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Results for “Scleroderma, Systemic”

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PubMed1992

Enhanced expression of high-affinity interleukin-2 receptors in scleroderma: possible role for IL-6.

Scleroderma (systemic sclerosis) is characterized by tissue fibrosis, a distinctive vascular and microvascular disorder, and a perivascular mononuclear cell infiltration of involved organs. The pathogenesis of scleroderma is not known; however, there is evidence for a cell-mediated immune mechanism in the disease. Enhanced IL-2 production has been documented both in vivo and in vitro. In this study, the effect of IL-2 on lymphocyte proliferation in vitro was examined. An enhanced proliferative response to IL-2 was seen in scleroderma lymphocytes over that in matched control lymphocytes. Since high-affinity IL-2 receptors (HIL-2-R) mediate the growth-promoting activity of IL-2, we examined HIL-2-R expression on lymphocytes from 13 scleroderma and 11 matched control subjects by a radioiodinated IL-2 binding assay. Significantly higher numbers of HIL-2-R were noted in scleroderma cells (3054 +/- 618 in scleroderma vs 1721 +/- 181 in control cells, mean +/- SD; P less than 0.001). The addition of IL-6 to control cell cultures 24 hr prior to binding determination led to changes in IL-2 binding that were identical to scleroderma cell binding characteristics, while the addition of neutralizing IL-6 antibody to scleroderma cells led to a reduction in HIL-2-R expression. Other cytokines (IL-1, IL-3, IL-4, IL-5, TNF, LT, IFN-gamma, and TGF-beta) had no effect on IL-2 binding, suggesting that IL-6 may mediate the enhanced expression of HIL-2-R. This conclusion was further supported by the finding that scleroderma lymphocytes released in vitro 10- to 20-fold higher concentrations of IL-6 than control cells. The data demonstrate an amplification of IL-2 binding in scleroderma and suggest IL-6 as the mediator of this phenomenon.

Humans↗

PubMed1996

Progressive systemic sclerosis sine scleroderma which developed after exposure to epoxy resin polymerization.

Progressive systemic sclerosis (PSS) sine scleroderma is well known as a special form of scleroderma. Because of its rarity, its pathogenesis has not yet been elucidated. We experienced a 33-year-old man who developed PSS sine scleroderma while working with epoxy resin polymerization. He had short white frenulum linguae, diffuse hyperpigmentation and facial telangiectasia, positive antinuclear antibody, and pulmonary dysfunction, but not acrosclerosis or sclerodactylia. Modest dermal collagen proliferation in the forearm skin confirmed PSS sine scleroderma. Epoxy resin polymerizer appears to be a potent causative agent for PSS sine scleroderma as well as for generalized morphea-like PSS.

Adult↗

PubMed1977

Direct immunofluorescence in the diagnosis of scleroderma syndromes.

Immunofluorescent study of the skin of nine patients with mesenchymal, inflammatory scleroderma (mixed connective tissue disease) revealed immunoglobulin and complement deposition at the basement membrane or within blood vessel walls. The skin specimens of ten patients with systemic scleroderma were negative for immunofluorescence. It is proposed that basement membrane or vascular (or both) immunofluorescence is an excellent means of identifying the infrequent patient who has scleroderma and myositis or lupus erythematosus in whom a corticosteroid response may occur.

Adolescent↗

PubMed2006

Scleroderma: it has been a long hard journey.

Progress in the understanding of the aetiopathogenesis of scleroderma (systemic sclerosis) has been painfully slow and this has greatly impeded the application of specific disease modifying treatments. This article provides a brief historical overview of scleroderma (including the important Australian contribution of Dr Alfred Barnett and colleagues--see accompanying article in this issue on pages 513-18), highlights some recent pathogenic developments and summarises some exciting new therapies. Cautious optimism can now be offered to scleroderma sufferers.

Animals↗

PubMed1977

[Scleroderma presenting as oedema (authors transl)].

The authors report 8 cases of scleroderma with oedema in the initial stages which could be divided amongst three groups on the basis of their clinical, biological, histological characteristics and course: normal systemic scleroderma, mixed connective tissue disease, Shulmans syndrome. The relationship between one of these cases and Buschke's scleroderma is discussed. These cases of scleroderma are characterized by diagnostic difficulties in the initial phase and by their relatively favourable vital prognosis (one death only). Corticosteroids may be effective to some extent in the presence of mixed connective tissue disease of Shulman's syndrome.

Adrenal Cortex Hormones↗

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↗

PubMed1997

Scleroderma fibroblasts show increased responsiveness to endothelial cell-derived IL-1 and bFGF.

Fibroblasts cultured from lesional skin in scleroderma (systemic sclerosis) demonstrate an activated phenotype that may be important in pathogenesis. Endothelial cell-derived cytokines can modulate fibroblast properties, and endothelial cell changes occur early in scleroderma. Thus, endothelial cell and fibroblast dysfunction may be linked through the paracrine activity of soluble endothelial cell products. We have explored endothelial cell-fibroblast interactions in vitro by investigating the modulation of scleroderma and control fibroblast properties by endothelial cell-conditioned medium (EC-CM). EC-CM caused a concentration-dependent stimulation of fibroblast DNA and protein synthesis and upregulation of cell surface ICAM-1 expression. Scleroderma fibroblasts showed consistently greater responses than control cells. Medium conditioned by mechanically wounded endothelial cells had a greater effect than that from resting endothelial cells. Pre-incubation of EC-CM with anti-bFGF significantly reduced the promotion of fibroblast thymidine incorporation but did not affect endothelial cell-induced leucine incorporation. Conversely, anti-IL-1 antibodies abrogated EC-CM-induced leucine incorporation and ICAM-1 expression but did not diminish thymidine incorporation. Recombinant bFGF or IL-1 modulated fibroblast properties similarly. These data demonstrate that endothelial cell-derived IL-1 and bFGF modulate fibroblast properties independently and that lesional scleroderma strains are more responsive than control fibroblasts to endothelial cell-induced modulation, which supports the hypothesis that altered endothelial cell-fibroblast communication may be involved in the pathogenesis of scleroderma.

Cell Count↗

PubMed1992

[Male-type scleroderma: the role of occupational exposure].

A number of occupational exposure risks have been suspected as potential causes of scleroderma (systemic sclerosis), particularly in males. These include silica dust and numerous organic chemicals. With particular reference to the type of employment, we have reviewed the charts of male scleroderma patients receiving treatment in our Rheumatology Department since 1976. Of 39 patients, 25 could be included in the present study. Among these, 14 (56%) had an occupational activity which put them at risk: 11 were exposed to silica dust, one to organic chemicals and two to both silica dust and chemicals. Mean exposure time was 14.5 years (range 4-33 years); and the interval between the beginning of exposure and the onset of scleroderma averaged 24.4 years (range 4-45 years). Considering these 14 cases as silica- or chemically-induced scleroderma, we found that, except for the absence of muscular involvement, the clinical features and prognosis were similar to those of "idiopathic" scleroderma patients. Antinuclear antibodies and autoantibodies directed against Scl-70 were also found in both occupationally induced and idiopathic scleroderma. Our results emphasize the unusually high frequency of occupational exposure in scleroderma in males. Hence, a detailed professional history should be taken for all scleroderma patients. Our results also suggest that, in certain cases, scleroderma should be classified as an occupational disease.

Adult↗

PubMed1986

[Serum lysosomal hydrolases in various forms of scleroderma].

The activities of several lysosomal hydrolases (beta-galactosidase, beta-hexosaminidase, alpha-mannosidase, beta-glucuronidase and acid phosphatase) were determined in serum and blood lymphocytes from patients affected with scleroderma. Statistical comparisons between means of patient and control groups (Student's t test) showed a significant difference in serum beta-galactosidase and acid phosphatase between patients and controls (serum beta-galactosidase: 36.5 +/- 22 nmol/h/ml in the scleroderma group versus 24 +/- 13 nmol/h/ml in the control group; serum acid phosphatase 853 +/- 345 nmol/h/ml in the scleroderma group versus 634 +/- 295 nmol/h/ml in the control group). These differences were significant (p less than 0.01). Both enzyme activities were also significantly increased in the group with systemic scleroderma, but the difference was less in the group with localized scleroderma (this is discussed in terms of statistics and pathophysiology). The other enzyme activities determined were not significantly modified. The validity of these results is discussed, together with their diagnostic value and with the pathophysiological hypotheses put forward to explain the high levels found.

Adult↗

PubMed1996

Increased pigmentation in scleroderma.

OBJECTIVE: Increased pigmentation is found in patients with scleroderma (systemic sclerosis, SSc) even in areas that have never clinically been involved with skin thickening. We wanted to determine if the pigmentation is due to subclinical sclerodermatous changes, or a systemic factor such as an increase in adrenocorticotropic hormone (ACTH). METHODS: To determine if clinically uninvolved skin that is pigmented differs from nonpigmented skin, skin biopsies were taken from patients with scleroderma from 2 different sites: (1) from pigmented but otherwise clinically normal skin, and (2) from nonpigmented clinically normal skin adjacent to the first site. Biopsies were examined by a dermatopathologist for evidence of dermal and epidermal changes. Cosyntropin stimulation tests were performed after baseline cortisol and ACTH samples were obtained. RESULTS: Six patients with diffuse SSc (dSSc) and 4 with limited SSc (ISSc) had skin biopsies. Patients with dSSc were more likely than those with ISSc to have increased pigmentation in uninvolved skin. Pigmented skin specimens had either a higher content of epidermal melanin and/or a more severe degree of pigmentary incontinence with a higher number of dermal melanophages in the superficial dermis. The cosyntropin stimulation tests were normal and there were no differences between subjects with diffuse and limited SSc or between those with and without increases in pigmentation. CONCLUSION: Clinically uninvolved skin in many of these patients with SSc was abnormal, and subtle changes of SSc were present, especially in the pigmented biopsies. There was no evidence of adrenal deficiency in these patients. It is difficult pathologically to differentiate the changes in pigmented compared to unpigmented skin in patients with SSc, except for changes of increased melanin and pigmentary incontinence.

Adrenocorticotropic Hormone↗

PubMed1988

[Combination of scleroderma and non bronchiolo-alveolar bronchial carcinoma. Report of a case and short review of the literature].

The authors report a case of broncho-pulmonary squamous cell carcinoma developed in a 59-year old woman who for 11 years had been suffering from systemic scleroderma for which she never consulted. The carcinoma was revealed by haemoptysis, dyspnoea and weight loss. It extended secondarily to the pleura. The authors have reviewed the literature on non-alveolar squamous cell carcinoma. They conclude that this type of cancer is rarely diagnosed before death and has a very poor prognosis.

Adenocarcinoma, Bronchiolo-Alveolar↗

PubMedDate not supplied

Cutaneous fibrinolytic activity in scleroderma.

Fibrinolytic activity is notoriously related to the maintenance of an adequate microcirculatory blood flow and its impairment is related to thrombosis. In this report cutaneous fibrinolytic activity has been evaluated with an autohistographic method in ten subjects with morphea and in five subjects with systemic sclerosis before and after injection of histamine into the skin. Cutaneous fibrinolytic activity (CFA) appeared normal (3/5 cases) or increased (2/5) in uninvolved and involved skin in systemic scleroderma and always increased after histamine injection. In circumscribed scleroderma the CFA appeared reduced in 2/10 cases, normal in 4/10 cases and increased in 4/10 cases. These data show that an endothelial pool of plasminogen activator is available in the skin of scleroderma patients and that fibrinolytic agents can rationally be employed only in certain stages of the disease.

Adult↗

PubMed1980

Atrioventricular nodal function in progressive systemic sclerosis: electrophysiological and morphological findings.

Electrophysiological data consistent with atrioventricular nodal dysfunction were obtained in 10 out of 19 patients with progressive systemic sclerosis (scleroderma). Conducting system studies were carried out in a further seven patients with progressive systemic sclerosis. In each the proximal portion of the atrioventricular node was consistently found to be smaller and more slender. It is postulated that there is a relation between proximal atrioventricular nodal structural alterations and impaired atrioventricular nodal function.

Atrioventricular Node↗

PubMed2006

Bier's white spots associated with scleroderma renal crisis.

Bier's spots are asymptomatic and permanent white macules, standing on a cyanotic background. These spots are associated with venous stasis and usually appear in physiological conditions. We report the case of a 47-year-old woman with a 2-year history of systemic scleroderma, who developed Bier's white spots associated with a vascular and renal crisis. Interestingly, these spots appeared with the crisis, and disappeared with the treatment and resolution of the crisis. They are probably a result of anatomical and functional damage to the small vessels of the skin. The pathophysiology in the skin is probably the same as that which happens in renal vessels during scleroderma renal crisis.

Angiotensin-Converting Enzyme Inhibitors↗

PubMed1990

Scleroderma esophagus.

Scleroderma (systemic sclerosis) is a connective tissue disorder characterized by thickening and fibrosis of the skin and visceral involvement that may include the heart, lungs, kidneys, and gastrointestinal tract. At least 40-50% of patients with scleroderma experience esophageal symptoms such as heartburn and dysphagia, while up to 90% of patients have esophageal dysfunction on objective testing at some point in their disease. The disease results in smooth muscle dysfunction that causes esophageal aperistalsis and reduced lower esophageal sphincter pressures. Gastroesophageal reflux with poor acid clearance results with an increased incidence of complications such as peptic stricture and Barrett's esophagus. Aggressive medical therapy is necessary to prevent these and other complications of gastroesophageal reflux.

Esophageal Diseases↗

PubMed2000

[POEMS syndrome revealed by a scleroderma-like skin thickening].

BACKGROUND: POEMS syndrome is a rare form of plasma-cell dyscrasia characterized by the various association of Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal component and Skin changes. The most frequent skin changes such as hyperpigmentation, hypertrichosis, scleroderma-like skin thickening and angiomas are not pathognomonic but nearly constant. However, they are rarely isolated. CASE REPORT: A 57-year-old Caucasian woman presented with a 6 month history of skin thickening on both hands and feet, Raynaud's phenomenon and facial telangiectasias. Physical examination on presentation revealed hepatomegaly, signs of a sensorimotor peripheral neuropathy which was demyelinating in type on electrophysiological assessment, and ankle edemas. Initial laboratory investigations revealed a platelet count of 900 000/mm(3), a monoclonal IgG lambda gammapathy. Plasma-cells were slightly increased (10 p. 100 of marrow elements) and full skeletal radiographs showed no focal osteosclerotic or lytic lesion. A diagnosis of POEMS syndrome was made. The patient was treated with tamoxifen, methylprednisolone and plasmapheresis without improvement in polyneuropathy or in skin changes. DISCUSSION: Our patient satisfied the criteria for POEMS syndrome. The most typical feature here was the scleroderma-like skin change, which has been recognized by other authors. But, in the present case, Raynaud's phenomenon, skin thickening and facial telangiectasias were present 6 months before the diagnosis, and initially suggestive of systemic scleroderma, confirmed histologically. The pathogenesis of POEMS syndrome might be regarded as the result of a marked activation of the proinflammatory cytokine network, but an increase in serum Vascular Endothelial Growth Factor (VEGF) levels could well account for other manifestations such as skin thickening.

Anti-Inflammatory Agents↗

PubMed1979

Cancer and scleroderma.

Of 2,141 patients with scleroderma who were seen at the Mayo Clinic, Rochester, Minn, between 1959 and 1975, 78 had 87 internal malignancies. The relative frequency of the types of cancers was similar to that for the general population. The increased numbers of patients with breast and uterine carcinomas were consistent with the female preponderance in scleroderma. Contrary to previous reports, carcinoma of the lung was not the most frequent malignancy associated with scleroderma. The lymphoma-leukemia malignancies were second only to breast carcinoma in frequency, comprising about 17% of the malignancies. Both conditions developed within a three-year period in 68% (45/66) of our patients affected with systemic scleroderma, and this subgroup comprised a high mortality group. This study is not an epidemiologic one in the strictest sense, but it is a review of the experience of the Mayo Clinic with patients who have had both scleroderma and an internal malignancy.

Adolescent↗

PubMed1977

[Lung and pleural involvement in collagen disease (author's transl)].

Frequency and type of pulmonary and pleural involvement in collagen disease (rheumatoid arthritis, progressive systemic scleroderma, polymyositis-dermatomyositis and lupus erythematodes are analysed on the basis of literature and of own cases with particular regard to the roentgenographic appearance.

Adult↗