SEARCH · PubMed Health

Results for “Scleroderma, Diffuse”

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 163 records · Page 9Linked to original sources

PubMed1990

[Histological study on localized scleroderma].

To define stage-specific and type-specific histological findings in morphea, 21 (14 plaque, 2 linear, and 5 en coup de sabre type) patients were examined. A) Fibrotic changes; 1) Every case had the fibrotic change of various degrees. 2) Nodular sclerotic fibrosis was found in 7 cases with morphea, namely in 5 of 11 cases with a plaque type and 2 of 4 en coup de sabre type morphea. And the above mentioned 7 cases were within two years since the onset, and no nodular fibrosis was found in the morphea with the longer duration than two years in history. Nodular fibrosis was located in the middle or lower dermis adjacent to the typical sclerotic dermis. It was strongly suggested that this nodular fibrosis is as an initial change of localized scleroderma. 3) Nodular fibrosis expanded to the neighboring area and made a typical histological feature of morphea which is completely different from that of diffuse scleroderma. B) Inflammatory findings; 1) Inflammatory changes were divided into, a perivascular, a diffuse or non-perivascular, and a mixed type. 2) The pure perivascular type was found in 10, the pure diffuse type only in one, the mixed type in 6 cases, and the other 4 cases had no inflammation. 3) Marked or moderate inflammation was found in cases with short history of the disease except for one case. 4) Inflammatory cells in the morphea were mainly composed of lymphocytes and histiocytes, but occasionally of plasma cells in 11 of 17 cases. C) Pigmentary changes; Incontinentia pigmenti was found in 18 of 21 cases.

Adolescent↗

PubMed1995

Variability of skin scores and clinical measurements in scleroderma.

OBJECTIVE: To determine the variability of several clinical outcome measurements commonly used in scleroderma clinical trials. METHODS: Ten researchers, members of a multicenter placebo controlled trial of methotrexate in early diffuse scleroderma, studied the intraobserver and interobserver variability of variables used to assess efficacy in scleroderma trials. RESULTS: For most measures, the variability within an observer was less than that found between observers, and therefore the intraobserver reliability was better than the interobserver reliability. The reliability of the modified Rodnan skin score exceeded the Rodnan skin score. Measures with inherent interpretation such as global assessments and skin scores had more variability than easily performed measurements such as grip strength and oral opening. CONCLUSION: Some of our variability was higher than variability previously reported; this could be due to the large number of examiners and patients in our study.

Female↗

PubMed1990

[Platelet function study in primary Raynaud's phenomenon and Raynaud's phenomenon associated with scleroderma].

We evaluated platelet function in 59 patients with Raynaud's phenomenon (RP): 24 had primary RP (PRP) and in 35 RP was associated with diffuse scleroderma (DS). In the group with PRP there were 10 males and 14 females, with a mean age of 43 +/- 12 years and a time of evolution of 5 +/- 5 years. In the group with RP associated with DS there were 31 females and 4 males with a mean age of 53 +/- 12 years and a time of evolution of 9 +/- 7 years. The control group consisted of 20 healthy individuals (14 males and 6 females with a mean age of 40 +/- 12 years). In all patients and controls beta-thromboglobulin (BTG) and platelet factor 4 (PF4) levels were measured in plasma, and platelet aggregation was evaluated in the presence of adenosine diphosphate (ADP), collagen and arachidonic acid. The patients with RP associated with DS had BTG and PF4 higher than those with PRP and controls (p less than 0.02). The BTG/PF4 ratio was also significantly greater in patients with DS (p less than 0.005). The platelets from patients with DS had a greater aggregation with ADP (1 microM and 0.5 microM) than those from PRP (p less than 0.03). We concluded that patients with RP associated with DS had in vivo activation and a greater aggregation of platelets, in contrast with the absence of these findings in the group with PRP and in controls.

Adult↗

PubMed1983

D-penicillamine treatment of progressive systemic sclerosis (scleroderma): a comparison of clinical and in vitro effects.

Punch biopsies of skin were obtained from the forearms of 3 patients with progressive systemic sclerosis with diffuse scleroderma before and after treatment for 1 year or more with D-penicillamine. While on therapy, each patient studied had demonstrated a marked reduction in skin thickening. Collagenase-sensitive protein and glycosaminoglycan accumulation were measured in fibroblast cultures derived from these biopsies. No differences were observed pre- and post-treatment. We conclude that although D-penicillamine may exert its effect in vivo on connective tissue synthesis, maturation and/or turnover, fibroblasts remaining in the thinned dermis retain their potential for increased synthesis of connective tissue.

Adult↗

PubMedDate not supplied

[Telangiectasias in progressive systemic sclerosis (generalized scleroderma). Observation on 120 cases].

120 patients with progressive systemic sclerosis (PSS) were studied and subdivided into five groups according to the PSS classification of Giordano et al. ("acute diffuse scleroderma", "intermediate syndrome", "acrosclerosis sensu stricto", "sclerodactylia" and "sclerosis sine scleroderma"). In all five subgroups telangiectasias incidence was high (from 75% to 100%). The "ramosus" and "telangiectatic mats" types occur more frequently than other forms of telangiectasias. The former particularly involves the face, neck and chest; whereas the latter more usually involves the upper extremities and is the only type which appears at the lips. Cuticular telangiectasia is a third type, as important as the others but less frequent. The incidence of telangiectasias is related to disease duration. Similar telangiectasias have been observed in rarer patients with other connective tissue diseases (SLE, RA, dermatomyositis and undifferentiated connective tissue diseases).

Arthritis, Rheumatoid↗

PubMed1979

Skin thickness and collagen content in progressive systemic sclerosis and localized scleroderma.

Skin biopsies of uniform location and surface area (7 mm diameter) were obtained from the extensor aspect of the forearm of 147 patients with progressive systemic sclerosis (PSS) (107 with diffuse scleroderma, 40 with the CREST syndrome variant) and 58 individuals with normal skin. After careful removal of all subcutaneous fatty tissue, the skin cores were weighed and their water and hydroxyproline content determined. Despite recent claims to the contrary, it was found that there is a marked and highly significant increase in the thickness of the skin during the indurative phase of PSS, and that this is associated with a proportionate increase in total dermal collagen content. A similar degree of thickening was found in the skin of patients with eosinophilic fasciitis and acromegaly. A close correlation was observed between clinical estimation of the degree of skin thickening and the weight of the skin biopsy cores. Change in the weight of skin cores was observed during the course of illness of the patients with PSS and may serve as a useful measurement of alteration in the degree of skin thickening.

Acromegaly↗

PubMedDate not supplied

[Studies of the mechanism of disorders of the natural killer cell activity in patients with systemic scleroderma].

The NK activity of peripheral blood mononuclear cells (PBMC) was evaluated in 90 patients with various subsets of systemic scleroderma (SSc). The NK activity, as performed with K-562 as target cell, was found to be significantly lowered in patients with diffuse scleroderma, but did not differ from the healthy control in patients with acrosclerosis. The lowest values in the NK activity assay were obtained in patients with most extensive skin involvement and severe internal organ changes. The NK activity of healthy donors' PBMC was significantly decreased by addition of SSc patients PBMC (50:1) to the cytotoxicity assay, but was not influenced by the patients sera.

Cytotoxicity, Immunologic↗

PubMed1989

[The Scl-70 antibody and its clinical significance].

In the review of the literature on the Scl-70 antigen, methods of detection of the Scl-70 antibody, the correlation between its presence and the clinical, genetic and immunological parameters are presented. It is emphasized that the Scl-70 antibody which is regarded as a marker of diffuse scleroderma, may have a prognostic significance. In indirect immunofluorescence on HEp-2 cells the Scl-70 antibody gives a characteristic pattern described as diffuse fine speckles with dotted nucleolus. A somewhat different but distinct staining pattern on hamster liver described by the authors was found to be characteristic of that antibody. The fluorescence associated with Scl-70 antibody is designated as nucleolar-reticular mixed staining with nucleolus predominating. It is suggested that hamster liver substrate which is easily available may replace the expensive HEp-2 cells for detecting Scl-70 antibody.

Antibodies, Antinuclear↗

PubMed1997

Scleroderma autoantigens are uniquely fragmented by metal-catalyzed oxidation reactions: implications for pathogenesis.

The observation that revelation of immunocryptic epitopes in self antigens may initiate the autoimmune response has prompted the search for processes which induce novel fragmentation of autoantigens as potential initiators of autoimmunity. The reversible ischemia reperfusion which characterizes scleroderma has focused attention on reactive oxygen species as molecules which might induce autoantigen fragmentation. We demonstrate that several of the autoantigens targeted in diffuse scleroderma are uniquely susceptible to cleavage by reactive oxygen species, in a metal-dependent manner. Multiple features of the fragmentation reaction and its inhibition indicate that these autoantigens possess metal-binding sites, which focus metal-catalyzed oxidation reactions (and consequent fragmentation) to specific regions of the antigens. These data suggest that the autoantibody response in scleroderma is the immune marker of unique protein fragmentation, induced by ischemia reperfusion in the presence of appropriate metals, and focus attention on abnormal metal status as a potential pathogenic principle in this disease.

Autoantigens↗

PubMed1989

Progressive systemic sclerosis: Part 1--The disease and medical management.

The incidence of scleroderma is approximately 700,000 people in the United States. Pathologic changes occur in a variety of body systems and can alter lifestyle, especially for people with diffuse scleroderma. Information from clinical practice and research concerning the nature, incidence, pathophysiology, complications, and medical management is discussed. Although there is no known cure for scleroderma, life expectancy has improved as management of renal hypertensive crisis and renal failure has improved.

Education, Nursing, Continuing↗

PubMed1989

[The PM-Scl (polymyositis-scleroderma) autoantibody and its nucleolar fluorescence pattern].

By indirect immunofluorescence studies of antinuclear antibodies on hamster liver imprints as a substrate performed to determine the nuclear staining pattern of sera from patients with connective tissue diseases 10 sera showed a distinct homogeneous nucleolar staining pattern associated with weaker speckled or homogeneous nucleoplasmic fluorescence. In all 10 cases the antibodies revealed the PM-Scl specificity in immunodiffusion. Clinically 9 patients had an acrosclerosis, in 56% overlapped with symptoms of polymyositis. Only one patient had a diffuse scleroderma. The homogeneous nucleolar immunofluorescence pattern of PM-Scl should be distinguished from mixed nucleolar and diffuse reticular nucleoplasmic pattern of Scl-70.

Antibodies, Antinuclear↗

PubMed1993

Systemic sclerosis (scleroderma) associated with discoid lupus erythematosus.

Six patients with systemic sclerosis (SS) and discoid lupus erythematosus (DLE) were studied to determine whether such cases have some common clinical and laboratory findings. DLE preceded SS in all cases. Three patients had diffuse scleroderma with lung and esophagus involvements and the others limited scleroderma. Three patients had anti-topoisomerase-I and antiribonucleoprotein antibodies, 2 had either of them and the remaining anticentromere antibodies. Four had DLE located on the scalp, leading to alopecia. The other 2 had DLE on the face and extremities. No case fulfilled criteria for systemic lupus erythematosus (SLE). The present cases with SS and DLE, but without SLE, indicate that this type of systemic-cutaneous collagen disease overlap does exist and may be not so rare.

Adult↗

PubMed1992

Lower extremity amputation in scleroderma.

Scleroderma or Systemic Sclerosis (SSC) is a disorder characterized by fibrosis of the skin and multiple internal organs. The pathological lesion is a triad of small artery intimal proliferation, medial thinning and adventitial scarring. Autoamputation of fingers and toes is often seen, but only a few cases of limb amputation in scleroderma patients have been reported. The Pittsburgh Scleroderma databank includes 1,030 patients with SSC. Among these were seven patients who sustained lower limb amputation. There were four patients with the CREST variant of SSC, two with diffuse scleroderma, and one who had SSC/rheumatoid arthritis/polymyositis overlap who sustained limb amputation. Of the seven, three were male and five had a significant smoking history. Ages ranged from 46 to 71 years. All patients underwent amputation for nonhealing ulcerations. No problems with postoperative wound healing were seen. Pathologic changes typical of SSC in addition to atherosclerotic peripheral vascular disease were described in one case. Three patients were successfully fitted with prostheses and became independent ambulators. Four patients could not be fitted with prostheses. No skin problems were reported related to prosthetic use. Our review demonstrates that SSC patients who undergo amputation can become successful prosthetic users and should be considered for prosthetic prescription.

Aged↗

PubMed1996

Cardiac involvement in scleroderma.

In summary, cardiac involvement in systemic sclerosis can be manifested as myocardial disease, pericardial disease, conduction system disease, or arrhythmias. Clinical cardiac involvement is a poor prognostic factor. Asymptomatic cardiac abnormalities are frequent, and all cardiac abnormalities are seen more often in diffuse scleroderma. Unlike other organs, the role of vascular involvement is unclear. At present, treatment of cardiac scleroderma is essentially symptomatic and empiric. The role of vasodilation and immunosuppression needs further exploration.

Arrhythmias, Cardiac↗

PubMed1988

Comparison between counterimmunoelectrophoresis and double radial immunodiffusion in the detection of antibodies to topoisomerase I in sera from scleroderma patients.

Sera from 146 patients with progressive systemic sclerosis have been submitted to the detection of anti-Scl 70 antibodies by both double radial immunodiffusion (ID) and counterimmunoelectrophoresis (CIE). CIE detected a significantly higher number of positive sera from patients with diffuse scleroderma, while no difference between the two techniques was noted in sera from patients with CREST variant. CIE increases the diagnostic importance of the serologic investigation in systemic sclerosis and gains a better distinction between subgroups of patients with different clinical expressions of this disease.

Autoantigens↗

PubMed1990

Early detection of connective tissue disease in patients with Raynaud's phenomenon.

Connective tissue diseases such as scleroderma frequently show an insidious onset. In their early stages a diagnosis of a specific CTD is hard to make, and the disorder may then be designated as "undifferentiated CTD." Raynaud's phenomenon (RP) is the first symptom in many cases and may precede the disease for many years. RP, however, is a common finding in the population, especially in young females. Thus, only a minority of patients with RP will develop a CTD. For reason of prognosis and early diagnosis and to get more insight in the initial pathophysiological processes, it is important to know which patients with RP will develop or are already evolving into a CTD. Patients referred to the clinician because of RP frequently (24-40%) show signs or symptoms of CTD, especially of scleroderma. Pulmonary function disturbances and esophageal hypomotility are asymptomatic in many cases, and should be sought for with sensitive methods. How can we distinguish patients with truly primary RP from those who will evolve into CTD (and thus should be screened for CTD and be followed)? The presence of antinuclear antibodies (ANA) and abnormalities at nailfold capillary microscopy (NCM) have proven to be early indicators of evolution into CTD, especially scleroderma and related disorders. The antigenic specificities of ANA indicate which syndrome the patient will develop, e.g., anti-CENP-B indicates the CREST-syndrome and anti-topoisomerase I diffuse scleroderma. Other factors of prognostic significance in patients with RP are age at onset and severity of RP: older age at onset and a highly severe RP represent risk factors for CTD. What do we know about the evolution of RP into CTD? Follow-up studies on patients referred because of RP have shown that some 15-20% of these patients have an insidious progress to limited cutaneous scleroderma including CREST. Risk factors have already been mentioned. From a pathophysiological point of view, studies in patients with early scleroderma have proved that microvascular changes are crucial in the disease process. These changes, demonstrated morphologically by NCM and functionally, e.g., by pulmonary function studies, are also reflected in increased levels of VIII antigen and in in vivo platelet activation. Both of these phenomena probably result from endothelial damage. There is increasing evidence that T-cells and their products are involved in vascular damage in early scleroderma. Future research should be directed to the elucidation of the target antigens for these T-cells.

Antibodies, Antinuclear↗

PubMed1990

Functional and phenotypic analysis of T lymphocytes cloned from the skin of patients with systemic sclerosis.

Activated T lymphocytes often accumulate in the lower dermis of patients with systemic sclerosis (scleroderma) and may play a role in the development of dermal fibrosis. We propagated and cloned these cells directly from skin biopsies in four of eight cases of early, untreated systemic sclerosis with diffuse scleroderma. The cloning frequency estimates were f = 0.20 and f = 0.48 for T cells derived from the skin of two patients versus f = 0.68 and f = 0.96 for autologous blood T lymphocytes. All but one of 24 skin-derived scleroderma clones were CD4+. Clonal analyses performed with CD4+ clones from patients and normal controls showed that all but one skin-derived clones synthesized either interferon-gamma (60%), glycosaminoglycan-stimulatory factor (26%) or both (9%) when induced in vitro by a mitogen, concanavalin A, but not by autologous dermal fibroblasts. In contrast, blood-derived clones had a different functional phenotype. All skin-derived clones produced tumour necrosis factor-alpha. Our results demonstrate that T lymphocytes obtained from the skin of patients with systemic sclerosis synthesized cytokines which could modulate functions of human dermal fibroblasts.

Adult↗

PubMed1982

[Anticentromere antibodies. Diagnostic and prognostic value].

The results of investigations for antinuclear antibodies in scleroderma and other connective diseases are reported. An indirect fluorescence method on Hep-2 cell smears was used. Antibodies directed against chromosomal centromeres were detected in 25 cases: 19 sera of patients with diffuse scleroderma, 2 cases of Raynaud's disease, 2 cases of rheumatoid arthritis and 1 case each of lupus and dermatomyositis. A significant correlation was found between the presence of anticentromere antibodies and benign forms of scleroderma, notably the CREST syndrome.

Antibodies, Antinuclear↗