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Results for “Scleroderma, Systemic”

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PubMed1994

[Tramadol in the vascular pains of patients with systemic scleroderma and other rheumatic diseases].

A study was made of the effect of tramadol on vascular pains in 20 patients with systemic scleroderma and other rheumatic diseases, using a visual analogue scale. In eleven patients, ischemic and ulceronecrotic lesions were at the basis of the painful syndrome. In this case the pains were most severe. In five patients, the lesions were of vascular and neuromuscular character. 50% of the patients demonstrated a good effect, 35% a satisfactory one, and 10% had a complete analgesia. Tramadol was administered in a daily dose of 100 to 200 mg, mainly per os. It is recommended that tramadol may be used in patients with the painful syndrome of vascular genesis.

Adolescent↗

PubMed1975

Initial change of glycosaminoglycans in systemic scleroderma.

7 clinically uninvolved as well as 8 involved (6 moderately, 2 markedly) back or forearm skin specimens from 12 patients with systemic scleroderma were subjected to quantitative evaluation and to qualitative analysis of glycosaminoglycans (GAG) by one-dimensional and two-dimensional cellulose acetate electrophoresis. Skin specimens from the back, clinically uninvolved but histologically demonstrating the initial change, revealed increased amounts of hyaluronidase, chondroitinase-resistant GAG of varying electrophoretic mobilities, and one of them was chemically confirmed to be heparan sulfate variant, whereas involved skin specimens showed hardly this increase.

Adult↗

PubMed1994

Long-term thymopentin treatment in systemic scleroderma.

A study was carried out to investigate whether thymopentin treatment is capable of inducing changes in the immunological status of patients with systemic scleroderma and to compare any such changes with modifications in clinical condition. Nine patients were given thymopentin, 1 ml in 10 ml saline solution, by slow intravenous infusion 3-times weekly for 5 weeks (cycle). The cycles were repeated at 3-month intervals. Treatment duration ranged from 1 to 5 years. Blood samples were drawn at the beginning and at the end of each cycle and the patients' lymphocytic sub-populations were examined. A control group of 9 comparable healthy subjects were similarly tested. Data analysis showed that a statistically significant decrease of CD16+ and CD25+ lymphocytes compared to pretreatment was already apparent at the end of the first thymopentin treatment cycle. An improvement was found in the clinical condition of 7 of the 9 patients at the end of the follow-up period with a significant correction of unbalanced lymphocytic subsets.

Adult↗

PubMed1989

Antibodies to Ro/SSA detected by ELISA: correlation with clinical features in systemic scleroderma.

Anti-Ro/SSA antibodies were determined by a newly developed enzyme-linked immunosorbent assay in serum specimens from 114 patients with systemic scleroderma in order to examine the relationship between Ro/SSA antibodies and clinical subsets of scleroderma. Sera of 42 patients (37%) were positive for Ro/SSA antibodies. Clinical investigations, including Schirmer's test and enzymatic profiles, demonstrated that 60% (16 of 27) of scleroderma patients with sicca syndrome and 63% (10 of 16) with polymyositis (PM) were Ro/SSA positive. In these patients there was a significant association between Ro/SSA antibodies and rheumatoid factor. HLA-antigens DR2, DR3 and B8 showed an increased frequency in anti-Ro/SSA positive patients.

Antibodies, Antinuclear↗

PubMed2002

[Role of interleukin-1 and interleukin-2 in the pathogenesis of systemic scleroderma].

The purpose of a research was to define the levels of interleukin-1 (IL-1) and interleukin-2 (IL-2) in patients with systemic scleroderma (SSc) and to analyse their dependence on the course of the illness. 75 patients with SSc and 20 apparently healthy people were examined. Besides the well-known clinical-biochemical and instrumental investigations, the levels of IL-1 and Il-2 in all the patients were determined in serum with an immunoenzymatic method using a set of reactants Pro Con IL-1b and Pro Con IL-2 (Russia). The data obtained give evidence for an increase in both IL-1 and IL-2 levels. As the correlation between the level of IL-1 and the acuteness of the pathological process has been shown to be especially distinct, it can be used for marking an acute form of this pathology. Thus, both parametres (IL-1, IL-2) depend on the acuity and peculiarities of SSc, and on the concomitant damage of the internal organs. The most pronounced increase in LL-2 level has been observed at visceralisation of SSc.

Adult↗

PubMed1991

[The total proteolytic and collagenolytic activity in the blood cells of patients with systemic scleroderma].

Collagenolytic (CA) and neutral caseinolytic activity (NCA) was studied in extracts from blood cells (neutrophils, mononuclear cells and platelets) of patients suffering from systemic scleroderma (SSD). 23 persons were examined. Of these, 15 had local skin lesions, 8 diffuse lesions. CA, both specific and per 10(6) cells was found to be dramatically decreased (2-4-fold) in all blood cells, whereas NCA was increased in neutrophils and mononuclear cells; in platelets, it remained within normal. The amount of protein in neutrophils and mononuclear cells was lowered 1.2 and 2-fold respectively. In diffuse skin lesions, the amount of protein in cells was lower than in local lesions. It should be noted that in SSD patients examined, the inflammatory process was unmarked. Therefore, the data on substantial changes in proteolytic activity cannot be related to the inflammatory process. A reverse correlation was established between CA in neutrophils and circulating immune complexes as was a reverse correlation between CA in mononuclear cells and blood antinuclear factor. The data presented indicate that proteinases are an important factor of the pathogenesis in SSD. Apparently, SSD is characterized not only by enhanced synthesis of collagen but also by a dramatic reduction of the activity of enzymes that specifically hydrolyze this group of proteins, which leads to a decrease of collagen catabolism and hence, to the development of fibrosis in tissues.

Adult↗

PubMed1994

"Paul Klee and the hypothesis of morphic resonance". Adjustment to illness and life history of patients with progressive systemic scleroderma.

Within the context of a Psychoanalytic Psychosomatic Medicine the biography of Paul Klee is analysed, who suffered during his last years from progressive systemic scleroderma. Amongst the pervading and characteristic motives of his life and his work, the motives of the dragon-like demon and that of the Dead are introduced. Both are shown to relate to his illness as well as to a highly significant narcissistic idealized picture of his grandmother on the mother's side. Finally, a general biological hypothesis--Sheldrake's hypothesis of morphogenetic fields and morphic resonance--is laid out for the domain of Psychosomatic Medicine. According to this hypothesis, primary process characteristics such as condensation and displacement are also seen to be valid in the somatic domain without being restricted only to conversion events.

Adaptation, Psychological↗

PubMed1986

[Clinical aspects of progressive systemic scleroderma (PSS). Multicenter studies of 194 patients].

Patients from five German Departments of Dermatology (Düsseldorf, Erlangen, Frankfurt am Main, Hamburg and Munich) affected with progressive systemic scleroderma (PSS) were classified and examined. The results of the clinical investigations are presented. In order to guarantee a uniform classification of all patients, the patients were divided into three groups according to the distribution of the affected skin: type I consisted of those with acrosclerosis distal to the wrist, type II had scleroderma extending along the wrist in a proximal direction, and type III had diffuse scleroderma beginning on the trunk. Altogether, 194 patients with PSS were investigated, and the following distribution was found: type I, 32%; type II, 60%; type III, 7%; 1% of the patients could not be classified. The distribution according to the patients' sex and age was in good agreement with published reference data. The incidence, significance, and localization of the major symptoms were investigated. The Raynaud symptom could be identified as being the main clinical symptom in 90% of the patients. Joint involvements (10%-73% depending in the applied parameters), dysphagia (51%), and rest dyspnea (30%) contributed to the main internal symptoms. The extensive clinical, chemical, and immunological results are summarized. In 80% of the cases, high ANA titers could be detected, but these were not correlated to the type of disease.

Adult↗

PubMed1986

[Treatment of systemic scleroderma with D-penicillamine. Results in 14 patients treated for more than a year].

The authors review the theoretical bases for the treatment of systemic scleroderma (SS) by D-penicillamine (DP). They then present a review of the papers published over the last 20 years on the subject. Although Rodnan's team has conducted studies against a control series and the majority of published works have concluded on the effectiveness of this treatment, there has not been any randomised prospective study, to date, to clearly demonstrate this effectiveness. The authors report a series of 14 patients with SS treated for a mean of 2 years with 900 mg/day of DP. DP seemed to be effective on the cutaneous lesions in 12 of the patients treated at a maximum of 3 years after the frank onset of the disease. Treatment failed in 2 patients who were only treated late in the disease. Objective results were obtained on the pulmonary lesion, but they are more difficult to interpret. 5 patients had to interrupt DP treatment because of intolerance. Tiopronine was tried in 4 of these patients with 3 interpretables follow-ups. This agent was well tolerated and effective in 2 of these 3 patients.

Adult↗

PubMed1976

Skin capillary changes in early systemic scleroderma. Electron microscopy and "in vitro" autoradiography with tritiated thymidine.

Skin biopsy specimens obtained from involved and noninvolved areas in a patient with early diffuse systemic scleroderma were processed for histology, electron microscopy, and "in vitro" autoradiography with tritiated thymidine. The affected area revealed cellular infiltrates around the eccrine sweat glands, consisting of plasma cells and lymphocytes. The capillaries showed thickening of the basement lamina, damage of endothelial cells, and obstruction of their lumens. However, in some vessels, endothelial cells were preserved and appeared in prophase. Autoradiography with tritiated thymidine showed a marked increase in endothelial and periendothelial cell labeling. Blood immunological studies revealed an increase in B-lymphocytes, IgG, and IgA and the presence of antinuclear and antismooth muscle antibodies.

Adult↗

PubMed2002

Transdermal application of prostaglandin E1 ethyl ester for the treatment of trophic acral skin lesions in a patient with systemic scleroderma.

An early dinical symptom in scleroderma patients is Raynaud's phenomenon. Later cutaneous manifestations of the disease include oedematous swelling in the extremities and in more extreme cases often very painful, refractory acral necroses. We report on a 56-year-old female patient who participated in a prospective, double-blind, multicentre comparative pilot study because of her severe Raynaud symptoms, with dystrophic skin lesions on both hands. The goal of the study was to evaluate the efficacy and safety of prostaglandin E1 ethyl ester in a transdermal drug delivery system compared with placebo in patients with secondary Raynaud's phenomenon associated with systemic scleroderma or mixed connective tissue disease. After 2 weeks of verum treatment the patient experienced a marked improvement of Raynaud's attacks, with increased capillary flow velocity, reduced blood stasis and dinical healing of the acral trophic lesions. For this patient the transdermal application of prostaglandin E1 ethyl ester in the form of a medicated patch proved to be a simple and effective therapy for the acral trophic skin lesions associated with systemic scleroderma.

Administration, Cutaneous↗

PubMed1983

[Systemic scleroderma in children. Apropos of 5 cases. A review of the literature].

Systemic scleroderma is a rare disease in childhood. 62 cases are analyzed. Cutaneous manifestations are identical to those seen in adults. However Raynaud phenomenon is much more frequently missing but follow-up of patients is only of 4 years' duration. We want to draw attention on possible worsening of clinical signs during intercurrent infectious episodes. We report exceptional roentgenological bone anomalies. Gastro-intestinal tract is frequently involved, particularly the oesophagus. We want to draw attention on latent small intestine involvement. A normal thoracic X-ray examination cannot rule out involvement of the lungs; systematic respiratory functional tests are absolutely necessary. All parts of the cardiac wall can be involved: we underline the particular seriousness of this involvement as it was exclusively responsible of death in 10 cases out of 21. Renal involvement is rare. We are reporting 2 cases where a staturo-ponderal retardation remains unexplained; 7 other cases in the literature report on isolated weight retardation. Biological anomalies are similar to those reported in adult. Treatment is not well-codified; we can hope that a better understanding of the disease's physiopathology will lead to the discovery of an efficient therapy.

Adolescent↗

PubMed1995

Compression of the ulnar nerve in Guyon's canal by pseudotumoral calcinosis in systemic scleroderma.

We report one case of ulnar nerve compression in Guyon's canal due to calcium deposits in a 50-year-old woman with long standing systemic scleroderma. To our knowledge, this is the second known case. The symptoms consisted of a motor and partial sensory disturbance. Calcification of the piso-triquetral joint was prolonging into Guyon's canal, lifting its contents, and into the subcutaneous tissue of the ulnar border of the wrist. Excision of calcium deposits and of the pisiform in combination with external neurolysis of the ulnar nerve resulted in complete relief of symptoms.

Calcinosis↗

PubMed2000

Double-blind, placebo-controlled study of oral calcitriol for the treatment of localized and systemic scleroderma.

BACKGROUND: Various treatments including corticosteroids, nonsteroidal anti-inflammatory drugs, D-penicillamine, interferon gamma, cyclosporine, and cytostatic drugs have been used with limited success in both morphea and systemic sclerosis (SSc). OBJECTIVE: We investigated the effect of treatment with oral calcitriol in patients with localized or systemic scleroderma. METHODS: A randomized, double-blind, placebo-controlled study of 9 months' duration with a 6-month follow-up was performed at the Department of Dermatology. A total of 27 patients (7 patients with SSc and 20 with morphea) were selected on a minimal skin score of 3 for patients with morphea and 12 for those with SSc. Each patient received calcitriol (0.75 microg/day for 6 months plus 1.25 microg/day for 3 months) or placebo for 9 months. Efficacy parameters included skin score, measurement of serum markers of collagen synthesis and degradation and, additional for the patients with SSc, oral aperture measurements, lung function studies, and esophagus motility. RESULTS: The skin score in patients with morphea after 9 months' treatment showed no significant difference between the placebo and calcitriol groups (mean percentage reduction [SD] in skin score in the placebo group was -29.3 [57.9]; in the calcitriol group it was -19.4 [46.6]). The small group of patients with SSc was inadequate to allow us to draw any conclusions regarding efficacy. No significant change was found in the serum markers of collagen metabolism. CONCLUSION: In this study calcitriol was not more effective than placebo in patients with morphea. Because of the small group of patients with SSc treated, no conclusions regarding efficacy in SSc can be drawn.

Administration, Oral↗

PubMed1991

Hemolytic-uremic syndrome with anticardiolipin antibodies revealing paraneoplastic systemic scleroderma.

Lupus anticoagulant was present in this case of paraneoplastic scleroderma revealed by hemolytic-uremic syndrome, suggesting that the autoantibody played a significant role in the sequence of events leading to anuria. Reviewing the literature we found several observations of paraneoplastic scleroderma, and in other series cases of scleroderma-linked (and in rare instances cancer-linked) antiphospholipid autoantibodies. Search for antiphospholipid antibodies should be considered in patients with systemic scleroderma as well as in patients with metastatic cancer. Presence of such procoagulant autoantibodies might predict future complications and should influence treatment strategy.

Aged↗

PubMedDate not supplied

[Effect of epinephrine on a culture of normal skin fibroblasts and fibroblasts in systemic scleroderma].

Decrease in content of cAMP and increase--of cGMP as well as stimulation of Ca2+ transport into cells were found in cell cultures of skin fibroblasts from patients with systemic scleroderma (SSD) as compared with normal state. Adrenaline affected dissimilarly the fibroblasts from the patients and healthy donors. In SSD the stimulating effect of adrenaline (10(-7)M) on the cAMP content was decreased more than 6-fold as compared with normal state; at the same time, transport of Ca2+ was inhibited by the hormone (2 X 10(-6)M) in SSD contrary to a stimulating effect of adrenaline in normal cells. Blocking of beta-adrenoreceptors with inderal removed completely the effect of adrenaline on Ca2+ transport both in normal and pathological states. Alteration in the reactions of fibroblasts to catecholamines might be important in deterioration of the physiological mechanism including inhibition of collagen synthesis in SSD.

Adenylyl Cyclases↗

PubMed1984

[Progressive systemic scleroderma. Clinical spectrum and prognostic parameters in 131 patients].

The course of progressive systemic scleroderma was analysed from data collected on 131 patients. Classification was according to the extent of cutaneous involvement, type I (28%): sclerosis as far as the wrist-joint; type II (65%): sclerosis beyond the wrist-joint; type III (7%): sclerosis beginning to affect the trunk. In addition, they were subdivided into those with or without signs of systemic inflammation. The sex ratio was 3.5 females to 1 male. In half of the patients the disease manifested itself between the age of 30 and 50 years, with a clear peak about the 40th year. Serological evidence of inflammatory and immunological phenomena was present in nearly 50%, predominantly in those with type II or III. Oesophageal involvement was present in 84%, of the lung in 56% of all patients. ECG and echocardiographic changes were demonstrated in 34% of patients, while liver, intestines and kidneys were only rarely affected. The degree of organ involvement increased from type I to type III. Signs of systemic inflammation were predominantly associated with a clinically severe course.

Adult↗

PubMed1979

Collagen heterogeneity in systemic scleroderma and other diseases.

Proportions of collagen type I and type III were investigated in different tissues (kidney, liver, heart muscle, esophagus, lung and skin) in a single case of malignant systemic scleroderma. The proportion of collagen type III was higher than in healthy controls. Similarly, an increase in the proportion of collagen type III was shown in tissues affected by other inflammatory diseases. Further the authors suggest that the increase in total kidney collagen, especially type III, has important consequences for kidney function; in the described case it had the critical influence on the fatal course of the disease, and the patient died from uremia.

Adult↗