefficient for carbon monoxide (DLCO/VA) under 70% of predictive value. Intravenous cyclophosphamide was administered monthly in a dose of 500 mg/m(2 )body surface. The efficacy was evaluated by comparison of forced vital capacity (FVC), DLCO, and DLCO/VA at the baseline and 1 month after the sixth pulse. Statistical analyses were performed using Student's T test and Wilcoxon's test. The difference between FVC at the baseline (86.6%) and at the end of the follow-up period (89.2%) was not statistically significant (t=-1.25, p>0.05). However, a significant increase of DLCO (from 61.2% to 70.5%, z=-2.04, p=0.04) and DLCO/VA (from 57.8% to 72.5%, z=-2.67, p=0.008) was observed. Minor side effects were noticed in some patients. Two patients had nausea after cyclophosphamide infusion, two patients had insignificant decrease of creatinine clearance, and two patients had temporary and mild leukopenia. In patients with systemic sclerosis and lung involvement, an improvement of lung-diffusing capacity was noticed 6 months after the beginning of cyclophosphamide pulse therapy, with only minor side effects.

Adult↗
PubMedDate not supplied

Abnormal exhaled ethane concentrations in scleroderma.

Scleroderma (systemic sclerosis) is a chronic multisystem autoimmune disease in which oxidative stress is suspected to play a role in the pathophysiology. Therefore, it was postulated that patients with scleroderma would have abnormally high breath ethane concentrations, which is a volatile product of free-radical-mediated lipid peroxidation, compared with a group of controls. There was a significant difference (p<0.05) between the mean exhaled ethane concentration of 5.27 pmol ml(-1) CO(2) (SEM=0.76) in the scleroderma patients (n=36) versus the mean exhaled concentration of 2.72 pmol ml(-1) CO(2) (SEM=0.71) in a group of healthy controls (n=21). Within the scleroderma group, those subjects taking a calcium channel blocker had lower ethane concentrations compared with patients who were not taking these drugs (p=0.05). There was a significant inverse association between lung diffusion capacity for carbon monoxide (per cent of predicted) and ethane concentration (b=-2.8, p=0.026, CI=-5.2 to -0.35). These data support the presence of increased oxidative stress among patients with scleroderma that is detected by measuring breath ethane concentrations.

Breath Tests↗