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PubMed1994

[Systemic sclerosis (scleroderma): etiology and pathogenesis--where are we today?].

Systemic sclerosis is a rare chronic disease with inflammatory and vascular features leading to increased fibrosis of skin and internal organs. The pathogenesis of systemic sclerosis and scleroderma like diseases appears to be influenced rather by environmental than by genetic factors. Clinical and pathogenetic aspects of systemic sclerosis show similarities to untreated chronic graft-versus-host-disease after bone marrow transplantation. New insights into the pathogenesis of systemic sclerosis give reason to hope that better therapeutic approaches will be developed in the future.

Adult↗
PubMed2004

[The association of systemic sclerosis with malignant neoplasms].

The rate of concomitance of systemic sclerosis and malignant neoplasm is not still ascertained. Therefore we reviewed 8,327 patients who were diagnosed with systemic sclerosis and received public financial aid from the Ministry of Health, Labour and Welfare of Japan in 1999. The concomitance rate was 3.1% of all patients, however, it was more frequent among men than among women (5.4% for men and 2.8% for women, respectively, P<0.01), and the mean age of patients with complicated malignant neoplasms was significantly higher than that of patients without malignancy (P<0.01). For comparison with the Japanese general population, O/E ratios (ratio of observed-to-expected malignant neoplasms) were calculated, and it was found that both men and women had significantly higher O/E ratios (O/E ratio 2.31, 95% confidence interval [CI] 1.68-2.94, P<0.001 for men and O/E ratio 1.64, 95% CI 1.41-1.86, P<0.001 for women). In addition we also assessed laboratory findings including autoantibodies and respiratory function tests for patients with malignant neoplasms by logistic regression analysis adjusted for sex and age. Decreased DLco (pulmonary CO diffusing capacity) appeared to be a risk factor for the concomitant malignant neoplasms among patients with systemic sclerosis (odds ratio 2.00 for DLco < or = 70%, CI 1.06-3.74, P=0.032). These results may help to elucidate the etiology of systemic sclerosis.

Adolescent↗
PubMed1986

The toxicity of D-penicillamine in systemic sclerosis.

We studied D-penicillamine toxicity in 259 patients with systemic sclerosis treated since 1972. The average daily dose of 635 mg was given for a mean of 1.8 years. Of patients with systemic sclerosis, 47% has side effects from D-penicillamine treatment, similar to the 56% of 807 patients with rheumatoid arthritis in seven separate series. Individual manifestations of toxicity included rash, proteinuria, gastrointestinal symptoms, dysgeusia, oral ulcers, thrombocytopenia, and neutropenia. Four episodes each of myasthenia gravis and pemphigus occurred in our patients; both were reported rarely in patients with rheumatoid arthritis. Adverse effects occurred more frequently after rapid increases in dosage. Treatment had to be discontinued due to toxicity in 29% of patients with systemic sclerosis and in 33% of those with rheumatoid arthritis. Although toxic, with a high frequency of adverse effects, D-penicillamine can be used safely in the treatment of systemic sclerosis. Pemphigus and myasthenia gravis may occur more frequently with therapy for systemic sclerosis than with that for rheumatoid arthritis.

Arthritis, Rheumatoid↗
PubMed1992

Lung involvement in systemic sclerosis sine scleroderma treated by plasma exchange.

Systemic sclerosis sine scleroderma can present in some patients as pulmonary interstitial fibrosis. Until now ten cases with this particular clinical variant, all men, have been reported in the literature. The knowledge of systemic sclerosis sine scleroderma presenting as lung interstitial involvement is important in clinical practice for an early diagnosis and correct therapeutic strategy. This work reports the clinico-serological features of two further cases, one a woman, of systemic sclerosis sine scleroderma with prevalent lung involvement, and describes the effects of therapeutic plasma exchange.

Female↗
PubMed2003

The prevalence and clinical significance of anti-U1 RNA antibodies in patients with systemic sclerosis.

We studied the prevalence and clinical significance of anti-U1 RNA antibodies in patients with systemic sclerosis. The presence of anti-U1 RNA antibodies was determined using immunoprecipitation in systemic sclerosis patients with anti-U1 RNP antibodies (n=36), antitopoisomerase I antibodies (n=20), or anticentromere antibodies (n=20), mixed connective tissue disease patients (n=23), systemic lupus erythematosus patients with anti-U1 RNP antibodies (n=26), and normal controls (n=20). Moreover, antigen specificities for anti-U1 RNP antibodies were examined in patients with systemic sclerosis by immunoblotting and enzyme-linked immunosorbent assay. Anti-U1 RNA antibodies was detected in 22 of 36 systemic sclerosis patients (61%) with anti-U1 RNP antibodies, 14 of 23 patients (61%) with mixed connective tissue disease, and eight of 26 systemic lupus erythematosus patients (31%) with anti-U1 RNP antibodies. Anti-U1 RNA antibodies were not detected in other groups. As for systemic sclerosis patients, the frequencies of pulmonary fibrosis and reduced percentage diffusion capacity for carbon monoxide were significantly greater in patients with anti-U1 RNA antibodies than in those without (76%vs 18%, p<0.005; 82%vs 27%, p<0.005, respectively). Moreover, patients with anti-U1 RNA antibodies had significantly lower percentage diffusion capacity for carbon monoxide and percentage vital capacity values than those without (51.9+/-16.8 vs 79.4+/-16.4, p<0.01; 83.8+/-21.4 vs 101.4+/-12.9, p<0.05, respectively). Regarding the antigen specificities of anti-U1 RNP antibodies in systemic sclerosis patients, the frequency of anti-70 kDa antibodies determined by immunoblotting was significantly higher in patients with anti-U1 RNA antibodies than in those without (77%vs 43%, p<0.05). This finding was also confirmed by enzyme-linked immunosorbent assay for anti-70 kDa antibodies (86%vs 43%, p<0.05). These results indicate that anti-U1 RNA antibodies may be a serologic indicator for pulmonary fibrosis in systemic sclerosis patients with anti-U1 RNP antibodies.

Autoantibodies↗
PubMed1977

Penicillamine therapy in systemic sclerosis.

Twenty-two patients with progressive systemic sclerosis were treated with D-penicillamine in doses ranging up to 1250 mg/day for periods varying between a few months and four years. Side-effects occurred in 7 patients, necessitating drug withdrawal in 2. Cutaneous benefit occurred in 15 patients, but owing to side-effects from the drug, relapses, and development, persistence or advancement of visceral complications, an overall good result only occurred in 5. Seven patients showed improvements in joint function, but only 3 were regarded as having an overall good result. Peripheral vascular disease and visceral involvement seemed not to be influenced by D-penicillamine and sometimes appeared or advanced during treatment. Six patients died from visceral manifestations of systemic sclerosis and one from another cause. D-penicillamine is of limited value for the cutaneous features of progressive systemic sclerosis, but probably of no value for the vascular and visceral manifestations of the disease.

Adult↗
PubMedDate not supplied

The anti-platelet effect of nifedipine in patients with systemic sclerosis.

The enhanced platelet reactivity and impaired thrombolysis in patients with systemic sclerosis may contribute to the microvascular insufficiency seen in this disease. Anti-platelet therapy has therefore been suggested for the treatment of Raynaud's phenomenon secondary to systemic sclerosis. Using a novel technique, the Haemostatometer, haemostasis (shear-induced) and thrombolysis (dislodgement of the haemostatic plug) were assessed initially in 15 patients with systemic sclerosis before and 90 minutes after a single oral dose of nifedipine (10 mg), and then at 4-week intervals for 16 weeks in 10 patients on long-term nifedipine (10-20 mg tid). Ninety minutes after a single oral dose of nifedipine in 15 patients with systemic sclerosis, haemostasis was significantly prolonged from 140 +/- 12 sec to 178 +/- 21 sec (mean +/- SE, p less than 0.005). The time until spontaneous thrombolysis occurred was significantly shortened following nifedipine, from an abnormal time of 57.8 +/- 2.8 min to a more normal value of 34.0 +/- 5.1 min (mean +/- SE, p less than 0.01). This improvement in haemostasis and thrombolysis was maintained through 16 weeks on longterm nifedipine treatment. These findings suggest that nifedipine may reduce the risk of developing multiple thrombo-emboli in patients with systemic sclerosis.

Administration, Oral↗
PubMed1992

Left ventricular diastolic function in systemic sclerosis: assessment by radionuclide angiography.

The aim of this study was to assess left ventricular function in subjects with systemic sclerosis. Twenty-four women with systemic sclerosis (mean age 48 +/- 11 yr) and 14 age- and sex-matched normal subjects were studied by radionuclide angiography performed at rest with a temporal resolution of 20 msec/frame. Left ventricular volume curves were generated and indices of systolic and diastolic function were computed. Left ventricular diastolic asynchrony was evaluated by dividing the left ventricle into five regions and then computing the time-to-peak filling rate for each region. After excluding the valvular region, the coefficient of variation of this index was obtained. The isovolumic relaxation period was prolonged in systemic sclerosis patients in comparison to normal subjects (127 +/- 39 msec versus 87 +/- 44 msec, p less than 0.05). Moreover, 38% of the systemic sclerosis patients had a subnormal peak filling rate. Left ventricular diastolic asynchrony was increased in the systemic sclerosis group, as expressed by a higher coefficient of variation of the regional time to peak filling rate (27.9% +/- 11.5% versus 14.5% +/- 8.6%, p less than 0.05). Our results indicate an impaired relaxation and an increased diastolic asynchrony in patients with systemic sclerosis.

Female↗
PubMedDate not supplied

Serotonin induced platelet aggregation in systemic sclerosis.

We studied in vitro platelet aggregation response to serotonin in 19 patients with systemic sclerosis and 18 matched controls. Individuals with early systemic sclerosis had increased % platelet aggregation at 3 minutes over a wide range of serotonin concentrations when compared to individuals with late systemic sclerosis. Patients with generalized disease did not differ from those with limited disease. No significant differences in initial slope of platelet aggregation were detected in any patient subgroup. These data support previous studies suggesting platelet activation in systemic sclerosis and further suggest that future studies of platelet abnormalities in systemic sclerosis should consider the effects of disease duration.

Adult↗
PubMed1988

Prognostic importance of cardiac arrhythmias in systemic sclerosis.

Ambulatory electrocardiography was performed in 183 patients with systemic sclerosis recruited from five centers who were selected to reflect a balanced population with respect to disease extent and duration. Ventricular ectopy occurred in 67 percent of patients and was strongly correlated by both univariate and multivariate analyses with total mortality and with sudden death. By multivariate analysis, ventricular ectopy was strongly associated with increasing patient age and with other evidence of cardiac and pulmonary involvement but not with clinical and laboratory measures of duration and extent of systemic sclerosis. Evidence of myocardial fibrosis thought to be secondary to microvascular alteration is common in systemic sclerosis, but the clinical implications of myocardial involvement are less well appreciated. The present data suggest the need for ambulatory electrocardiography in the clinical assessment of selected patients with systemic sclerosis, especially those with cardiac or pulmonary involvement, as well as for studies of the effects of antiarrhythmic therapy.

Adolescent↗
PubMed1997

Pulmonary function in patients with systemic sclerosis.

The lungs are frequently affected in systemic sclerosis (SSc), a generalized connective tissue disorder. We evaluated the prevalence of respiratory functional abnormalities and their correlation with symptoms and radiograph features in a group of 34 patients who fulfilled the American Rheumatism Association criteria for the diagnosis of systemic sclerosis. Patients were submitted to a specific respiratory questionnaire and to lung function tests. Measurements were performed according to the European Coal and Steel Community (ECSC) recommendations and results expressed as a SD score, an accurate method that, taking into account the dispersion of the parameters in the reference population, allows precise definition of pathological subjects. Of the patients examined, 38% reported dyspnoea at rest or on exertion. No other respiratory symptoms were reported. Fifty percent had a normal chest radiograph. This study documents the high prevalence of respiratory functional abnormalities in patients with SSc. A restrictive pattern was found in 41% and an isolated diffusion impairment in 18%. No significant relationship was found between the isolated impairment of transfer factor of the lungs for carbon monoxide (TL,CO) and the mean duration of the scleroderma: thus, it does not seem to represent an early sign of severe restrictive disease. No bronchial or bronchiolar obstructive patterns were observed: it can be stated that small airways dysfunction is not a characteristic manifestation of SSc as considered previously. A significant association was found between the group of subjects with chest radiographic abnormalities and that with a restrictive pattern or isolated TL,CO alteration (p = 0.018). Chest radiographic abnormalities were also found in 29% and dyspnoea in 35% of the patients with normal respiratory function. The mean duration of scleroderma was not significantly different between the groups with and without abnormalities on chest radiography, between the groups with and without a restrictive pattern or isolated diffusion impairment, and between the groups of patients with and without dyspnoea. In conclusion, an accurate evaluation of respiratory function is recommended in the assessment of patients with systemic sclerosis, since the functional involvement of the lung cannot be predicted on the basis of the chest radiograph and the respiratory symptoms.

Dyspnea↗
PubMed1997

Pericardial involvement in systemic sclerosis.

OBJECTIVE: To determine the frequency and histological characteristics of pericardial involvement in systemic sclerosis. METHOD: Necropsy sections of pericardium from 44 patients with systemic sclerosis were studied, together with sections from 19 age/sex matched controls. Sections were stained with haematoxylin and eosin, acid toluidine blue, and elastic van Gieson. Mast cells were counted in 10 random high power fields and the degree of fibrosis was quantified using a Chalkley count. RESULTS: Chronic pericarditis was seen in 31 (77.5%) of the systemic sclerosis cases, but in only one of the controls. The characteristic changes of uraemic pericarditis were not seen. The degree of fibrosis was greater in those with systemic sclerosis, though numbers of mast cells, thought to be important in fibrogenesis, were similar in both groups. Myocardial fibrosis was seen in 15 (37.5%) of systemic sclerosis cases but in none of the controls. CONCLUSION: The incidence of pericarditis and myocardial fibrosis is much greater than in controls. The results indicate that pericarditis is a primary disease (rather than secondary to uraemia).

Adolescent↗
PubMed2004

Acute and sustained effects of dihydropyridine-type calcium channel antagonists on oxidative stress in systemic sclerosis.

PURPOSE: To evaluate the potential antioxidant properties of dihydropyridine calcium channel antagonists in systemic sclerosis. METHODS: Forty-two patients with systemic sclerosis were included (mean [+/- SD] age, 54 +/- 12 years; mean disease duration, 8 +/- 7 years). Plasma markers of oxidative stress (carbonyl residues, advanced oxidation protein products, malondialdehyde, nitrosothiols, and total thiol groups) were determined 72 hours after the discontinuation of usual dihydropyridine treatment (with either nifedipine or nicardipine), shortly after reinitiation of treatment, and 9 to 12 months later (long-term treatment) in 19 of the patients. Baseline values were compared with those in 23 healthy volunteers. RESULTS: Mean levels of plasma markers of oxidative stress were much higher in patients with systemic sclerosis than in controls (carbonyls, 0.4 +/- 0.1 nmol/mg protein vs. 0.3 +/- 0.1 nmol/mg protein, P = 0.0001; advanced oxidation protein products, 111 +/- 13 micromol/L vs. 47 +/- 7 micromol/L, p = 0.003; malondialdehyde, 11.3 +/- 3.3 micromol/L vs. 5.5 +/- 1.3 micromol/L, P <0.0001; nitrosothiols, 1.6 +/- 0.2 micromol/L vs. 0.6 +/- 0.2 micromol/L, P <0.0001). In contrast, thiol levels were lower in systemic sclerosis patients (264 +/- 80 micromol/L vs. 435 +/- 50 micromol/L, P <0.0001). Short-term treatment led to a significant decrease in oxidative stress markers (carbonyls, 0.3 +/- 0.1 nmol/mg protein, P <0.0001), advanced oxidation protein products (60 +/- 3 micromol/L, P <0.0001), malondialdehyde (8.8 +/- 5.6 micromol/L, p = 0.0002), and nitrosothiols (1.4 +/- 0.2 micromol/L, p = 0.0001), but an increase in thiol levels (340 +/- 84 micromol/L, P <0.0001). These decreases persisted with long-term treatment. CONCLUSION: Dihydropyridines significantly decrease oxidative stress in systemic sclerosis patients, in both the short and long term.

Adult↗
PubMed2006

Urticarial vasculitis appearing in the progression of systemic sclerosis.

We report a case of urticarial vasculitis that appeared during the course of limited cutaneous systemic sclerosis. The urticarial lesions responded to systemic administration of prednisolone. After the appearance of urticarial vasculitis, the progression of scleroderma in distal sites of her elbow and knee joint became apparent. We consider this case to be consistent with limited cutaneous systemic sclerosis. The patient started treatment with prednisolone and her edema as well as scleroderma softened gradually. We analyzed, by immunohistochemistry, the number of tryptase-positive mast cells of this case in the lesions of urticarial vasculitis as well as systemic sclerosis. The number of tryptase-positive mast cells in the lesions of urticarial vasculitis as well as systemic sclerosis was significantly increased compared to normal skin (P < 0.05 and P < 0.005, respectively). We demonstrate that, in the present case, mast cells might be involved in both courses of urticarial vasculitis and systemic sclerosis as a common factor.

Diagnosis, Differential↗
PubMed1990

Mitral regurgitation and diastolic flow profile in systemic sclerosis.

To evaluate the left ventricular filling characteristics in systemic sclerosis, we examined 30 consecutive patients, 15 men and 15 women, and related the findings to those from 48 age- and sex-matched controls. All patients were investigated by pulsed and continuous wave mitral Doppler, and M mode echocardiography. We found the A wave of the mitral flow velocity as recorded by pulsed wave Doppler to be higher in patients (0.74 +/- 0.07 vs 0.54 +/- 0.02 m/sec, P less than 0.002), while the E wave did not differ. The high A/E ratio indicating reduced distensibility, correlated to interventricular septal thickness (r = 0.53, P less than 0.001), and atrial emptying index (r = -0.55, P less than 0.001). Early filling was impaired, with a prolonged pressure half time (99 +/- 6 vs 84 +/- 4 msec, P less than 0.05), and a reduced first third filling fraction (0.41 +/- 0.02 vs 0.48 +/- 0.01, P less than 0.001). Mitral regurgitation was found in 67% of systemic sclerosis patients and in 15% of controls (P less than 0.001). Doppler measures of left ventricular filling properties were not related to the presence of mitral regurgitation or systolic blood pressure. We conclude that left ventricular distensibility and early filling properties are impaired in systemic sclerosis and not related to blood pressure, but rather to left ventricular wall thickness and therefore probably secondary to myocardial fibrosis. Mitral regurgitation is a common finding in systemic sclerosis.

Adult↗
PubMed1993

Progressive systemic sclerosis sine scleroderma in a child presenting as nocturnal seizures and Raynaud's phenomenon.

Progressive systemic sclerosis sine scleroderma, as well as neurological manifestations of progressive systemic sclerosis are rare in adult-onset cases. Neither have been reported in children with progressive systemic sclerosis, either separately or together. We describe a six-year-old girl with nocturnal seizures and Raynaud's phenomenon of three years' duration. She died of cardiopulmonary sclerosis without ever fitting the required criteria of systemic sclerosis. Nailfold capillaroscopy revealed the specific "scleroderma-pattern" and provided the only clue for a diagnosis of progressive systemic sclerosis, confirmed eventually by skin biopsy.

Child↗
PubMed1982

Autologous and allogeneic mixed lymphocyte reactions in progressive systemic sclerosis.

The autologous and allogeneic mixed lymphocyte reactions (MLR), observed when peripheral blood mononuclear cells from 20 patients with progressive systemic sclerosis were used, were compared with those of age-, sex-, and race-matched normal controls. Such cells were separated by gradient centrifugation of sheep red blood cell (E) rosettes into stimulator (E- or non-T cell) and responder (E + or T cell) populations. The autologous MLR of both the progressive systemic sclerosis and normal peripheral blood mononuclear cells varied widely but there was no statistical difference between the means of each group. In the allogeneic MLR, proliferation between progressive systemic sclerosis non-T cells and normal T cells was significantly less than that of normal non-T cells and progressive systemic sclerosis T cells (P = 0.001). A decreased autologous MLR, while noted with other autoimmune diseases, was lacking in progressive systemic sclerosis. This suggests a different defect. The differences in the allogeneic MLR also suggest that either progressive systemic sclerosis non-T cells were poor stimulators or T cells associated with this disease were better responders when compared with similarly prepared cell populations from normal individuals. The MLR differences could have also resulted from compositional subset alterations or the sharing of a common antigen. HLA-DR5 was found in 9 of the 17 white patients with progressive systemic sclerosis. Although these individuals were evenly distributed as low, medium, and high responders, this finding showed that some progressive systemic sclerosis non-T cells shared a common antigen.

Adult↗