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PubMed2001

Meta-analysis of systemic sclerosis and exposure to solvents.

BACKGROUND: Although systemic sclerosis is a rare disease, incidence rates have increased recently in the United States. This study investigated the association between systemic sclerosis and exposure to solvents. METHODS: A Medline search of articles published between 1966 and 2000 dealing with solvent exposure and systemic sclerosis identified eight studies that met inclusion criteria of the meta-analysis. The studies included seven case-control studies and one cohort study published between 1989 and 1998. A series of meta-analyses of studies on systemic sclerosis and solvent exposure were performed for all studies and for case-control studies. A random effect model was used to calculate a combined estimator of relative risk. RESULTS: The combined estimator of relative risk for all studies was 2.91, with a 95% confidence interval (CI) of 1.60 to 5.30. The combined estimator of relative risk for the seven case-control studies was 3.14 (95% CI, 1.56-6.33). CONCLUSIONS: A statistically significant increased relative risk for all studies and for case-control studies suggests that exposure to organic solvent may be a risk factor for developing systemic sclerosis. Further studies using other study designs and better control of confounders are needed.

Case-Control Studies↗

PubMed1993

Messenger RNA expression for growth factors in glomeruli from focal glomerular sclerosis.

Focal glomerular sclerosis was induced in rats by chronic injections of puromycin aminonucleoside (PAN) on Days 0, 27, 34, and 41 and by unilateral nephrectomy on Day 22. Rats were sacrificed on Days 0, 8, and 20 (acute phase) and on Days 48, 60, and 80 (sclerotic phase). The percentage of sclerosing glomeruli was 16.6% on Day 48 and increased significantly to 72.8% on Day 80. We examined glomerular mRNA levels for proliferating cell nuclear antigen (PCNA), platelet-derived growth factor (PDGF)-A and B chains, transforming growth factor (TGF)-beta, epidermal growth factor (EGF), insulin-like growth factor (IGF)-I, and basic fibroblast growth factor (bFGF) on Days 0, 8, 20, 48, 60, and 80. Although these growth factor mRNA levels showed little change in glomeruli until Day 20, all growth factor mRNA levels increased in glomeruli during the sclerotic phase of PAN nephrosis as glomerular sclerosis progressed. On Day 80, mRNA levels for PCNA, PDGF-A and B chains, TGF-beta, EGF, IGF-I, and bFGF increased 12-, 10-, 12-, 15-, 2-, 2-, and 8-fold, respectively, in the glomeruli of PAN-treated rats with marked glomerular sclerosis when compared with control rats. Unilateral nephrectomy without PAN administration did not cause glomerular sclerosis up to Day 80 and mRNA levels for PCNA, PDGF-A and -B chains, TGF-beta, EGF, IGF-I, and bFGF in this group were almost the same as those in the normal sham-operated group. These data suggest that changes in growth factor mRNA levels in glomeruli may contribute to the development of PAN-induced glomerular sclerosis.

Animals↗

PubMed1995

Elevated plasma endothelin levels in systemic sclerosis.

Endothelin is a novel potent vasoconstrictor peptide produced mainly by endothelial cells. Thrombomodulin is a high-affinity thrombin receptor on vascular endothelial cells that plays an important role as a natural anticoagulant. In this study, we measured plasma levels of endothelin and thrombomodulin in patients with systemic sclerosis or Raynaud's disease. Plasma levels of endothelin and the ratio of thrombomodulin to creatinine were significantly increased in patients with systemic sclerosis compared with normal controls, and there was a positive correlation between these two indicators (r = 0.615, P = 0.004). Moreover, plasma levels of endothelin were significantly higher in patients with diffuse systemic sclerosis than in patients with limited systemic sclerosis. In contrast, plasma levels of endothelin in patients with Raynaud's disease were not significantly increased. These results suggest that increased plasma levels of endothelin and thrombomodulin may reflect microvascular damage in systemic sclerosis.

Adult↗

PubMed1981

Presence of eosinophilia in progressive systemic sclerosis and localized scleroderma.

Recent studies on eosinophilic fasciitis have lead to this investigation of eosinophilia in progressive systemic sclerosis and localized scleroderma. Eosinophilia (eosinophilic count greater than 300/cmm) was found in ten of 63 progressive systemic sclerosis patients (15.8%) and in two of nine localized scleroderma cases (22.2%). In patients with progressive systemic sclerosis eosinophilia was found occasionally in six cases; it was transitory, but frequent in three cases; it was constant and very high in one case. In patients with localized scleroderma eosinophilia was found occasionally in one case and frequent, but not constant, in the other one. The possible influence of drugs could be excluded in five cases: three progressive systemic sclerosis cases and two affected with localized scleroderma. Therefore, it is possible to confirm that eosinophilia is not a distinctive sign of eosinophilic fasciitis in patients suffering from scleroderma-like syndromes. While eosinophilia is related to inflammation in eosinophilic faciitis, eosinophilia and disease activity could not be correlated in our patients with PSS. Recently, it has been suggested that eosinophilia might be an unfavorable prognostic criterion in progressive systemic sclerosis. Our data does not allow confirmation of this assumption.

Adult↗

PubMed1997

Anorectal function in systemic sclerosis: correlation with esophageal dysfunction?

PURPOSE: This study was designed to compare esophageal and anorectal function parameters in patients with systemic sclerosis and to define the role of anorectal manometry in the diagnosis of gastrointestinal involvement of systemic sclerosis. PATIENTS AND METHODS: Twenty-six consecutive patients (22 females) with systemic sclerosis originally referred for assessment of esophageal function were evaluated by esophageal and anorectal manometry. Anorectal function parameters were compared between patients with normal and those with disturbed esophageal function. RESULTS: A total of 17 of 26 patients (65 percent) had severe esophageal dysfunction with aperistalsis of the lower two-thirds of the esophagus, whereas 9 patients (35 percent) had normal esophageal manometry. Only three patients (11.5 percent) suffered from occasional fecal incontinence. Anorectal function parameters (resting pressure, maximum squeeze pressure, perception threshold) were not significantly different between patients with normal and those with disturbed esophageal motility. Rectoanal inhibitory reflex was excitable in nearly 90 percent of patients. CONCLUSION: In an unselected group of patients with systemic sclerosis, fecal incontinence and abnormal anorectal function are rather rare findings. Anorectal manometry cannot differentiate between patients with and without gastrointestinal involvement of systemic sclerosis.

Adolescent↗

PubMed1994

Gastrobiliary motility after liquid fatty meal in progressive systemic sclerosis. A sonographic study.

Gastric emptying, antral motility, and gallbladder emptying after a liquid fatty meal were studied by ultrasound in 25 patients with progressive systemic sclerosis and in 25 sex- and age-matched controls. In patients with systemic sclerosis, the possible role of autonomic dysfunction was evaluated by four noninvasive cardiovascular reflex tests. Despite a significant delay of gastric emptying and a significant postprandial antral hypomotility in the patients with systemic sclerosis, the fat-induced gallbladder emptying was only slightly reduced, reaching no significant level when compared to the controls. The prolongation of gastric emptying correlated significantly with the duration of the disease. Although 36% of the patients in the systemic sclerosis group exhibited signs of autonomic cardiac dysfunction, there was no evidence of an association between these signs and gastric motor dysfunction. In conclusion motility disorders of the gallbladder seem to play a minor role in the upper gut involvement of systemic sclerosis, whereas motility disorders of the stomach are frequent and can be easily recognized noninvasively by real-time ultrasound.

Adult↗

PubMed1991

Diffuse systemic sclerosis and related diseases in Thailand.

Diffuse systemic sclerosis and the related syndromes were diagnosed in 115 patients during 1977-1987. Sixty-nine patients were available for clinical evaluation and analysis. These included 46 patients with diffuse systemic sclerosis, 2 with limited cutaneous systemic sclerosis, 2 with linear scleroderma, 18 with overlap syndrome (OS), and one with primary Raynaud's disease. The major clinical manifestations among the diffuse scleroderma patients were the involvement of the following systems; cutaneous (93.3%), musculoskeletal (69.6%), gastrointestinal (54.3%), and pulmonary (43.3%) while renal involvement (4.3%) was less common. Antinuclear antibodies were found in 89.7% with predominating speckle pattern (86.2%) and anti Scl 70 antibody (40.6%). In contrast to the Western series, HLA-DR2 was significantly increased in our diffuse systemic sclerosis patients (p less than 0.01). Among the 18 OS patients, the involvement of pulmonary system (44.4%) was the major cause of morbidity and renal involvement (55.6%) was found frequently although it was clinically mild. In conclusion, our diffuse systemic sclerosis and related diseases patients seem to have milder clinical manifestations than those of the Western series.

Adult↗

PubMed1998

Serum levels of soluble Fas/APO-1 receptor are increased in systemic sclerosis.

It has been suggested that rheumatic diseases may result from a deficit in Fas-mediated T-cell apoptosis. Recent studies have demonstrated increased soluble Fas in sera from lupus erythematosus patients. We were interested to determine whether elevated soluble Fas levels are associated with systemic sclerosis. Soluble Fas levels were retrospectively assayed using a sandwich enzyme-linked immunosorbent assay in serum from 30 patients with systemic sclerosis and 15 normal controls. Hospital medical records were retrospectively reviewed for clinical and laboratory characteristics of the patients. Soluble Fas levels were analysed in subsets of patients with limited (lcSSc) versus diffuse cutaneous systemic sclerosis (dcSSc) and correlated with inflammatory activity. In systemic sclerosis soluble Fas serum levels (lcSSc, 2.19 +/- 0.71 ng/ml, dcSSc 2.53 +/- 1.37 ng/ml) were significantly higher than in normal controls (1.26 +/- 0.36 ng/ml). However, there were no significant differences in soluble Fas levels between lcSSc and dcSSc and poor correlation between soluble Fas levels and inflammatory activity status. Detection of elevated soluble Fas might serve as a clinical marker for immunological dysregulation in systemic sclerosis, but not for inflammatory disease activity.

Antibodies, Antinuclear↗

PubMed2006

Acute myocardial infarction in systemic sclerosis patients: a case series.

To characterize the clinical manifestations of patients with systemic sclerosis who develop a myocardial infarction (MI), a retrospective review of the medical records of all patients who were admitted to our institution between 1982 and 2002 and had the dual diagnosis of systemic sclerosis and an acute MI was done. From 1,009 systemic sclerosis hospital admissions, 11 (1.09%) were for an acute MI. Three of these patients had normal coronaries, and instead of wall motion abnormalities, left ventricular hypertrophy was the predominant finding of an echocardiography. The odds ratio of finding normal coronaries in systemic sclerosis vs the general population who develops an acute MI is 33.89 (14.08-81.39). Seven of our patients had an elevated creatinine level on presentation. Acute MI is an uncommon manifestation in systemic sclerosis patients. Normal coronaries are seen more commonly in these patients as compared to the general population, while vascular, gastrointestinal, and renal involvement is prevalent in these patients.

Coronary Artery Disease↗

PubMed1988

Nuclear sclerosis after vitrectomy for idiopathic epiretinal membranes.

We performed a retrospective analysis of lens changes occurring after vitrectomy for idiopathic epiretinal membranes causing macular pucker on 75 consecutive phakic eyes. Thirty-five eyes (47%) had appearance or progression of nuclear sclerosis. There were no changes in anterior subcapsular cataracts and three eyes (4%) had minimal changes in posterior subcapsular cataracts. Based on univariate analysis, presence of preoperative nuclear sclerosis, length of follow-up period, and surgeon were found to be associated with the occurrence or progression of nuclear sclerosis. Differences between surgeons were largely accounted for by the difference in proportions with preoperative nuclear sclerosis, and differing lengths of follow-up. Kaplan-Meier life table analysis performed on 53 eyes with examination of fellow eyes and longer follow-up showed significantly more nuclear sclerosis in the operated on eye when compared to the fellow eye (log-rank test, P less than .0001).

Actuarial Analysis↗

PubMed1989

Abnormal postcibal antral and small bowel motility due to neuropathy or myopathy in systemic sclerosis.

Two hypotheses were tested: first, the postcibal motility disorders occur in both the antrum and small bowel of systemic sclerosis patients with gastrointestinal symptoms; second, that dysmotility may result from a neuropathic stage in this disease. Upper gut perfusion manometry (3 h fasting, 2 h fed) was performed and compared with data from similarly studied healthy laboratory controls (n = 15). Only 1 of 8 scleroderma patients had an interdigestive motor complex during the 3-h fasting period [0.1 +/- 0.1 (SEM) for systemic sclerosis vs. 1.4 +/- 0.16 (SEM) for controls, p less than 0.05]; 2 other patients had fasting duodenal incoordinated bursts of phasic pressure activity in the duodenum or proximal jejunum. Distal antral motility index [MI = In (sum of amplitude x number of contractions + 1)] was lower (12.4 +/- 0.5, p less than 0.01) than that in controls (14.4 +/- 0.14); both the amplitude and frequency of antral pressure activity were lower (p less than 0.05) in systemic sclerosis. The intestinal fed pattern was characterized by reduced amplitude and frequency of contractions in 6 patients; 2 patients had excessive phasic or tonic small bowel pressure activity, or both. Thus, antral hypomotility is present in most symptomatic scleroderma patients; proximal small bowel postcibal motility is characteristically reduced; a minority of such patients have incoordianted fasting or postcibal hypermotility suggestive of a neuropathy. Manometry identifies the type and region of dysmotility in systemic sclerosis; our results suggest that there are neuropathic and myopathic stages of the upper gut involvement in systemic sclerosis.

Adult↗

PubMed1988

Analysis of the oral manifestations of systemic sclerosis (scleroderma).

Thirty-one female patients with systemic sclerosis and 30 age- and sex-matched control subjects underwent a clinical and radiographic oral examination. The intercommissural distance, the interincisal distance, and the maximum oral aperture were all reduced in patients with systemic sclerosis. Xerostomia was present in 70% of the patients and was associated with an increased frequency of dental caries. Diseased, missing, and filled surfaces, mobile teeth, and periodontal disease were more prevalent in patients with systemic sclerosis than control subjects. Periodontal membrane width was increased in patients with systemic sclerosis and involved all groups of teeth. Mandibular erosions were seen in 9 of 31 patients and involved the mandibular angle, the condylar head, the coronoid process, and the digastric region. These lesions were associated with the presence of periodontal membrane thickening and were seen in patients with more severe systemic sclerosis.

Adult↗

PubMed1994

Analysis of the oral manifestations of systemic sclerosis.

The oral signs and symptoms in 32 patients with systemic sclerosis were evaluated. Oral mucosal telangiectasia was present in 18 cases (56.3%) and was not restricted to the limited form of systemic sclerosis. The interincisal distance was significantly decreased in the patients with systemic sclerosis compared with the 17 controls (p < 0.001). The distance between the vermillion borders was also significantly decreased when the 14 patients with salivary hypofunction were compared with the 18 cases without decreased salivary secretion (p < 0.05). Twenty-two (69%) of the patients exhibited keratoconjunctivitis sicca, salivary hypofunction, or both. Lip biopsy was performed in 16 cases. Two cases with inflammatory signs characteristic of Sjögren's syndrome were found, and six patients showed the histologic signs of labial gland fibrosis. Five of these cases belonged to the group of limited cutaneous systemic sclerosis that indicates the generalized nature of the fibrotic processes even in systemic sclerosis with less extensive skin involvement. Of the 10 cases investigated by electron microscopy, all but one showed a thickening of the capillary basal lamina, lamellar arrangement within the basement membrane, or capillary endothelial vacuolization. Three of these cases belonged to the patients with disease onset within 2 years, showing that capillary vascular lesion is present in the early cases and that vascular injury affects even those tissues that do not seem to be evidently involved by clinical examination.

Antibodies, Antinuclear↗

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↗

PubMed2002

High prevalence of polymorphisms of angiotensin-converting enzyme (I/D) and endothelial nitric oxide synthase (Glu298Asp) in patients with systemic sclerosis.

PURPOSE: Systemic sclerosis is characterized by progressive microvascular occlusion and fibrosis and by an imbalance in the fibrinolytic system. In vivo and in vitro studies suggest that the renin-angiotensin system partly regulates vascular fibrinolytic balance. Angiotensin II increases the production and secretion of plasminogen activator inhibitor-1, while angiotensin-converting enzyme (ACE) contributes to reduced production of tissue plasminogen activator and endothelial nitric oxide synthesis by bradykinin degradation. The aim of our study was to investigate the effects of ACE insertion/deletion (I/D) and endothelial nitric oxide synthase (eNOS) Glu298Asp (G894-->T) and T-786-->C polymorphisms in patients with systemic sclerosis. SUBJECTS AND METHODS: We studied 73 consecutive patients (47 with limited and 26 with diffuse cutaneous systemic sclerosis) and 112 control subjects. ACE I/D and eNOS polymorphisms were genotyped by polymerase chain reaction-restriction fragment length polymorphism analysis. RESULTS: The ACE I/D and the eNOS G894-->T polymorphisms were more common in patients than in controls (for the ACE D allele: odds ratio [OR] = 3.4; 95% confidence interval [CI]: 1.5 to 7.9; P = 0.003; for the eNOS T allele: OR = 1.9; 95% CI: 1.0 to 3.4; P = 0.04). There was no association between the eNOS T-786-->C polymorphism and systemic sclerosis. CONCLUSIONS: Our findings of an increased risk of systemic sclerosis in ACE D and eNOS 894T allele carriers suggest that these polymorphisms may contribute to the pathogenesis of the disease.

Adult↗

PubMed1989

Progressive systemic sclerosis sine scleroderma presenting as pulmonary interstitial fibrosis.

PURPOSE: The purpose of this work was to report the clinical features of 10 patients with systemic sclerosis presenting with lung disease in the absence of cutaneous involvement (systemic sclerosis sine scleroderma). PATIENTS AND METHODS: The study involved a retrospective review of patients with systemic sclerosis at a tertiary care facility presenting with clinical and/or radiographic evidence of interstitial lung disease in the absence of cutaneous manifestations of scleroderma, and a MEDLINE, computer search for patients who presented with lung disease and later developed taut skin (scleroderma). RESULTS: Ten patients with systemic sclerosis sine scleroderma presenting with pulmonary signs or symptoms were discovered. A positive antinuclear antibody (seven of eight), esophageal dysmotility (eight of nine), restrictive lung disease (six of eight), Raynaud's phenomenon (five of eight), and abnormal nailfold capillaries (six of six) were present in these patients. Six of these 10 patients developed typical scleroderma from four months to seven years after presentation. Lung pathology was examined in six patients and all had changes consistent with interstitial pneumonitis and fibrosis. Four of the 10 patients had occupations that could have exposed them to potential pulmonary toxins. In contrast to patients with typical scleroderma, all 10 patients we discovered were men. conclusions: Systemic sclerosis should be considered in patients with idiopathic interstitial lung disease even in the absence of classic sclerodermatous cutaneous involvement. An esophagram, antinuclear antibody, and widefield nailfold examination may facilitate the diagnosis.

Diagnosis, Differential↗

PubMed1995

Plasma endothelin-1 levels, pulmonary hypertension, and lung fibrosis in patients with systemic sclerosis.

PURPOSE: To investigate the behavior of circulating endothelin (ET)-1 concentrations in patients affected by systemic sclerosis, and to elucidate the possible relationships existing in this disease among plasma peptide levels, pulmonary hypertension, and lung fibrosis. PATIENTS AND METHODS: Circulating ET-1 levels were determined by reverse-phase, high-pressure liquid chromatography followed by sensitive radioimmunoassay in 20 patients affected by systemic sclerosis (18 women and 2 men, mean age 48.1 +/- 13.7 years) with or without pulmonary hypertension as evaluated by Doppler echocardiography, or lung fibrosis as measured by a score method based on lung examination by high-resolution computed tomography (HRCT). A group of 18 normal volunteers served as controls (15 women and 3 men, mean age 45.0 +/- 10.1 years). RESULTS: Plasma ET-1 levels were significantly higher (P < 0.001) in patients with systemic sclerosis (1.72 +/- 0.28 pg/mL) than in control subjects (0.63 +/- 0.06 pg/mL). Pulmonary artery systolic hypertension was detected in 10 patients (50%) with systemic sclerosis (56.2 +/- 18.0 mm Hg, range 37 to 97) versus none of the control subjects (30.2 +/- 2.2 mm Hg, P < 0.0001). Lung fibrosis was present in 12 patients (60%), with an HRCT overall score of 9.0 +/- 4.6. There were no significant differences in plasma ET-1 levels between patients with pulmonary hypertension (1.58 +/- 0.20 pg/mL) or without it (1.76 +/- 0.39 pg/mL, P = 0.188, not significant [NS]); or between patients with lung fibrosis (1.65 +/- 0.14 pg/mL) or without fibrosis (1.78 +/- 0.37 pg/mL, P = 0.290, NS). In particular, 6 patients had neither pulmonary hypertension nor lung fibrosis. In these patients, plasma ET-1 levels were similar compared with the others (1.85 +/- 0.49 versus 1.66 +/- 0.13, respectively; P = 0.180, NS). No correlations were observed between ET-1 levels and either pulmonary pressure levels or HRCT overall scores. CONCLUSIONS: The use of a sensitive assay, highly selective for ET-1, showed higher levels of circulating peptide in patients affected by systemic sclerosis than in control subjects. Neither pulmonary hypertension nor lung fibrosis was accompanied by a further rise in plasma ET-1 concentrations.

Adult↗

PubMed1997

Endothelin may be pathogenic in systemic sclerosis of the heart.

We evaluated 30 consecutive patients and 48 age- and sex-matched controls to explore the possibility of a pathogenic contribution by plasma endothelin-1 in the cardiac expression of systemic sclerosis. Venous plasma endothelin-1 was measured by radio-immunoassay and left ventricular function by echocardiography. The patient group had elevated plasma endothelin-1 (2.6 +/- 0.2 vs. 1.8 +/- 0.1 pmol/1, P < 0.001), but endothelin-1 was not related to age, heart rate, blood pressure, total peripheral resistance, disease duration or systemic sclerosis score. Endothelin-1 was related to left ventricular hypertrophy in terms of septal thickness (r = 0.33, P < 0.01) and left ventricular mass index (r = 0.32, P < 0.01). Plasma endothelin-1 was further related to measures indicating reduced left ventricular filling; left atrial emptying index (r = -0.50, P < 0.0005), the first third filling fraction (r = -0.31, P < 0.05) and the time velocity integral of Doppler early/late filling velocity (r = -0.40, P < 0.001). Furthermore, circulating endothelin-1 was related to impaired left ventricular contractility as estimated by pre-ejection period/left ventricular ejection time (r = 0.32, P < 0.01) and end-systolic wall stress/volume index (r = -0.30, P < 0.05). We conclude that plasma endothelin-1 is elevated in relation to the degree of left ventricular hypertrophy, diastolic dysfunction and impaired contractility in systemic sclerosis. It may be of pathogenic importance to the cardiac involvement in systemic sclerosis which is not mediated via an increase in systemic blood pressure. It is not yet clear whether our findings are exclusive to systemic sclerosis patients or represent a generalized phenomenon in patients with impaired left ventricular function.

Adult↗