PubMed Health⌕ Search

SEARCH · PubMed Health

Results for “Scleroderma, Systemic”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 739 records · Page 41Linked to original sources
PubMed2006

Systematic review: pathophysiology and management of gastrointestinal dysmotility in systemic sclerosis (scleroderma).

Gastrointestinal dysmotility in systemic sclerosis (scleroderma) is prevalent in 90% of patients, increasing morbidity and in some cases mortality. The resultant gastrointestinal complications are usually extensive, involving many regions of the gut from the oesophagus to the anus. Collagen replacement of vascular and enteric smooth muscle results in hypomotility, lumen dilatation, tensile rigidity and eventual loss of organ functions. The aim of this paper is to provide an overview of systemic sclerosis-related gastrointestinal dysmotility and available/potential therapeutic options. We evaluated published data on the pathophysiology and management of gastrointestinal dysmotility in systemic sclerosis patients using the MEDLINE database for English and non-English articles from 1966 to July 2005. Based on this systematic review, lifestyle and medical therapy approaches are preferred as they often improve and/or ameliorate symptoms. Surgery is only recommended with serious, rare complications such as bowel perforation or ischaemia. Alternative therapies such as acupuncture-based therapies are well tolerated, with clinical improvement and may be of potential therapeutic benefit for systemic sclerosis gastrointestinal dysmotility. Further elucidation of initiating and persistent mechanisms of systemic sclerosis-related gastrointestinal dysmotility will optimize the development of a multidisciplinary and more directed treatment regimen.

Colon↗
PubMed2002

Scleroderma in children.

Juvenile systemic scleroderma (jSSc) is a rare childhood disease. In the review period covered within this article, there were few reports devoted exclusively to it. In the past year, there was no breakthrough regarding pathogenesis, classification, or treatment. In the pediatric field, the proposed classification system for jSSc shows significant progress toward a definition of the pediatric population. It should allow better evaluation of this patient group. In adult systemic scleroderma, European efforts to establish indices for disease activity have been published. They should help to assess the disease activity in a standardized way and therefore enable earlier adequate treatment for patients. Biologic markers and new assessment methods for particular organ involvement help to determine disease activity/severity as well. There is still no effective disease-modifying treatment for systemic scleroderma and jSSc. The summarized data of the phase I/II autologous stem cell transplantation trial have been published. The first guidelines for autologous stem cell transplantation for jSSc are proposed. In organ-specific treatment, the introduction of bosentan, a dual endothelin receptor antagonist, to control pulmonary hypertension is a real gain in the therapeutic options. New methods to assess prognosis are evolving and need to be validated in a larger patient population.

Adolescent↗
PubMed2002

[How to recognize scleroderma].

Systemic sclerosis is a multisystem disorder characterized by the presence of connective tissue sclerosis, vascular abnormalities concerning small-sized arteries and the microcirculation, and autoimmune changes. This disease is usually preceded by Raynaud's phenomenon. Cutaneous changes are useful for diagnosis. Sclerosis is initially proeminent on hands with frequently pitted scars. Telangiectases and calcium deposits are mainly observed in a good prognosis subset of the disease (CREST) characterized by the association of limited skin involvement and anti-centromere antibodies with possible late onset of pulmonary hypertension. The bad prognosis subset is associated with diffuse cutaneous sclerosis, pulmonary fibrosis, gastrointestinal disease, myocardial involvement and anti-topo-isomerase I antibodies. Histological skin changes are similar to those observed in morphea. In morphea, there is only localized sclerosis of the skin with highly variable aesthetic disability. The condition may be subdivided clinically into the following types: circumscribed plaques, linear morphea and frontoparietal lesions. The last two forms may be associated with atrophy of the underlying anatomic structures. Many other diseases may have a scleroderma-like appearance. In these diseases, nail folds abnormalities, visceral involvement and immunological changes are usually different from those observed in systemic scleroderma.

Diagnosis, Differential↗
PubMed1978

Immunopathology of the renal vascular lesion of progressive systemic sclerosis (scleroderma).

Patients with progressive systemic sclerosis (PSS, scleroderma) exhibit a variety of immunologic abnormalities. To verify whether the renal vascular lesions of such patients might be mediated by an immunologic mechanism, kidney tissues of 16 patients with PSS were investigated by means of fluorescence, light, and electron microscopy; elution of tissue-bound antibody; and fixation of heterologous (guinea pig) complement. Controls consisted of 12 nonsclerodermatous patients with similar levels of hypertension with no evidence of associated immunologic abnormalities. Diffuse vascular deposits of immunoglobulins (predominantly IgM) and/or complement (predominantly Clq) were found in all 16 patients with PSS. These deposits were bound to the intima of intralobular and arcuate arteries which, by light microscopy, often exhibited typical fibromucinous alterations. Elution of antibody and heterologous complement fixation studies suggested that such reactants may represent the interaction of complement-fixing antibody and antigen. Electron microscopies studies demonstrated abundant fibrillar and ground substance material in the arterial intima but features of deposited (circulating) immune complexes were not found. By contrast, in the hypertensive (control) group, deposits of immunoglobulin (s) and/or complement were rare and, when present, were mostly confined to the arterioles. As judged by the results of elution and heterologous complement fixation, these arteriolar deposits appeared to represent trapped rather than specifically bound serum proteins. The possible signficance of these findings are discussed in relation to immunologic mechanisms which might be implicated in the pathogenesis of the renal vascular disease of PSS.

Adult↗
PubMed2004

Reliability and validity of the Duruoz Hand Index in persons with systemic sclerosis (scleroderma).

OBJECTIVE: To examine the test-retest reliability and concurrent validity of the Duruoz Hand Index (DHI) in persons with scleroderma. METHODS: Forty patients with scleroderma and no other major medical problems completed the DHI at 2 points in time to assess test-retest reliability. To assess validity, participants were administered the Arthritis Hand Function Test (AHFT), the Hand Mobility in Scleroderma Test (HAMIS), and the Keital Function Test (KFT), all performance-based tests. In addition, participants completed the Health Assessment Questionnaire (HAQ), a self report of functional ability. RESULTS: Test-retest reliability intraclass correlation coefficients for the DHI ranged from 0.81 to 0.97. Scores on the DHI did not correlate with HAMIS scores, but were significantly correlated with scores from the KFT (rs = 0.48, P < 0.01), HAQ (rs = 0.79, P < 0.01), and all sections of the AHFT (rs = 0.34-0.60, P < 0.05-0.01). CONCLUSION: The results from this study show the DHI to be a reliable and valid test for persons with scleroderma.

Activities of Daily Living↗