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Results for “Scleroderma, Diffuse”

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PubMed1977

[Successive connective tissue diseases (scleroderma, then lupus). Clinical and biological study of 4 cases].

The authors present 4 patients who had suffered for several years from isolated systemic scleroderma and in whom, after 3, 10, 19 and 22 years, clinical, biological and histological signs of DLE appeared. There was no current further progression of the scleroderma in two cases, at the time of onset of DLE. In the oter two cases, it had disappeared. All four patients had a serum cryoglobulin. In all cases the kidneys were involved histologically : extra membranous glomerulitis in one, segmental glomerulitis in a second, and diffuse progressive glomerulitis in the last two. The authors analyse the biological changes which accompanied the clinical transformation. One of the patients had anti-ribonucleoprotein antibodies. She died from severe renal involvement which would be against the good prognosis usually associated with the presence of this type of antibody.

Adult↗
PubMed2007

Peripheral nervous system lesion syndromes and the mechanisms of their formation in connective tissue diseases.

Systemic rheumatological diseases are often accompanied by the development of central and peripheral nervous system pathology. Data providing evidence of the high incidence of peripheral nervous system lesions in systemic lupus erythematosus and systemic scleroderma are presented. These diseases in particular are characterized by polyneuropathies and tunnel syndromes. Our own observations, along with published data, revealed the following major pathogenetic mechanisms of peripheral nervous system lesions in diffuse connective tissue diseases - ischemic, immunological, and metabolic. Consideration of these mechanisms will lead to pathogenetically based treatment and improved therapeutic outcomes.

Connective Tissue Diseases↗
PubMed2001

D-penicillamine is not an effective treatment in systemic sclerosis.

Based on open studies. D-penicillamine (DPA) has been used for the treatment of systemic sclerosis (SSc) but we believe the controlled trial of this drug in SSc does not support its use to treat this disease. Open trials are inevitably biased by selection bias and randomized, blinded, controlled studies are required to minimize both known and unknown confounding variables. The high vs. low dose DPA trial was a well-controlled, randomized, double-blind study which met criteria for a high quality study, although it was not placebo-controlled. Toxicity was increased in the high dose group, thus showing a biologic response, although the study showed no clinical efficacy differences between a mean dose of 120mg DPA every other day (equivalent to 60mg qd) and a mean dose of 822mg DPA daily. One might argue that 60mg DPA is effective, but we believe this is highly unlikely, as doses significantly higher than this have been shown to be ineffective in other connective tissue diseases such as rheumatoid arthritis. In our opinion. D-penicillamine is, unfortunately, ineffective in treating early, diffuse, systemic sclerosis.

Antirheumatic Agents↗
PubMed2006

Scleroderma: health services utilization from patients' perspective.

OBJECTIVE: To evaluate utilization of the health care system by patients with scleroderma by determining which physicians are diagnosing and following patients, what tests are being used, and what is the time to diagnosis, as measured over the past 3 decades. METHODS: A self-administered questionnaire (available in English and French) was mailed up to twice to 1,437 members of 12 provincial chapters of the Scleroderma Society of Canada. RESULTS: The overall response rate was 63%. Eighty-nine percent of respondents were female. Sixty percent were between the ages of 30 and 59 years. Forty-three percent were diagnosed by a rheumatologist. Among patients with diffuse disease, 90% have been followed by a rheumatologist; however just over half of patients have seen a gastroenterologist (54%), cardiologist (51%), respirologist (67%), and less than half have seen a dermatologist (42%), nephrologist (13%), physiotherapist (46%), or occupational therapist (34%). The mean time to diagnosis over the last 3 decades is 2.4 years. At diagnosis less than 50% of patients had an electrocardiogram, echocardiogram, gastroscopy, thorax CT, or skin thickness measurements, whereas over 50% of patients had a chest radiograph and pulmonary function testing. CONCLUSION: Less than half of patients were diagnosed by a rheumatologist, and time to diagnosis from onset of symptoms has remained unchanged over the last 3 decades. Despite their complex, multisystemic disease, less than 50% of patients see sub-specialists or had baseline screening tests for organ involvement of their systemic sclerosis. Further research is needed on health services utilization and on determinants of access to care by patients with scleroderma.

Adult↗
PubMed1982

Antinuclear antibodies as immunologic markers for a benign subset and different clinical characteristics of scleroderma.

Antinuclear antibody (ANA) test results were correlated with the clinical status of 56 patients with systemic scleroderma. Three groups were identified. (1) The speckled pattern represented a benign clinical subset. Acrosclerosis, Raynaud's phenomenon, calcinosis, and esophageal dysmotility characterized this group. None of these patients had pulmonary, renal, or cardiac disease. (2) Two patterns and ANA-negative test results were associated with a different incidence of certain clinical characteristics. The thready pattern was associated with pulmonary involvement. Diffuse skin involvement and Raynaud's phenomenon were found with the nucleolar pattern. Patients with ANA-negative test results had the most severe disease, including renal failure. (3) Two patterns were not associated with different clinical characteristics. These were the small speckle-like thready pattern and the homogeneous pattern. This study supports the theory that ANA patterns may be used as immunologic markers for different clinical characteristics of patients with scleroderma as they have already been used in lupus erythematosus.

Antibodies, Antinuclear↗
PubMed2006

[Systemic sclerosis].

Systemic sclerosis (SSc; syn. systemic scleroderma) is a rare autoimmune disorder with characteristic cutaneous manifestations. Prevalence in women is fivefold higher than in men. The course of the disease is slowly progressive with a variable degree of internal organ involvement due to fibrosis and obliteration of small vessels. The diffuse form shows more frequent and severe organ manifestations compared to the limited form. Increased mortality is particularly related to a cardiopulmonary involvement leading to a 5-year survival of around 75%. Treatment indications are dependent on the severity of the disease. In the acute state, immunosuppressive agents are needed in case of significant organ involvement. Vasodilative drugs are often used for the symptomatic treatment of Raynaud's phenomenon.

Autoantibodies↗
PubMed1992

[The clinical significance of detecting the inhibition of topoisomerase I by the sera of patients with systemic scleroderma].

Topoisomerase I activity was studied by electrophoresis in agarose gel according to plasmid DNA relaxation. Sera from 62 patients with systemic scleroderma, 35 with Raynaud's syndrome, 8 with focal scleroderma, 15 with systemic lupus erythematosus, 20 with rheumatoid arthritis and 20 healthy subjects were examined. Out of 62 sera from SSD patients, anti-topoisomerase activity was found in 67.8% of cases. The test appeared positive in 79% of patients with diffuse and 63% with limited disease patterns. The mean age and disease standing were similar in the positive and negative groups. An increase of the skin count and more frequent occurrence of trophic disorders in patients with inhibition of the enzyme were recorded. 40% of the patients demonstrated the coincidence of the results with the use of the topoisomerase test and ELISA. In patients with other rheumatic diseases and in the healthy subjects, no inhibition of the enzyme was found.

Animals↗
PubMed1995

Comparison of three commercially available enzyme immunoassays for the screening of autoantibodies to extractable nuclear antigens.

The initial screening test used in the diagnosis of connective tissue diseases is based on the detection of antinuclear antibodies (ANA) by indirect immunofluorescence (IFA). When the ANA screen is positive, it is often useful to determine the specificity of the autoantibody to a series of extractable nuclear antigens (ENA), a procedure that has been classically performed by double immunodiffusion. Testing large numbers of clinical specimens for autoantibodies to ENA by double diffusion techniques can be time-consuming and expensive. ENA screening systems that employ enzyme immunoassay (EIA) technology have recently become commercially available. We compared three EIA ENA assays to classic Ouchterlony double diffusion techniques. Furthermore, the sensitivity of each antigen and methodology (including ANA immunofluorescence using HEp-2 cells) was tested using ENA positive sera possessing single autoantibodies. Two of the three EIAs that detected immunoglobulin G type autoantibodies to Smith (Sm), ribonucleoprotein (RNP), Sjögren's syndrome-associated antigens Ro (SSA) and La (SSB), were provided by INOVA and Sigma Diagnostics. A third EIA, which also included scleroderma-associated antigen 70 (SCL-70/DNA-topoisomerase I) and histidyl-tRNA synthetase (Jo-1) in addition to the four previously stated antigens, was provided by Clark Laboratories. This latter ENA screen detected IgG, IgA, and IgM type autoantibodies. Included in the study were sera covering a wide variety of anti-nuclear and other autoantibodies. Sensitivity was 100% for all EIA ENA screens when compared to Ouchterlony double diffusion and specificity exceeded 95% in each case. Sensitivity studies showed Ouchterlony to be less sensitive than EIA when detecting low levels of autoantibodies to ENA.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1989

[HLA-A, B, C phenotype frequencies in progressive systemic scleroderma].

In 41 patients suffering from progressive systemic sclerosis (PSS) the phenotype frequencies of 28 HLA-A, B, C antigens were determined. Compared to 2947 blood donors the PSS patients exhibited an increase in the HLA-B8 frequency (37% versus 19%). This difference revealed to be not significant after the correction according to the number of antigens tested. The same was with the increase in the frequency of HLA-B5, B13, B27, Cw2 and the decrease in the frequency of HLA-A2, B12, B14, B15, B4 and Cw3. The results were compared with the data reported in the literature. Additionally, there were no differences when compared the diffuse PSS with the acral form and CREST, respectively.

Adolescent↗