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Results for “Systemic sclerosis”

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At least 217 records · Page 12Linked to original sources

PubMed2005

[Genetic factors predisposing to fibrosis in systemic sclerosis].

PURPOSE: Physiopathology of systemic sclerosis includes autoimmunity factors, endothelial lesions and abnormal fibrotic process which characterizes this disease in the field of systemic autoimmune disorders. Genetic factors of susceptibility are showed by possibility of familial forms of the disease, Choctaw American Indians homogenous population with high disease prevalence of systemic sclerosis and experimental animal models. KEY POINTS: We propose a review of the articles published to date in the literature concerning genetical analysis of genes coding for factors potentially involved in the fibrotic process of systemic sclerosis. This includes cytokines (TNF-alpha, interleukin-1, chemokines), growth factors (TGF-beta), extracellular matrix proteins (collagen, fibrillin, fibronectine) and agents acting on vascular tone (angiotensin-converting enzyme and NO synthase). PERSPECTIVES: Identification of genetic factors involved in the susceptibility to fibrosis of systemic sclerosis would lead to a better understanding of physiopathological mechanisms of this disease and to therapeutic targets using immunomodulation with drugs, such as already performed in rheumatoid arthritis.

Animals↗

PubMed2003

Fibroblast and endothelial apoptosis in systemic sclerosis.

PURPOSE OF REVIEW: Systemic sclerosis is a disease characterized by vascular and skin changes associated with activation of fibroblasts and increased synthesis of matrix components. These abnormalities lead to fibrosis and impaired function of internal organs such as the lung, kidney, and gastrointestinal tract. Recent evidence suggests that although activation of cells in and around the blood vessels and in the skin occurs in systemic sclerosis, injury to the vascular endothelium and defective apoptosis of skin fibroblasts may also contribute to disease. The purpose of this review is to discuss these findings in the context of the pathophysiology of systemic sclerosis. RECENT FINDINGS: This review highlights concepts and recent findings relating to apoptosis of vascular endothelium and skin fibroblasts. Important paradigms of fibroblast cell death in wound healing and keloid formation are discussed. Recent observations describing resistance of systemic sclerosis fibroblasts to Fas-mediated apoptosis and activation of the antiapoptotic protein kinase, Akt, are mentioned as possible contributors to fibroblast selection in this disease. SUMMARY: Improved understanding of how death and survival signals affect vascular endothelial cells and skin and visceral fibroblasts will lead to new approaches to therapy.

Apoptosis↗

PubMed2005

[Systemic sclerosis--clinical course and treatment possibilities].

Systemic sclerosis is an autoimmune disease, characterized by fibrosis of the skin and internal organs, such as esophagus, heart, lungs, and kidneys. Three main forms of systemic sclerosis are conspicuous: diffuse systemic sclerosis, limited systemic sclerosis and scleroderma sine sclerosis. Respective variants differ in skin sclerosis exacerbation, progress and prognosis; whereas organ involvement, osseous and articular alterations are comparable in all forms of the disease. Due to the fact that etiology of systemic sclerosis is not known, no specific therapy has been developed. The therapy is based on influencing main pathogenetic factors, such as peripheral circulation disturbances, abnormal immune activation and increase in collagen accumulation. An overview of clinical course of systemic sclerosis and new therapeutic modalities is provided.

Connective Tissue↗

PubMed1999

Bowel problems in patients with systemic sclerosis.

BACKGROUND: The impact of systemic sclerosis on bowel function is still unknown. The aim of this study was therefore to assess the frequency and severity of colorectal problems among patients with systemic sclerosis and to determine whether these problems are associated with age, gender, type of systemic sclerosis, or time since diagnosis. METHODS: A detailed questionnaire describing diarrhoea, constipation, obstructed defecation, faecal incontinence, bowel habits, social activities, and quality of life was sent to 96 consecutive patients with systemic sclerosis. RESULTS: Among 83 respondents (86%) 16% did not have a normal desire to defecate, 18% regularly needed digital stimulation or evacuation of the rectum, and 38% had faecal incontinence. Most patients (79%) had episodes of diarrhoea, and 38% had this once or more each month. Overall, 20% reported that colorectal dysfunction caused some or a major restriction of social activities or the quality of life. CONCLUSIONS: Colorectal dysfunction is very common among patients with systemic sclerosis, often restricting social activities and the quality of life. Therefore, further studies of colorectal pathophysiology in patients with systemic sclerosis are needed.

Anal Canal↗

PubMed1979

Trigeminal neuropathy as the presenting symptom of systemic sclerosis.

Three cases of systemic sclerosis in which trigeminal neuropathy was the presenting symptom are described. All 3 patients had progressive diseases and 2 died from its complications. In each case extensive neurological investigation was undertaken before the disease was recognized and it is suggested that earlier recognition of the systemic sclerosis might obviate the need for this. Trigeminal neuropathy in systemic sclerosis was associated with a poor prognosis in 2 of the patients.

Adult↗

PubMedDate not supplied

Defective vasculogenesis in systemic sclerosis.

BACKGROUND: Typical vascular features of systemic sclerosis include low capillary density and vascular obliteration. The formation and repair of blood vessels in adults involve vasculogenesis, which is mediated through the recruitment of bone-marrow-derived circulating endothelial precursors (CEP). We investigated whether vasculogenesis is impaired in patients with systemic sclerosis. METHODS: Peripheral blood was obtained from 11 patients with systemic sclerosis, 11 with rheumatoid arthritis, and 11 healthy controls. Factors potentially affecting the CEP number were matched among the three groups. CEP (identified as circulating cells positive for CD34, CD133, and the type 2 receptor for vascular endothelial growth factor) were quantified by cell sorting and three-colour flow cytometry. The circulating concentrations of angiogenic factors were measured by ELISA. The potential of CEP to differentiate into endothelial cells was assessed by the upregulation of von Willebrand factor after in-vitro maturation treatment. Findings The absolute number of CEP was much lower in patients with systemic sclerosis than in patients with rheumatoid arthritis or healthy controls (median 274 [IQR 178-395] vs 1154 [653-1524] and 1074 [713-1186] per 20 mL peripheral blood, respectively; p<0.0001 by Kruskal-Wallis test. Paradoxically, circulating concentrations of most angiogenic factors were significantly higher in patients with systemic sclerosis than in healthy controls. The proportion of CEP that differentiated into endothelial cells was significantly lower in patients with systemic sclerosis than in healthy controls (p<0.0001, Mann-Whitney test). INTERPRETATION: Insufficient vascular repair machinery due to defective vasculogenesis might contribute to vasculopathy in systemic sclerosis. RELEVANCE TO PRACTICE: As well as providing an important insight into the pathogenesis of this disorder, these findings suggest that dysregulated vasculogenesis might be important in other disorders with abnormalities in vascular formation, including those with excessive formation of new vessels such as cancer and those with deficient vessel formation such as atherosclerosis. Circulating endothelial precursors could be a novel target for therapeutic strategies for ischaemic complications in patients with systemic sclerosis.

AC133 Antigen↗

PubMed1986

Benoxaprofen in treatment of systemic sclerosis.

Ten patients with systemic sclerosis were treated with benoxaprofen, a potent lipoxygenase inhibitor, for a period of 6 months. In an attempt to evaluate the efficacy a number of physical disease parameters were followed during the trial. None of these parameters revealed any significant differences. There was, however, a trend for a change towards normalisation of a defect monocyte chemotaxis. In view of the slow and progressive nature of systemic sclerosis the present study leaves undetermined whether benoxaprofen exerts a beneficial effect on systemic sclerosis.

Adult↗

PubMed1999

Surgery in patients with systemic sclerosis of the hand.

Systemic sclerosis of the hand is an uncommon form of arthritis that can cause significant functional loss in the hand. It poses serious problems for the surgeon because of the diminished circulation of the digits. The tightness of the skin and joint stiffness that are characteristic of this condition make restoration of function difficult. Calcinosis, which is common in patients with systemic sclerosis of the hand, can be treated by excision or curettage. Patients with severe deformities of the digits and thumb often require fusions to improve grasp. Joint replacements with implants are not as successful in patients with systemic sclerosis as they are in patients with rheumatoid or degenerative arthritis. There have been recent attempts to improve the circulation to the fingers by sympathectomy or by vein grafts. These techniques, however, provide only temporary improvement and do not prevent or correct the severe digital and thumb deformities associated with this disease.

Arthritis↗

PubMed1992

Neurologic manifestations of progressive systemic sclerosis.

Neurologic involvement in progressive systemic sclerosis is considered uncommon. We retrospectively examined the prevalence and nature of neurologic complications in 50 patients with progressive systemic sclerosis. In 20 (40%), neurologic abnormalities were detected, with a total of 28 neurologic manifestations. All levels of the central and peripheral nervous system were affected: muscle (22%), peripheral nerve (18%), spinal cord (8%), and brain (6%). Of note were the presence of myelopathy in four patients and inclusion-body myositis in two. In 10 patients (20%), no definable cause of the neurologic dysfunction could be identified, apart from progressive systemic sclerosis. Thus, neurologic presentations of progressive systemic sclerosis are much more common than previously reported and may be due to direct involvement of the nervous system by a primary pathologic process in a significant number of patients.

Adult↗

PubMed2003

Pupillocynetic activity of substance P in systemic sclerosis.

OBJECTIVE: In systemic sclerosis (SSc), dysfunctions of peripheral nervous system (PNS) have been observed. Substance P (SP) instillation in human eye induces a cholinergic-independent pupil myosis. Pupil basal diameters (PBD) and pupil responsiveness to SP, expressed as area under the curve (AUC), were studied by pupillometry to assess SP-ergic fiber state and function in SSc. METHODS: Forty SSc patients [24 with limited (lSSc), 16 with diffuse (dSSc) disease] and 40 controls underwent pupillometric evaluation. After evaluation of PBD, SP 10-3 M was instilled in one eye and placebo in the contralateral eye. Antinuclear (ANA), anticentromere (ACA), and anti-Scl-70 autoantibodies were correlated with PBD and AUC. RESULTS: PBD was significantly lower in SSc patients versus controls (p < 0.001). PBD was minor in lSSc versus both dSSc and controls (p < 0.05), but no difference was found between dSSc and controls. In SSc, SP 10-3 M induced greater myosis compared to controls (p < 0.001). SP 10-3 M-induced myosis was higher in lSSc versus both dSSc and controls (p < 0.05). ACA significantly correlated with decreased values of PBD and AUC (p < 0.001). CONCLUSION: Our results show that PBD is reduced in patients with SSc and that SP induces a more intense myosis in SSc than controls. Moreover, in lSSc PBD is lower and SP increases the myosis in lSSc compared to dSSc and controls. This suggests a peculiar dysfunction of PNS in patients with the limited subset of SSc.

Aged↗

PubMed1996

Signal-averaged electrocardiography and echocardiography in the evaluation of myocardial involvement in progressive systemic sclerosis.

To assess the myocardial involvement in progressive systemic sclerosis we evaluated the presence of late potentials by signal-averaged electrocardiography (signal-averaged ECG) and the left ventricular function by M-mode, two dimensional and Doppler echocardiography. Fifteen outpatients, 7 with diffuse progressive systemic sclerosis and 8 with CREST syndrome variant, without clinical or electrocardiographic evidence of cardiac disease were studied and compared with 18 normal subjects. Late potentials occurred in 5 out of 15 progressive systemic sclerosis patients (33%) with a significant difference versus controls (P < 0.05) and were present only in the patients with diffuse progressive systemic sclerosis (P < or = 0.001 vs. controls). All progressive systemic sclerosis patients showed a normal left ventricular systolic function. Abnormal left ventricular filling was found in 9 progressive systemic sclerosis patients (5 with diffuse progressive systemic sclerosis and 4 with CREST). A more severe impairment of the mean values of diastolic function indexes was found in diffuse progressive systemic sclerosis than in CREST. In all diffuse progressive systemic sclerosis patients at least one method showed altered results, whereas half the CREST patients showed no pathological findings with both techniques. These results confirm a lower myocardial involvement in the CREST syndrome than in diffuse progressive systemic sclerosis and consequently this is probably related to a better prognosis.

Adult↗

PubMedDate not supplied

Gastrointestinal systemic sclerosis in serologic mixed connective tissue disease.

Mixed connective tissue disease is a clinical entity defined by overlapping features of progressive systemic sclerosis, systemic lupus erythematosus, polymyositis, rheumatoid arthritis, and distinct serologic findings. Esophageal dilatation and dysmotility have been the only gastrointestinal manifestations reported. Three patients with serologic findings of mixed connective tissue disease and extensive gastrointestinal involvement compatible with the changes found in progressive systemic sclerosis are presented. Gastrointestinal manifestations of progressive systemic sclerosis are reviewed and were found to be indistinguishable from the findings in these patients.

Adult↗

PubMed1988

Acute gouty arthritis in 4 patients with systemic sclerosis.

Gout with rheumatoid arthritis or systemic lupus erythematosus is rarely reported. There has been only one previous case report of gout with systemic sclerosis. We report 4 patients with chronic systemic sclerosis who developed acute gouty arthritis. Gout does occur in systemic sclerosis although the incidence is unknown. Synovial fluid analysis may be necessary to differentiate gout from the arthropathy of systemic sclerosis.

Acute Disease↗

PubMed2000

Development of systemic sclerosis in a patient with systemic lupus erythematosus and topoisomerase I antibody.

We describe a patient with systemic lupus erythematosus (SLE) associated with topoisomerase I (topo I, Scl-70) antibody, a specific marker for systemic sclerosis (SSc). SSc patients who produce this antibody have severe cutaneous and visceral involvement, and eventually have a poor prognosis. It is rare to find this antibody in patients with other collagen diseases. Only four SLE patients have been reported in the English language literature who were topo I antibody-positive but had no clinical evidence of scleroderma. The serum of our patient with SLE had precipitating topo I antibody from the onset of his disease. Twelve years after the onset of SLE, he developed proximal scleroderma and pulmonary fibrosis. This case reconfirms the prognostic significance of topo I antibody as a predictive marker, and indicates that SLE patients with topo I antibody require careful follow-up for future development of scleroderma.

Adult↗

PubMed2006

Pathophysiology and clinical consequences of Raynaud's phenomenon related to systemic sclerosis.

According to the so-called vascular hypothesis, Raynaud's phenomenon (RP) is one initial event in the pathophysiological cascade leading to sclerosis in systemic sclerosis (SSc). It is characterized by recurrent, reversible spasms of small arterioles and digital arteries, usually triggered by cold and emotional stress. Clinical signs of RP are a sudden pallor of single digits of fingers followed by reactive hyperaemia and in severe cases also by cyanosis. Besides imbalances between vasoconstrictive and vasodilatory processes, structural alterations of the involved vessels are fundamental to secondary RP in SSc. The latter is the reason why secondary RP in SSc, in contrast to primary RP, often leads to ischaemia and re-perfusion injuries. New insights into the pathophysiology of RP feature a special role for alpha2c-adrenoreceptors, Rho-kinase signalling pathways and soluble mediators. They have resulted in promising therapeutic options, including antagonism of endothelin receptors, inhibition of phosphodiesterases or selective blockade of alpha2c-adrenoreceptors. They should also have a positive impact on the course of SSc in general.

Fibrosis↗

PubMed1986

The cardiovascular manifestations of systemic sclerosis (scleroderma).

In conclusion, systemic sclerosis is both a fascinating and frustrating affliction. It is a systemic disease of multiple stages. Prognosis is dependent on the site and extent of visceral involvement. There is evidence to implicate the vascular system as the primary target organ of the disease. The cardiovascular manifestations include myocardial fibrosis, pericarditis, and a variety of arrhythmias and conduction abnormalities. Intractable heart failure or sudden cardiac death can ensue. Cardiac involvement in systemic sclerosis portends an ominous prognosis, and is probably most directly related to the extent of myocardial fibrosis which is present. The pathogenesis of myocardial fibrosis has not been determined, but it appears to be a result of an impairment of myocardial perfusion at both the small artery and microvasculature level. Obstructive, vasospastic, and devascularization factors all may be playing a role.

Blood Vessels↗

PubMed2001

[Postural changes in systemic sclerosis: preliminary results].

Systemic Sclerosis (SSc) is a generalized disease of unknown etiology which frequently presents neurological involvement of the central or peripheral nervous system. Besides various types of neuropathies, at times postural alterations of uncertain genesis can be found. Such alternations have never undergone organic study. The present work evaluates the static posture of subjects suffering from SSc without any subjective otoneurological symptoms. A total of 30 subjects-10 normal volunteers and 20 patients suffering from SSc-underwent full clinical examination, immunological testing (immunoglobulin; ANA; anti-ENA, anti-centromere, anti-SCL70, anti-DNA, anti-cardiolipin antibodies; C3, C4; circulating immunocomplexes) and capillaroscopy of the periunguis vallecula. The capillaroscopy data showed 2 distinct general pictures of sclerodermic microangiopathy: 1) a "slow pattern", not particularly aggressive, where capillarectasia and neoangiogenesis prevail; 2) an "active pattern", highly aggressive, where architectural disorder, capillary loss with the formation of avascular areas prevail. The 20 SSc subjects following the same therapy and free of any significant otoneurological, vasculo-metabolic or osteoarticular pathologies and the 10 healthy volunteers of compatible age and sex, underwent computerized Static Posturography (S.Ve.P-Amplaid). The immunological examinations showed that all patients were positive for ANA, 12 tested positive for anti-SCL70 antibodies and 7 for anti-centromere antibodies. The results of posturography showed a significant increase in parameter: "surface" and "standard deviation of velocity" with closed eyes. Spectra frequency analysis, processed using a personal method, showed a significant increase in panfrequency oscillations vs. the controls on both axes and both with eyes open and closed. The preliminary results of this study, which from reviewing the literature we believe to be the first of its kind performed, appear to indicate a subclinical suffering upon postural control in SSc: the alterations in the posturographic spectral frequency analysis indicate that all postural control mechanisms are extensively compromised, causing a pluriplanar, multifrequency destabilization both with and without the use of vision.

Adult↗

PubMed1976

The cardiac conduction system in progressive systemic sclerosis. Clinical and pathologic features of 35 patients.

Progressive systemic sclerosis may be associated with focal myocardial fibrosis. Electrocardiographic abnormalities including conduction block are common in progressive systemic sclerosis but whether they are due to direct destruction of the specialized conduction tissue of the heart is uncertain. The conduction systems of 35 patients with progressive systemic sclerosis were studied. Of these 35 patients, 17 (50 per cent) had myocardial fibrosis of the type seen in progressive systemic sclerosis. In 10 of the 17, it was severe. Sinus node fibrosis was present in 13 patients and was nearly as frequent in those with as in those without the progressive systemic sclerosis myocardial lesion. Overlying pericarditis may have contributed to the fibrotic changes within the sinoatrial nodes in seven of the 13 patients. The atrioventricular node and main His bundles were normal. However, fibrotic changes were found in the proximal bundle systems in six patients. In three of the six, severe myocardial progressive systemic sclerosis was present, two had focal fibrous atrophy of the left bundle, and one had complete interruption of the right bundle. In only the latter patient was this reflected in the electrocardiogram which showed a right bundle branch block. Three patients without progressive systemic sclerosis myocardial lesions also had fibrous atrophy of a portion of the proximal left bundle branch, and in one the electrocardiogram showed an isolated left anterior hemiblock. Thus, morphologic abnormalities within the conduction system in our patients are difficult to attribute to progressive systemic sclerosis per se. Furthermore, although conduction abnormalities were more frequent in patients with myocardial disease, specific conduction system disease was not the cause in most patients. As has been noted in ischemic heart disease, the conduction system appears to be relatively spared from the myocardial changes of progressive systemic sclerosis, and the high incidence of conduction disturbances in this condition may be a consequence, rather, of damage to working myocardium.

Adolescent↗