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Results for “Scleroderma, Systemic”

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PubMed1988

Main manifestations of carditis in diffuse connective tissue diseases in children.

238 children with diffuse connective tissue diseases were clinically examined with the use of echography. The presence was documented of characteristic morphofunctional changes in the heart in collagen diseases. Characteristic haemodynamic changes (predominantly contraction disturbances in systemic lupus erythematodes and dermatomyositis, and relaxation disturbances in systemic scleroderma) were identified.

Child↗

PubMed1976

Pneumatosis intestinalis in association with connective tissue disease.

Pneumatosis intestinalis in association with connective tissue diseases is an unusual combination whose pathogenesis is not yet understood. Furthermore, steroid medication, often used to treat these diseases, may itself cause pneumatosis. Three cases of scleroderma, systemic lupus erythematosus, and amyloidosis in association with pneumatosis and without prior steroid therapy are presented. The small vessel occlusive pathologic processes in these diseases may cause focal areas of mucosal ischemia resulting in small, perhaps transient ulcerations that allow gas to enter the gut wall from the lumen.

Adult↗

PubMed1999

Sera from patients with scleroderma inhibit fibroblast micromotions monitored electrically.

OBJECTIVE: To investigate scleroderma fibroblast behavior, and the effect of different human sera on fibroblast behavior, we established a model using the newly developed electrical biosensor, electrical cell-substrate impedance sensing (ECIS). METHODS: Cellular locomotion, defined as cellular micromotion, measured by ECIS indicates the dynamic vertical motion of a given group of cells. The junctional resistance (Rb) between adjacent cells and the average height (h) between basal cell surface and substratum derived from ECIS were quantified for dermal fibroblasts obtained from patients with scleroderma and normal controls. The cellular micromotions of both scleroderma and normal control fibroblasts were compared in the presence of different human sera, including those from patients with scleroderma, systemic lupus erythematosus (SLE), and Sjögren's syndrome (SS) and from normal individuals. RESULTS: The Rb and h levels for scleroderma and normal fibroblasts were 2.7 omega.cm2, 150 nm and 0.8 omega.cm2, 303 nm respectively. The micromotions of scleroderma fibroblasts were more active than those of normal fibroblasts. Sera from patients with scleroderma can inhibit the micromotions of normal fibroblasts but not those of scleroderma fibroblasts, while sera from patients with SLE and SS have no inhibitory effect on either normal or scleroderma fibroblast micromotions. CONCLUSION: We have demonstrated the previously unrecognized characteristics of dermal fibroblasts and sera derived from patients with scleroderma. It is possible that in vivo activities cause scleroderma fibroblasts to display active cellular micromotions, while sera from patients with scleroderma inhibit the micromotions of normal fibroblasts. The use of ECIS technology has also provided a new approach to the study of scleroderma.

Aged↗

PubMed1998

[Scleroderma in a hospital setting in Togo].

The purpose of this retrospective study was to determine the epidemiological and semiological profile of scleroderma in Black Africa. Medical records of patients who consulted in the Dermatology Clinic and the Rheumatology Clinic of the Teaching Hospital in Lomé, Togo between 1980 and 1996 and between 1989 and 1996 respectively were reviewed. Scleroderma was diagnosed in 18 of the 34,169 patients seen during the study periods (0,05%). The disease was systemic in 13 cases (8 females, 5 males; mean age: 31 years) and localized in 5 cases (mean age: 20 years). There were no predisposing occupational or therapeutic factors. The clinical manifestations were Raynaud's phenomenon in six of the 13 patients with systemic scleroderma. Extensive cutaneous sclerosis in 10 cases, poikiloderma in seven cases, dysphagia in three cases, pulmonary fibrosis in four cases, and polyarthralgia in two cases. Myalgia and renal involvement were not observed. The findings of this study documents the low incidence of scleroderma in Togo. The high frequency of extensive cutaneous sclerosis, poikiloderma and low frequency of Raynaud's phenomenon which were often mild are the most remarkable features of the disease in Lomé. These features have been pointed out previously but epidemiologic data is still insufficient to confirm the existence of an African form of scleroderma.

Adolescent↗

PubMed1988

[Quantitative assessment of the status of patients with rheumatic diseases].

A system of operative quantitative assessment of the status of patients with rheumatoid arthritis (RA), osteoarthrosis (OA), systemic lupus erythematosus (SLE) and systemic scleroderma (SSD) was developed using a mathematical model of discriminant analysis. Data contained in case histories of 120 patients with RA, 112 with OA, 106 with SLE and 63 with SSD were used to determine discriminant coefficients of linear discriminant equations of some functional systems: locomotor, cardiovascular, respiratory, renal, digestive, nervous, skin and subcutaneous fat, immunological as well as laboratory findings on disease standing. Knowing discriminant coefficients, one can determine an index in any values of functional indicators that characterize the status of one or another system in any phase of the treatment of a patient. The system can be used in practice in a rheumatological hospital using CM-1600 minicomputer.

Arthritis, Rheumatoid↗

PubMed1986

Interstitial lung disease in scleroderma. Immune complexes in sera and bronchoalveolar lavage fluid.

Interstitial lung disease is a common feature of scleroderma (systemic sclerosis), and it may be a major determinant of morbidity and mortality. Analysis of bronchoalveolar lavage fluid from patients with scleroderma has shown evidence of inflammation in the lower respiratory tract of many patients. We have analyzed sera and bronchoalveolar lavage fluid from scleroderma patients for the presence of immune complexes, which may play a role in the inflammatory process. Using a solid-phase C1q enzyme-linked immunosorbent assay, we detected immune complexes in the sera of 6 of 23 patients (26%) and in none of 32 controls (P less than 0.01). All 6 patients with serum immune complexes had inflammatory cells in bronchoalveolar lavage fluid, and the presence of serum immune complexes correlated with the percentage of neutrophils in lavage fluid (P less than 0.02). Immune complexes were detected in lavage fluid of 11 of 21 patients (52%) compared with 1 of 7 normal controls (14%). Subjects having immune complexes in lavage fluid had a lower forced vital capacity than did those without lavage fluid immune complexes (P less than 0.05). The levels of immune complexes in bronchoalveolar lavage fluid exceeded those in serum by a mean of 45-fold, suggesting either local formation or selective deposition of immune complexes in the lower respiratory tract of some scleroderma patients.

Adult↗

PubMed1996

Computerized evaluation of skin stiffening in scleroderma.

Modern non-invasive biometrological methods have proven to be helpful in monitoring some skin disorders. The present study was designed to evaluate the mechanical properties of skin in systemic scleroderma. The Cutometer SM 474 was used to measure the rheological properties of the skin in 33 patients suffering from scleroderma. The most prominent abnormality consisted in a marked reduction in skin extensibility. Changes occurring in time objectivate the evolution of the disease. Measuring the mechanical properties of skin in patients with scleroderma may be useful in monitoring the disease and its treatment.

Adult↗

PubMed2006

Phototherapy: a promising treatment option for skin sclerosis in scleroderma?

In systemic sclerosis (SSc; scleroderma) fibrosis of the skin can lead to considerable morbidity. No significant improvement has been reported from studies investigating antifibrotic therapies so far. In dermatology, phototherapy with ultraviolet (UV) irradiation is successfully used for treatment of several diseases because of its anti-inflammatory as well as immunosuppressive mechanisms, and its low-risk profile. In addition, the UVA spectrum in particular exerts antifibrotic effects as it leads to reduction of procollagen synthesis and expression of collagenase-1 in vitro. Accordingly, treatment with long-wavelength UVA-1 irradiation or photochemotherapy with UVA plus the photosensitizer psoralen (PUVA) have been successfully used to reduce skin fibrosis in localized scleroderma (morphea). There are only in particular few reports on treatment of skin sclerosis in SSc, but the results are in concordance with the good experience that have been observed at our and other dermatological centres. Phototherapy is able to stop or inhibit the fibrotic processes and to induce softening of sclerotic skin, especially in limited SSc. Phototherapy thus represents a therapeutic alternative for antifibrotic treatment with a low rate of adverse effects, which should be applied before the sclerotic process has proceeded too far.

Fibrosis↗

PubMedDate not supplied

What did we learn by studying scleroderma fibroblasts?

Systemic sclerosis (SSc, scleroderma) is a complex disease manifesting itself by fibrosis of skin and other internal organs. Fibroblasts isolated from SSc lesions and cultured in vitro are characterized by the increased synthesis of collagen and other extracellular matrix (ECM) proteins, consistent with the disease phenotype. Cultured SSc fibroblasts therefore serve as a principal experimental model for studying the molecular and cellular mechanisms involved in collagen overproduction in this disease. This article provides an overview of transcription factors that are deregulated in SSc fibroblasts. The possible origin of SSc fibroblasts is also discussed.

Collagen↗

PubMed1976

Pathogenesis and staging of scleroderma.

Scleroderma is reviewed as a complex disease of vascular, connective-tissue, and inflammatory reactions. If the scleroderma syndromes related to occupational, immunologic, inflammatory, metabolic, and genetic factors are recognized, then true progressive systemic scleroderma (PSS) appears to be a disease of vascular fibrosis with secondary inflammatory phases. The primary pathology appears to be a fibromucinous change of the vascular endothelium. The vessels also demonstrate altered reactivity to cold and to serotonin. Biopsy of the skin of the inflammatory or mesenchymal scleroderma may reveal (by positive direct immunofluorescence) deposition of globulins at the basement membrane zone. Evaluation of scleroderma is discussed and a system of clinical staging of PSS is proposed which relates the organ involvement to the prognosis.

Calcinosis↗

PubMedDate not supplied

[Acupuncture in treatment of inflammatory rheumatic diseases].

Seventeen studies were examined with regard to efficacy and scientific quality of acupuncture in rheumatoid arthritis, spondarthropathy, lupus erythematosus, local and progressive systemic scleroderma. Acupuncture cannot be recommended for treatment of these diseases. By far, the most studies examined failed to show sufficient quality.

Acupuncture Therapy↗

PubMed1983

[Periungual capillaroscopy in children with scleroderma and dermatomyositis].

The capillary microscopy is of high value for the early diagnosis of scleroderma: the pattern of megacapillaries is classical in adults. The authors describe the original observation of ramified capillaries in children's with systemic scleroderma (two cases) and dermatomyositis (one case). Such observations lead them to discuss several hypothesis regarding pathogeny (neo-angiogenesis), prognosis and diagnosis of children connectivities.

Adolescent↗

PubMed1979

Scleroderma: increased biosynthesis of triple-helical type I and type III procollagens associated with unaltered expression of collagenase by skin fibroblasts in culture.

To assess potential abnormalities in collagen metabolism in systemic scleroderma, skin fibroblast lines from patients with this disease were established and compared to control cell lines derived from healthy subjects. For studies on the biosynthesis of procollagen, the cells were incubated with [(14)C]proline in a medium supplemented with ascorbic acid and beta-aminopropionitrile, and the synthesis of nondialyzable [(14)C]hydroxyproline, in relation to DNA or cell protein, was taken as an index of procollagen formation. Five of eight scleroderma fibroblast cell lines demonstrated procollagen biosynthesis rates significantly higher than the controls, and the mean rate of procollagen synthesis by scleroderma fibroblasts was about twice that of the control cells. Control experiments demonstrated that the specific activity of the intracellular free proline was not different in scleroderma and control fibroblasts, and the mean population doubling times of the scleroderma and the control fibroblast cell lines were the same. The relative synthesis of the genetically distinct procollagens was examined by isolating type I and type III procollagens from the cell culture medium using DEAE-cellulose chromatography. The ratios of type I/III procollagens in scleroderma cell lines did not differ from the controls. The helical stability of the collagenous portion of type I and type III procollagens, estimated by the resistance of (14)C-collagen to limited proteolytic digestion with pepsin under nondenaturing conditions, was the same in both scleroderma and control cultures. The capacity of the cells to synthesize enzymatically active and immunologically reacting collagenase was also studied; no marked differences in these parameters could be observed. The results suggest that cultured skin fibroblasts from patients with scleroderma demonstrate a metabolic abnormality expressed as increased synthesis of type I and type III procollagens in a normal ratio. This abnormality may play a role in the excessive accumulation of collagen in the skin and other organs affected in scleroderma.

Adolescent↗

PubMed1989

[Spectrum of antinuclear and anti-cytoplasmic antibodies in dermatomyositis and polymyositis overlap syndromes].

Antinuclear antibodies (ANA) of the IgG class were detected in 60% of patients with dermatomyositis. Only in 16% could we demonstrate precipitating antibodies against the nuclear Mi-2 antigen. These antibodies are considered to be serological markers of dermatomyositis. The ANA spectrum in polymyositis-overlap syndrome was considerably more heterogeneous: patients with polymyositis/progressive systemic scleroderma overlap had antibodies against the nucleolar PM-Scl antigen or the nuclear Ku antigen. Cytoplasmic antibodies to Jo-1 were present in polymyositis associated with Sjögren's syndrome and pulmonary fibrosis. Antibodies against ribosomal ribonucleoprotein were found in polymyositis with systemic lupus erythematosus and antibodies to nuclear ribonucleoprotein in polymyositis associated with mixed connective tissue disease. The investigation demonstrates that the characterization of ANA specificities helps to differentiate between dermatomyositis and distinct forms of polymyositis-overlap syndrome.

Adult↗

PubMed2005

Scleroderma in Australian aborigines.

Scleroderma (systemic sclerosis) has not been reported before in Australian Aborigines. We describe in detail a community middle-aged Aboriginal woman whose diffuse scleroderma terminated fatally with a renal crisis. Moreover, we have identified a further five Aboriginal patients on the South Australian Scleroderma Register (two with diffuse, two with limited and one with overlap scleroderma), a number consistent with that expected from the 2001 census data for our state. However, an analysis of all antinuclear antibody (ANA) requests from the Top End of Australia over a 6-year period revealed only two Aborigines with low titre anticentromere antibody (despite frequent occurrence of ANA with other specificities). Neither of these Aborigines had features of scleroderma. In conclusion, scleroderma does occur in indigenous Australians but further studies are needed to confirm the apparent infrequency of centromere-associated limited scleroderma (which is the commonest form of scleroderma in our Caucasian population).

Antibodies, Antinuclear↗

PubMed2003

Stem-cell transplantation for autoimmune diseases.

The use of intensive immunosuppressive treatment coupled with BM stem-cell transplantation (SCT) to treat human autoimmune diseases (AID) follows anecdotal observations of responses of AID to allogeneic SCT and an extensive background of experience with SCT in animals with AID. In the last decade, numerous clinical trials have been initiated to explore a potential benefit of (mainly autologous) SCT in advanced and debilitating cases of rheumatoid arthritis, scleroderma, systemic lupus erythematosis and multiple sclerosis. In this review the etiology of AID and the experimental basis of SCT is presented, together with recent clinical results of SCT for AID. While much has been learned about the risks and benefits of SCT in AID, the underlying mechanisms regulating remission and relapse of AID after treatment remain largely unknown.

Autoimmune Diseases↗

PubMed2006

[The clinical analysis of pulmonary arterial hypertension in connective tissue disease].

OBJECTIVE: To investigate the clinical features and prognosis of pulmonary arterial hypertension (PAH) secondary to connective tissue disease (CTD). METHODS: Eighty-two patients diagnosed as having CTD associated PAH (CTDaPAH) were retrospectively analyzed among 2189 patients with CTD in Peking Union Medical College Hospital between January 1997 and September 2004. RESULTS: Eighty-two (3.7%) patients (75 females and 7 males), aged 41 years, were found to have CTDaPAH. The underlying diseases included mixed CTD (MCTD), systemic scleroderma, primary Sjogren's syndrome (pSS), systemic lupus erythematosus (SLE), undifferentiated CTD, dermatomyositis and Behcet disease. PAH was an earlier complication in patients with SLE or MCTD (median onset, 3 and 2 years, respectively), compared to those with pSS (6 years). The most common clinical manifestations of CTDaPAH were dyspnea on exertion (84.1%) and Raynaud's phenomenon (56.1%). The mean pulmonary artery systolic pressure (PASP) level of these patients was (65.71 +/- 20.44) mm Hg, with the decrease of PaO2 and PaCO2 level [(70.37 +/- 15.02) mm Hg, (27.88 +/- 6.46) mm Hg, respectively]. The diffusing capacity of the lungs measured using carbon monoxide was decreased [D(L)CO pred% (51 +/- 14)%]. 13%, 32%, 29% and 8% of these patients were grouped to class I, II, III and IV, respectively, on NYHA functional classification. Although the patients received treatment for underlying CTD and traditional vasodilators, the condition of elevated PASP had not been improved with the exception of a marginal decrease of PASP in SLE patients [(76.47 +/- 18.20) mm Hg --> (69.08 +/- 20.77) mm Hg]. Thirteen (15.85%) patients died during an average of 4.33 years follow-up, compared to the mortality of those with non-CTDaPAH (2.75%, P < 0.01). Lower level of PaO2 [(58.51 +/- 16.16) mm Hg] and higher proportion of NYHA class III, IV [38.46% (5/13), 30.77% (4/13), respectively] were observed in death group compared to survivors [PaO2 (73.25 +/- 14.32) mm Hg; NYHA class III, IV 34.78% (24/69), 5.80% (4/69), respectively] (P < 0.01; P < 0.05; respectively). CONCLUSIONS: PAH is a common complication of CTDs, which occurs often in the forth year after initial CTD manifestations and is earlier in patients with SLE or MCTD, compared to those with pSS. The most common manifestations of CTDaPAH are dyspnea on exertion and Raynaud's phenomenon. CTDaPAH could be very severe, which will lead to lower level of PaO2 and more advanced NYHA classification, and therefore reduce the survival rate of the patients. Doppler echocardiography and lung function test are necessary to detect PAH early.

Adolescent↗

PubMed1993

Familial scleroderma: HLA antigens and autoantibodies.

We report clinical and serological findings as well as the results of extended (HLA-A, B, C, DR and complotype) haplotype determinations of a family with two cases of systemic scleroderma and one case of primary biliary cirrhosis and incomplete CREST syndrome in a sibship of eight. In addition, one of these eight siblings has showed immunological findings of autoimmune disease for years but has not developed clinical symptoms. This family was studied by Soppi et al. in 1982; one member of the family has since then developed primary biliary cirrhosis and incomplete CREST type scleroderma. All family members with scleroderma or related disease as well as their sister with immunological abnormalities share the A2; B8; DR3 haplotype. Also some members of the family share the same haplotype but have remained healthy. This haplotype seems to be a predisposing factor but additional genetic or environmental factors are probably necessary for expression of autoimmune disease.

Adult↗