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 1,135 records · Page 63Linked to original sources
PubMed1981

Abnormalities of T lymphocyte subsets in patients with progressive systemic sclerosis (PSS, scleroderma).

Thirty-one patients with PSS (scleroderma) were examined for evidence of abnormalities in T lymphocyte subsets. TG and TM subpopulations of peripheral blood T lymphocytes were enumerated by rosetting techniques. The helper-suppressor functions of these lymphocytes were studied in the PWM-activated in vitro assay. The absolute numbers of TG cells in patients with DS as well as those with the CREST syndrome variant of PSS were decreased in comparison with those in age-matched healthy controls (p < 0.05). The numbers of TM and T "null" cells were not significantly altered (p > 0.05). The reduction in the number of TG cells was not caused by lymphocytotoxic antibodies. The mean helper-suppressor scores for patients with PSS and DS (1.25) and those with CREST syndrome (1.36) were higher (p < 0.05) than the mean score for healthy individuals (0.80). No relationship between the immunoregulatory abnormality and the presence or titer of CICs, ANAs, hypergammaglobulinemia, or various clinical manifestations of PSS could be demonstrated in individual patients. The finding of abnormalities in T lymphocyte subpopulations and the demonstration of their altered functional expression in patients with PSS indicate that immunoregulatory mechanisms may play an important role in this connective tissue disease.

Adolescent↗
PubMed1983

Progressive systemic sclerosis (PSS, scleroderma) dermal fibroblasts synthesize increased amounts of glycosaminoglycan.

Fibroblast monolayers were established by explant culture of dermis from seven patients with early, rapidly advancing PSS (scleroderma) and from six normal individuals. Net GAG synthesis was measured as [3H]glucosamine incorporation into the polymer and also as total uronic acid accumulation in the cultures. Cultures from patients with PSS accumulated up to fivefold more GAG than did normal cultures. Evidence suggests that this is a result of increased synthesis rather than decreased degradation. PSS cultures simultaneously accumulated increased amounts of CSP as well as GAG. Data obtained from studies of several monolayers, followed for as many as 10 generations, show that the difference between PSS and normal cultures can be propagated. The total quantity of GAGs was increased in PSS cultures, and there were differences in the distribution of individual GAGs on a percentage basis in PSS and normal cell cultures. GAG, as well as collagen, may be important in the development of connective tissue thickening and induration in scleroderma.

Adult↗
PubMed1977

Mixed connective tissue disease: the spectrum of radiographic manifestations.

Mixed connective tissue disease (MCTD) is a serologically distinct entity defined by a ribonuclease-sensitive extractable nuclear antigen. This unusual overlap syndrome has clinical features of scleroderma, systemic lupus erythematosus, polymyositis, and rheumatoid arthritis. In order to define the radiographic changes in MCTD, radiographs of the hands of 17 patients were studied, utilizing a fine-detail technique. Diffuse and periarticular osteopenia were found in 8 and 10 patients, respectively; soft-tissue swelling in 11; erosive changes in 9; joint-space narrowing in 7; tuft resorption and soft-tissue atrophy in 6; and subluxations in 2. In individual cases radiographs may appear normal or exhibit features of scleroderma, systemic lupus erythematosus or rheumatoid arthritis, thereby mirroring the clinical diversity of this entity.

Adolescent↗
PubMed1992

Altered regulation of collagen metabolism in scleroderma fibroblasts grown within three-dimensional collagen gels.

In systemic scleroderma (SSc) excessive deposition of collagen leads to fibrosis of various tissues including the skin. Previous studies have demonstrated that scleroderma fibroblasts in explant monolayer cultures are heterogeneous with respect to their levels of collagen synthesis. The critical role played by the extracellular matrix (ECM) in the modulation of fibroblast metabolism prompted us to study the regulation of collagen synthesis in scleroderma fibroblasts grown within three-dimensional collagen gels, a culture system representing more physiological conditions than monolayer cultures. Normal fibroblasts grown in this system dramatically reduce their collagen synthesis as compared to monolayer cultures. Quantification of total protein and collagen synthesis showed that scleroderma fibroblasts did not demonstrate the down regulation of collagen synthesis as observed in control fibroblasts, resulting in a much higher collagen synthesis in scleroderma fibroblasts compared to controls. However, also in this system scleroderma fibroblasts were heterogeneous in their response to the collagenous lattice. Ten strains were investigated, of which 3 were indistinguishable from controls, while 7 maintained higher levels of collagen production. In addition, our data showed that the changes in collagen synthesis on the protein level were accompanied by respective up- or downregulation on the mRNA level. These results indicate that an altered response to the surrounding ECM is an important factor in the disturbed regulation of connective tissue synthesis in scleroderma fibroblasts observed in vivo.

Adult↗
PubMed1994

Internal involvement in localized scleroderma.

We studied 76 consecutive patients with localized scleroderma (morphea with or without linear scleroderma) and analyzed the frequency, prognosis, and predictors of internal involvement in a subset of 53 patients systematically investigated for the presence of such involvement. Internal involvement was found by systematic examination in 16 patients. Only 2 of them, including 1 patient who developed systemic scleroderma, had symptomatic and severe visceral disease. The other 14 patients had asymptomatic and minor abnormalities consisting of abnormal lower sphincter pressure, and/or peristaltic failure in the esophagus and slightly impaired carbon monoxide diffusion in the lung. Frequent seroimmunologic abnormalities had no predictive value. Three parameters were found to be associated with internal involvement: male gender (p < 0.05), increasing number of plaque-like lesions (p = 0.02) and hypergamma-globulinemia at 1st examination (p < 0.005). Mild esophageal and pulmonary involvement are not rare in morphea but are usually silent. In our series, after a median follow-up of 48 months, such involvement did not impair the prognosis. The mildness of these visceral abnormalities suggests that they do not justify routine detection in asymptomatic patients. Morphea and systemic scleroderma behave as 2 different diseases.

Female↗
PubMed2002

[Clinical factors essential for dental caries intensity in rheumatic patients].

A total of 349 patients with rheumatoid arthritis, 129 with systemic lupus erythematosus, and 92 with systemic scleroderma were examined. A higher incidence of caries (CDL index) in comparison with the norm (p < 0.05) was observed in all patients; the most significant factor promoting caries development was involvement of the salivary glands presenting as Sjogren's syndrome (p < 0.005). The presence and severity of temporomandibular involvement virtually did not affect the intensity of caries (p > 0.1). The highest CDL index was observed in patients with rheumatoid arthritis and systemic scleroderma with pronounced dysfunction of the hand joints (p < 0.01) and higher activity of disease (a higher percentage of patients with Sjogren's diseases of the second and third degrees of activity) (p < 0.05). These data confirmed the multifactorial nature of dental caries etiology in rheumatic diseases.

Dental Caries↗
PubMed2006

[Classification criteria of scleroderma].

Classification criteria of scleroderma aim to delineate the different clinical forms of the disease, which are associated with different prognoses. The basic classification between localized and systemic scleroderma remains pertinent. Among the former, the problems are essentially esthetic or sometimes functional; but the second may yield many other problems which are pertinent to function but also to life, owing to the involvement of body organs, including lungs, heart, digestive tract, kidneys and locomotor system. Overall, the quality of life of scleroderma patients is often poor. The patients are subclassified according to the skin involvement. Those patients with systemic scleroderma involving the trunk are classified as "Cutaneous diffuse systemic scleroderma"; the association of Raynaud's phenomenon, capillaroscopic abnormalities and specific autoantibodies defines "limited systemic scleroderma"; among the latter patients, those with distal skin involvement are classified "Cutaneous limited systemic sclerosis". The old term "CREST" tends to be abandoned due to its lack of specificity.

Biomarkers↗
PubMed1993

[Colonic adenocarcinoma and scleroderma].

The association of cancer and connective tissue disease is well known, the most frequent being certainly with dermatomyositis. The association cancer and PSS is more controversial. The incidence of neoplasia in that group seems to be comparable with the general population but the proportion of certain types of cancer is different, and the temporal relationship with the apparition of symptoms of PSS is stunning. The hypothesis actually in favor is an imbalance of the immune system, which cause the diminution of the immune surveillance and the apparition of cancer and a concomitant dysregulation of the system, causing the fibrosis of the PSS. We describe a 75 year-old white female who developed a colic adenocarcinoma; she also had, concomitantly, a systemic scleroderma, with sclerodactyly and pulmonary fibrosis. The patient corresponded to the criteria of the American Rheumatism Association for progressive systemic scleroderma (PSS). The prognosis of patients with PSS depends on their systemic involvement but also, we believe, in the more aged group, on the apparition of a neoplasia.

Adenocarcinoma↗
PubMed1982

Immune response to connective tissue components of the basement membrane.

The immune response to connective tissue components of basement membrane (type IV collagen and laminin) and to interstitial collagen (type I) has been examined in human and murine systems. We also examined the role that immunologic sensitization to autologous connective tissue components might play in inducing an inflammatory response resulting in pathologic sequelae. Mice receiving a single subcutaneous injection of 5 micrograms type IV or type I murine collagens, or murine laminin in complete Freund's adjuvant mount a delayed-type hypersensitivity response characterized by a mononuclear cell infiltrate when challenged in the footpad with the sensitizing antigen. Cell-mediated immunity to these connective tissue antigens can be transferred to normal syngeneic mice with sensitized T-lymphocytes. In addition, repeated immunizations with these homologous connective tissue components elicit antibody responses in mice. Our data demonstrate the immunogenic nature of types IV and I collagen, and of laminin in a syngeneic murine model. We have demonstrated autoantibodies to the basement membrane and interstitial collagens in the sera of patients with scleroderma (systemic sclerosis); ELISA ratios correlate directly with the extent of pulmonary fibrosis in these patients. Anti-type IV collagen autoantibodies were found to be primarily IgM and anti-type I collagen antibodies, primarily IgG. An antibody response to autologous connective tissue antigens could lead to complement activation, immune complex formation, and deposition of the complexes along vascular endothelium with recruitment of blood monocytes in situ, mirroring the early scleroderma lesion (perivascular mononuclear cell filtrates). In vitro we examined the role of human peripheral blood mononuclear cells in the activation of fibroblasts. Adherent human blood monocytes release mediators which stimulate fibroblast proliferation and collagen deposition. A model is presented for the induction of immunity to autologous connective tissue components, leading to mononuclear cell inflammation, fibroblast activation and fibrosis. Selective immunity to basement membrane collagens may influence the clinical expression of diffuse connective tissue syndromes such as scleroderma (systemic sclerosis).

Animals↗
PubMed1984

Lung involvement in scleroderma.

Lung involvement (LI) was studied by lung function (LF) in 101 scleroderma patients (circumscribed scleroderma, n = 17; progressive systemic scleroderma [PSS], n = 84; with the subtypes I, acroscleroderma [n = 19]; 2, proximal ascending scleroderma [n = 61]; 3, trunk scleroderma [n = 4]). Eighteen percent of morphea, 32 percent of type 1, 56 percent of type 2, and 75 percent of type 3 patients had impaired LF. The LI was more frequent (57 percent vs 45 percent) and more severe (20 percent vs 3 percent) in PSS with systemic inflammation (form A) compared to those without (form B). Elevated lymphocytes/neutrophils in bronchoalveolar lavage (BAL) were found associated with form A and severe LI. The LF of patients showing an inflammatory cell pattern in initial BAL (n = 3) worsened, whereas those with normal BAL findings (n = 4) did not. Collagenase activity in BAL was significantly elevated in those with elevated lymphocytes/neutrophils in lavage. Patients with type 2 or 3 of PSS, especially form A, carry a higher risk of developing severe LI than circumscribed scleroderma, type 1, or form B patients. Differential cell count and collagenase activity in BAL is correlated with active disease and provides prognostic information.

Adolescent↗
PubMed1991

Use of angiotensin-converting-enzyme inhibitors in the management of renal disease.

The role of angiotensin-converting-enzyme inhibitors (ACEIs) in the treatment of progressive renal disease is described. Researchers have evaluated the use of ACEIs in patients with two types of progressive kidney disease: diabetic nephropathy and renal insufficiency associated with connective-tissue disease. When introduced early in the course of diabetic nephropathy, ACEIs appear to produce greater reductions in urinary protein concentrations than do other antihypertensive agents, even when systemic blood pressure does not decrease. They also slow the rate of decline of renal plasma flow and glomerular filtration rate. Results are less impressive when ACEIs are initiated at a later stage of disease. Captopril has been shown to be associated with a decline in serum creatinine level in patients with systemic scleroderma, and with reductions in blood pressure and increases in the glomerular filtration rate in patients with systemic lupus erythematosus. Because these patients were receiving other agents that might have influenced study outcome, specific conclusions about the efficacy of ACEIs in connective-tissue disease cannot yet be drawn. They may, however, be recommended as adjunctive agents in treatment of this condition. The relative safety of the ACEIs, combined with their ability to arrest or slow the progression of renal failure, makes them primary options in the management of selected renal diseases.

Angiotensin-Converting Enzyme Inhibitors↗
PubMed1987

[Intestinal scleroderma: clinical aspects, diagnostic possibilities and therapy].

Progressive Systemic Scleroderma (PSS) is a generalized disease of the connective tissue, caused by vascular changes, an increased collagen synthesis as well as inflammatory and immunological phenomena. Manifest intestinal scleroderma is a rare complication. The connection of internal symptomatology with esophagus attack is of practical importance. The bacterial deconjugation of bile acids is of pathogenetic relevance, regarding the release of an intestinal symptomatology. This results in a direct application of Tetracycline or Metronidazole by parenteral administration. The increase of unconjugated serum bile acids, such as cholic acid and chenodeoxycholic acid, gives a diagnostic indication which can be carried out in special laboratories by means of gas-chromatography.

Bile Acids and Salts↗
PubMed1992

Lung involvement in systemic sclerosis sine scleroderma treated by plasma exchange.

Systemic sclerosis sine scleroderma can present in some patients as pulmonary interstitial fibrosis. Until now ten cases with this particular clinical variant, all men, have been reported in the literature. The knowledge of systemic sclerosis sine scleroderma presenting as lung interstitial involvement is important in clinical practice for an early diagnosis and correct therapeutic strategy. This work reports the clinico-serological features of two further cases, one a woman, of systemic sclerosis sine scleroderma with prevalent lung involvement, and describes the effects of therapeutic plasma exchange.

Female↗