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Results for “Scleroderma, Systemic”

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PubMed1996

[Systemic scleroderma and sarcoidosis: 3 new cases].

We observed 3 patients with successive scleroderma (SS) and (what is considered to be) sarcoidosis (SA). The diagnosis SS included in the 3 patients: Raynaud's syndrome with pulpal necrosis and capillaritis, sclerodactylia and acro-osteolysis, multiple joint pain and FAN+. Also observed were: esophagus involvement (n = 3), pulmonary artery hypertension (n = 1), telangiectasia (n = 2) and anti-Scl 70 (n = 2). Initially, all patients had restrictive pulmonary disease. SS was diagnosed 5 to 9 years prior to SA in 2 patients. Diagnosis of SA was based on the following arguments: Loëfgren's syndrome with erythema nodosa (n = 1), parotiditis (n = 2), sicca syndrome (n = 2), myalgia (n = 2), joint involvement (n = 2), non-infectious pluropericarditis (n = 2), epitheloid and giant cell granulomas without caseous necrosis (lung = 3, liver = 1, lymph nodes = 1, salivary glands = 1, synovia = 1), negative search for bacilli, elevated conversion enzyme (n = 1) and, in each case, by the lack of any other cause. One patient died from lung cancer and another from respiratory failure. Nome of the patients had primary biliary cirhosis. This rare association between SS and SA demonstrates the confluent limits of certain systemic diseases and raises a difficult problem to differentiate pulmonary involvement in these diseases. The gravity of this localization and the poor sensitivy to corticosteroids.

Adult↗
PubMed1999

[Scleroderma--systemic sclerosis. Serology, lung function and survival].

Patients with systemic sclerosis (SSc) were studied with regard to the presence of antinuclear antibodies (ANA) and their clinical correlates (n = 230), pulmonary function (n = 176), and mortality and causes of death (n = 344). ANA were found in 85%. Anti-centromere antibodies were found in 34%, anti-Scl-70 antibodies in 13% and anti-U1-RNP antibodies in 6.5%. These serological groups were associated with limited SSc, diffuse SSc, and myositis/arthritis, respectively. The most prevalent finding at first lung function test was isolated reduction of diffusion capacity (47%). Further deterioration of diffusion capacity was related to the presence of anti-centromere antibodies and increased sedimentation rate. The standardized mortality rate (SMR) was 2.9, higher in young patients (SMR = 13) and patients with diffuse SSc (SMR = 4.5).

Adolescent↗
PubMed1987

[Update on the treatment of systemic scleroderma].

Renewed clinical trials of drugs used for many years and the availability of new symptomatic therapies allows selection of appropriate treatment for progressive systemic sclerosis. Colchicine damages intracytoplasmic microtubules and when administered at a dose of at least 1.5 mg per day during early stages of the disease reduces cutaneous sclerosis without improving other manifestations of scleroderma. D-penicillamine inhibits bridge formation during collagen maturation, acting mainly on cutaneous infiltration with more uncertain effects on visceral localizations of the disease. It can be effective against pulmonary lesions but, as with colchicine, must be prescribed early. Its action is delayed and its side effects limit its use. Corticoids are of very limited efficiency and although they may be useful for relief of muscular and articular localizations they have been accused of precipitating onset of renal insufficiency. Immunodepressants have until now been assessed as ineffective. The demonstration in patients with progressive systemic sclerosis of abnormal activation of immunity system cells suggested the use in this collagen disease of a new immunosuppressive agent, cyclosporine A, but clinical utility of this drug remains to be demonstrated. In systemic sclerosis efficacy of plasma exchange is mainly directed against vasomotor disorders and digital ulcerations, but study results are difficult to assess because of associated therapies. Constraints and risks of this treatment also considerably reduce its interest. The coagulation factor XIII acts as a stabilizing factor of collagen. It reduces cutaneous infiltration but its efficacy is based on results of a single controlled trial and its use is limited by the need for repeated intravenous injections.(ABSTRACT TRUNCATED AT 250 WORDS)

Humans↗
PubMed2004

Pellucid marginal degeneration and scleroderma.

Systemic scleroderma is a progressive multi-system connective tissue disease. Ocular involvement includes keratoconjunctivitis sicca, progressive shallowing of conjunctival fornices, peripheral ulcerative keratitis and eyelid tightness. No association has been reported between scleroderma and pellucid marginal degeneration, which is a rare bilateral corneal ectasia. Pellucid marginal degeneration is characterised by non-inflammatory and progressive peripheral corneal thinning inferiorly, often with high against-the-rule astigmatism. We report a case of a 55-year-old woman with systemic scleroderma who presented with rapidly progressing against-the-rule astigmatism. The differential diagnosis of peripheral corneal thinning and the challenge of the surgical management of pellucid marginal degeneration are briefly discussed.

Astigmatism↗
PubMed1990

[Anti-Scl-70 antibodies in systemic scleroderma].

We looked for anti-Scl-70 and anti-centromere antibodies in 109 patients (26 men and 83 women). Mean age was 43 +/- 15 years. Forty patients had systemic sclerosis according to ARA criteria. The extension of cutaneous involvement was defined by using Barnett and Coventry criteria: 12 patients were type I (sclerodactyly), 20 type II (acrosclerosis) and 8 type III (diffuse scleroderma). Among the 12 patients with type I, there were 8 cases of CREST syndrome defined as follows: presence of sclerodactyly, Raynaud's phenomenon and 2 of the 3 following criteria: oesophageal dysmotility, calcinosis, telangiectasia. Other organ involvement was recorded. Control patients had idiopathic Raynaud's phenomenon (n = 22), other connective tissue diseases (n = 20), and miscellaneous diseases (n = 28). Ninety-nine patients were prospectively included in this study. Patients' sera were stored at -20 degrees C. Ten previously stored sera obtained from patients with systemic sclerosis were also analyzed. Immunological tests were performed simultaneously and with no information on the diagnosis. When antinuclear antibodies were detected by indirect immunofluorescence, double immunodiffusion and immunoblotting were performed. Anti-Scl-70 antibodies were detected in systemic sclerosis only: 1 of 12 type I, 11 of 20 type II and 4 of 8 type III. One serum negative by immunodiffusion was positive using immunoblotting. We found that the specificity of anti-Scl-70 antibodies for systemic sclerosis was 100 p. 100 and their sensitivity 40 p. 100. There was a correlation between the presence of anti-Scl-70 antibodies and the presence of antinuclear antibodies (p less than 0.05) and the extent of cutaneous involvement (p less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed2006

Is occupational exposure to solvents associated with an increased risk for developing systemic scleroderma?

BACKGROUND: Our study was aimed to investigate in a German collective if there are any hints for an increased occupational or environmental risk to develop systemic sclerosis, especially, focussing on work-related exposure to solvents. Moreover, we tried to evaluate the feasibility of a sampling method addressing support groups. METHODS: A standardised questionnaire was published in two journals subscribed by members of two different support groups and all members were asked to complete the questionnaire and to return it anonymously. The subjects were not informed on the scientific hypotheses, nor did they know who of them belonged to the case group (scleroderma) or to the control group (multiple sclerosis). RESULTS: 175 questionnaires could be included in the statistical analysis. As expected, a female predominance was in our collective. In the male subpopulation, the occupational exposure to solvents was higher in the case group than in the control-group (70% versus 45.8%). Based only on the male subgroup, a tendency for an association between occupational exposure to solvents and the risk to develop systemic sclerosis was found. CONCLUSION: According to our experience in this case-control-study exposure misclassification, qualitative or quantitative, was an eminent problem. Within such a setting, it is generally very difficult to establish an exact dose-response relationship due to incomplete, imprecise or missing data concerning duration of exposure, frequency of use and kind of solvent. Additionally, a well-known problem in studies based on self-reported questionnaires is the so-called volunteer bias. Unfortunately, but similar to other studies assessing epidemiologic factors in such a rare disease, our study was of limited power, especially in the subgroups defined by gender.

Journal Article↗
PubMed1984

[Digestive localizations of scleroderma].

Systemic scleroderma involves the gastro-intestinal tract in over 50 p. 100 of cases, the commonest target organs being the oesophagus, the small intestine, the colon and the stomach in that order. The G-I symptoms of this collagenosis are all related to disorder of motility secondary to disturbances of innervation and then to atrophy of the smooth muscle and fibrous infiltration. Oesophageal involvement results in gastro-oesophageal reflux and/or dysphagia due to the lack of tonicity of the lower oesophageal sphincter and a reduction of peristalsis. Disease of the small intestine may cause pseudo-intestinal obstruction or a secondary malabsorption syndrome due to abnormal intraluminal bacterial flora. Colonic involvement causes severe constipation with formation of faecoliths. Finally, scleroderma may be complicated by an acute abdominal syndrome: occlusion due to diffuse reduction in small intestinal motility, peritonitis due to perforation of the small intestine, ileo-colonic infarction, gastro-intestinal haemorrhage complicating telangiectasia. Treatment is purely symptomatic: classical remedies for gastro-oesophageal reflux and its complications, and antibiotics for malabsorption syndromes.

Anus Diseases↗
PubMed2002

Systemic sclerosis-scleroderma.

Systemic sclerosis is a clinically heterogeneous, systemic disorder which affects the connective tissue of the skin, internal organs and the walls of blood vessels. It is characterized by alterations of the microvasculature, disturbances of the immune system and by massive deposition of collagen and other matrix substances in the connective tissue. This review discusses epidemiology and survival, clinical features including subsets and internal organ involvement, pathophysiology and genetics, microvasculature, immunobiology, fibroblasts and connective tissue metabolism and environmental factors. Early diagnosis and individually tailored therapy help to manage this disorder, which is treatable, but not curable. Therapy involves immunomodulation as well as the targeting of blood vessel mechanics and fibrosis. Physical therapy and psychotherapy are also important adjunctive therapies in this multifactorial disease.

Animals↗
PubMed2001

Familial occurrence frequencies and relative risks for systemic sclerosis (scleroderma) in three United States cohorts.

OBJECTIVE: To determine the frequency with which scleroderma (systemic sclerosis; SSc) recurs in families and the familial relative risk (lambda) in the US. METHODS: Family histories of SSc were prospectively surveyed in 3 large US cohorts of SSc patients, 2 in Texas and 1 in Michigan. Diagnoses of familial SSc were verified by rheumatologist evaluation and/or review of medical records. Familial relative risks for first-degree relatives (lambda1) and siblings (lambdas) were calculated using actual reported counts of first-degree relatives in 2 cohorts and recent estimates of SSc prevalence in the US. RESULTS: Compared with the estimated prevalence of SSc in the US (2.6 cases/10,000 population [0.026%]), the disease occurred in 1 or more first-degree relatives in 1.5-1.7% of SSc families in the 3 cohorts (or 11 of 703 families [1.6%]), a significant increase. Familial relative risks in first-degree relatives in the 3 cohorts ranged from 10 to 16 (13 combined), and in siblings they ranged from 10 to 27 (15 combined). CONCLUSION: SSc occurs significantly more frequently in families with scleroderma (1.6%) than in the general population (0.026%). A positive family history of SSc is the strongest risk factor yet identified for SSc; however, the absolute risk for each family member remains quite low (<1%).

Black People↗
PubMed2002

Increased circulating concentrations of the counteradhesive proteins SPARC and thrombospondin-1 in systemic sclerosis (scleroderma). Relationship to platelet and endothelial cell activation.

OBJECTIVE: To determine whether circulating concentrations of the counteradhesive proteins SPARC (secreted protein acidic and rich in cysteine) and thrombospondin-1 (TSP-1) are elevated in scleroderma (systemic sclerosis, SSc). The relationship of these counteradhesive proteins to measures of platelet and endothelial cell activation was examined. METHODS: Plasma from 45 patients with SSc (26 limited form, 19 diffuse) and 22 age and sex matched controls was assayed for SPARC, TSP-1, beta-thromboglobulin (betaTG), and platelet factor 4 (PF4), 2 distinct platelet a-granule products, and soluble E-selectin, a marker of endothelial cell activation. RESULTS: The mean (+/- SE) SPARC concentration was greater in patients with limited SSc (124.0 +/- 9.6 ng/ml) compared to controls (66.8 +/- 8.0 ng/ml) (p = 0.0005), whereas in patients with diffuse SSc (74.1 +/- 7.9 ng/ml) it was not. Elevated SPARC concentrations in the limited SSc group could not be ascribed to either platelet or endothelial cell activation. TSP-1 concentrations were also increased in SSc patients (n = 29) compared to controls (n = 11) (2.98 +/- 0.12 vs 2.4 +/- 0.21 log transformed ng/ml; p < 0.02). Unlike SPARC, TSP-1 concentrations correlated with both betaTG (r = 0.57, p = 0.0014) and PF4 (r = 0.41, p = 0.026) levels, indicating that increased TSP-1 could, in part, be explained through elevated platelet a-granule release in SSc patients. Plasma levels of betaTG, PF4, and E-selectin were each similarly elevated (p < 0.003) in patients with both limited and diffuse SSc compared to controls. CONCLUSION: That circulating SPARC and TSP-1 are elevated in patients with SSc raises the possibility that counteradhesive proteins, which regulate vascular organization and remodeling, might contribute to the pathogenesis of SSc vasculopathy.

Adult↗
PubMed1990

[Antinuclear, anticentromere and anti-ScL-70 antibodies in rheumatic diseases].

Methods are described that are used for the titration of antinuclear, anticentromere, and anti-Scl-70 antibodies in systemic scleroderma, systemic lupus erythematosus, and rheumatoid arthritis: indirect immunofluorescence with various antigenic substrates (sections of fresh-frozen rat liver and Hep-2 cell culture), counter-current immunoelectrophoresis, isolation of Scl-70 antigen. Use of Hep-2 cells as a substrate for indirect immunofluorescence was found clinically and diagnostically more effective since it permitted the detection of anticentromere antibodies and anti-Scl-70. Nucleolar, mottled, homogeneous, marginal immunofluorescence types were observed when rat liver sections and Hep-2 cells were used for substrates. Anticentromere antibodies and anti-Scl-70 were isolated significantly more frequently in systemic lupus erythematosus or rheumatoid arthritis.

Antibodies, Antinuclear↗