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At least 595 records · Page 33Linked to original sources
PubMed1998

[Madecassol treatment of systemic and localized scleroderma].

AIM: The trial of efficacy of 6-month therapy with madecassol (tablets, ointment, powder) of patients with systemic and focal scleroderma (SS and FS). MATERIALS AND METHODS: 54 patients (49 females and 5 males) aged 15 to 70 years with scleroderma running from 3 months to 15 years entered the study. 30 patients had typical SS, 24 patients had FS. Tablets were given to 18 patients, ointment was applied in 42 patients, powder in 3 and tablets + ointment in 9 patients. Madecassol 10 mg tablets were taken 3 times a day by patients with SS and advanced FS. The ointment was preferred in ulcers and scars on fingers and toes in SS and vascular trophic lesions in FS. In active focal scleroderma the ointment was applied to the skin lesions. The ointment was used 2 times a day (in the morning and evening) for 1-6 months. Madecassol powder was employed rarely, primarily of anal and vulval lesions. RESULTS: 6-month oral course (30 mg/day) in 12 SS patients brought about a decrease of indurative lesions, hyperpigmentation (8), vascular trophic disorders (6) and improvement of general condition (5). Subjective response was good in 10 patients and corresponded to absence of progression. In progressive disease and diffuse skin lesions the drug was ineffective. The best response was obtained in local application of madecassol ointment on digital ulcers in SS. CONCLUSION: Madecassol is effective and well tolerated and therefore recommended for oral and local use in combined treatment of SS adn FS. Indications for per os utelization are: chronic or subchronic SS with limited skin involvement, advanced and/or prone to progression FS in which combined administration of the tablets and ointment is proposed.

Adolescent↗
PubMed1981

[Ablation of haemorrhoids by cryosurgery in high risk patients (author's transl)].

A group of 48 patients of differing age and sex, suffering from 3rd degree hemorrhoids with pulmonary, cardiac or renal disease, epilepsy or scleroderma, so that the traditional operation under general anaesthesia is contraindicated, is examined. Cryosurgical haemorrhoidectomy, without hospitalisation, under local anaesthesia of anal sphincter led in all these patients to cure verified rectoscopically 2-3 months after the operation. The postoperative course was entirely normal, with pain symptoms that were sharp in 7 cases, limited in 14. A serohematic secretion occurred for about a week in 6 patients. Normal living and working activity was resumed on average after about 11 days.

Cryosurgery↗
PubMed1983

Connective tissue activation. XXVII. The behavior of skin fibroblasts from patients with scleroderma.

Four normal (NF) and 4 scleroderma skin fibroblast (SF) strains were compared with respect to 1) basal 14C-glucosamine and 35SO4-labeled glycosaminoglycan (GAG) synthesis, 2) responsiveness to autacoid mediators, and 3) performance following maximal stimulation. Under basal conditions, SF synthesized and secreted 2-3 times more radioactive hyaluronic acid than the NF (P less than 0.001); molecular volume by gel chromatography was similar and suggested a high molecular weight product. SF were essentially as responsive to normal lymphoid and platelet factors as were NF. No consistent qualitative or quantitative differences in sulfated GAG synthesis were noted between the 2 groups of cells. Incubation of NF and SF with a false "core protein" such as p-nitrophenyl-beta-D-xyloside suggested that synthesis of the core protein was rate limiting; SF and NF were equally facile in SO4-GAG chain synthesis in the presence of a beta-xyloside. SF appear to retain in vitro a partially activated state for many generations, at least with respect to hyaluronic acid synthesis.

Adult↗
PubMed1993

[Serology in patients with scleroderma].

In order to evaluate the practical clinical value of centromere, Scl-70, and nucleolar antibodies as demonstrated routinely by the Autoimmune Department of the Serum Institute of Copenhagen, 1293 sera from 497 patients with scleroderma (SSc) and other connective tissue diseases were tested for the three antibodies and for other nuclear antibodies. The three antibodies were found in 32, 15 and 15%, respectively, of sera from patients with SSc. Since more than one of the three antibodies was rarely demonstrated in any one serum, one of them was found in two thirds of sera from patients with SSc. The specificity of the three antibodies for SSc was 95% or more. Centromere antibody was found most frequently in patients with limited SSc. Scl-70 antibody was found almost exclusively in sera from patients with extensive SSc (involving the skin of the trunk). In such sera, centromere antibody was found in only 21%. Scl-70 antibody was overrepresented and centromere antibody was underrepresented in sera from patients with pulmonary involvement, the converse being true for sera from patients with calcinosis, esophageal involvement and telangiectasia.

Antibody Specificity↗
PubMed1999

[Pulmonary involvement in systemic scleroderma. Part II. Isolated pulmonary arterial hypertension, bronchopulmonary cancer, alveolar hemorrhage].

INTRODUCTION: Pulmonary interstitial fibrosis is the most frequent cause of lung disease in systemic sclerosis. However, other pulmonary complications exist, including lung cancer, alveolar hemorrhage, and in particular isolated pulmonary arterial hypertension, which is still considered the bête noire as regards this disease. CURRENT KNOWLEDGE AND KEY POINTS: The prevalence of pulmonary arterial hypertension has been reported to range from 5 to 60% in cases of systemic sclerosis; isolated pulmonary arterial hypertension has been principally observed in subjects with a ten-year history of limited forms of the disease. As the patient remains asymptomatic for a long period, with nonspecific respiratory clinical manifestations, the diagnosis is made at a much later stage in the course of the disease. The diagnostic method of choice is echocardiography-doppler, which should be performed during the preliminary investigation, and at follow-up. The prognosis is poor, and patient survival rate at 2 years after onset of symptoms amounts to 40%. To date, no curative therapy for pulmonary arterial hypertension has yet been found. FUTURE PROSPECTS AND PROJECTS: A knowledge of the mechanisms involved in the development of isolated pulmonary arterial hypertension is essential to the determination of new and relevant therapeutic strategies. Vasodilatory treatment, notably calcium channel blockers, prostacyclin and analogs such as iloprost, may be effective at an early stage of the disease before the appearance of permanent vascular damage.

Echocardiography, Doppler↗
PubMed1997

Hemopoietic blood and marrow transplants in the treatment of severe autoimmune disease.

The past year of activities represents virtually the first year of activities in hemopoietic blood and marrow transplants in the treatment of severe autoimmune disease. The concept of profound hematoimmunoablation followed by blood or marrow transplants for severe autoimmune disease is not new, but the first publications of such cases treated for autoimmune disease alone occurred only in late 1996. Other case reports followed, and the activity has expanded rapidly such that over 40 treated patients have been described, 35 of whom have been entered into a recently created European League Against Rheumatism/European Group for Blood and Marrow Transplantation database (now extended to include data from Australasia and the United States). Four international meetings have been held, the published proceedings of which have formed the basis for an ongoing international collaborative effort. Although very early, some trends have been observed: the majority of patients (mostly with multiple sclerosis or scleroderma) have clinically improved or stabilized; the toxicity of the procedure (mostly autologous bone marrow transplantation) was as observed previously in other disorders; and better results are suggested in patients achieving significant T-cell depletion, either through conditioning, graft purging, or both. These impressions must be confirmed by prospective comparative studies using a limited number of regimens.

Animals↗
PubMed2004

[Level of soluble tumor necrosis factor receptor type I in patients with systemic scleroderma].

AIM: To study content and clinical correlations of sTNF-RI in patients with systemic sclerosis (SS). MATERIAL AND METHODS: Thirty nine SS patients were examined with enzyme immunoassay for serum levels of sTNF-RI and soluble adhesion molecules (sSAM) sVSAM-1, sISAM-1 and sR-selectine using R&D System kits (USA). The control group consisted of 14 healthy subjects (donors). Content of sTNF-RI and sSAM was considered as elevated if it exceeded relevant mean values by 1SD. RESULTS: sTNF-RI content was significantly higher in the patients than in the controls (p = 0.0001) and was similar in patients with diffuse and limited forms of the disease. A rise in sTNF-RI correlated with a rise in pulmonary artery pressure. In patients with pulmonary hypertension or restrictive pulmonary affection sTNF-RI was higher than in patients free of pulmonary hypertension or impaired external respiration function. A marked correlation was found between sTNF-RI and sVSAM-1. Patients with high CRP had significantly higher sTNF-RI level than patients with normal CRP. CONCLUSION: SS is characterized by elevated content of sTNF-RI. This content may serve a diagnostic marker of the disease progression.

Adult↗
PubMed1996

[Systemic scleroderma and sarcoidosis: 3 new cases].

We observed 3 patients with successive scleroderma (SS) and (what is considered to be) sarcoidosis (SA). The diagnosis SS included in the 3 patients: Raynaud's syndrome with pulpal necrosis and capillaritis, sclerodactylia and acro-osteolysis, multiple joint pain and FAN+. Also observed were: esophagus involvement (n = 3), pulmonary artery hypertension (n = 1), telangiectasia (n = 2) and anti-Scl 70 (n = 2). Initially, all patients had restrictive pulmonary disease. SS was diagnosed 5 to 9 years prior to SA in 2 patients. Diagnosis of SA was based on the following arguments: Loëfgren's syndrome with erythema nodosa (n = 1), parotiditis (n = 2), sicca syndrome (n = 2), myalgia (n = 2), joint involvement (n = 2), non-infectious pluropericarditis (n = 2), epitheloid and giant cell granulomas without caseous necrosis (lung = 3, liver = 1, lymph nodes = 1, salivary glands = 1, synovia = 1), negative search for bacilli, elevated conversion enzyme (n = 1) and, in each case, by the lack of any other cause. One patient died from lung cancer and another from respiratory failure. Nome of the patients had primary biliary cirhosis. This rare association between SS and SA demonstrates the confluent limits of certain systemic diseases and raises a difficult problem to differentiate pulmonary involvement in these diseases. The gravity of this localization and the poor sensitivy to corticosteroids.

Adult↗
PubMed2000

Ultraviolet A1 (340-400 nm) phototherapy for scleroderma in systemic sclerosis.

BACKGROUND: The presence of an inflammatory infiltrate consisting of helper T cells and a dysregulated matrix metabolism leading to excessive deposition of collagen are two pathogenetic factors responsible for the developments of fibrosis and sclerosis in patients with systemic sclerosis. In previous studies, ultraviolet A1 (UVA1) radiation phototherapy was shown to deplete skin-infiltrating T cells through the induction of T-cell apoptosis and to up-regulate the expression of matrixmetalloproteinase-1 (collagenase-1) in dermal fibroblasts. OBJECTIVE: Our purpose was to determine whether UVA1 phototherapy is effective for systemic sclerosis. METHODS: Lesional skin on the forearms of patients with systemic sclerosis (diffuse type, n =3; limited type, n =1) was exposed to medium-dose UVA1 radiation (60 J/cm(2)) daily. RESULTS: In all patients studied, UVA1 phototherapy-treated skin lesions were markedly softened after 9 to 29 exposures. Clinical improvement was associated with an increase in (1) joint passive range of motion values (P <.05), (2) skin temperature (thermography, P <.05), and (3) cutaneous elasticity (cutaneous elastometry, P <.05). Histologic evaluation of skin specimens obtained before and after UVA1 phototherapy revealed loosening of collagen bundles and the appearance of small collagen fibers. CONCLUSION: These studies indicate that UVA1 phototherapy is effective for patients with systemic sclerosis.

Aged↗
PubMed1999

Evaluation of costs in rheumatic diseases: a literature review.

The aim of our review was to examine recently published cost-evaluations presenting originally developed data in rheumatic conditions. We identified 21 articles: 9 presenting original data on rheumatoid arthritis and/or osteoarthritis; 7 focusing on other musculoskeletal conditions such as back pain, scleroderma, Lyme disease, and fibromyalgia; and 5 assessing costs in total knee and hip arthroplasty. Most of the studies originated in the United States. In contrast to earlier reviews in this journal, fewer studies focused on only pharmacoeconomic aspects. In reviewing these studies, we found a lack of standardization in cost-assessment leading to a limited comparability of study results. As main tasks to improve the evidence achieved by performing cost-evaluations in clinical settings, we identified a standardization of main cost-components that should be covered by each clinical trial and the assessment of validity, reliability, and comparability of different data sources used to collect cost-data.

Arthritis, Rheumatoid↗
PubMedDate not supplied

Dermatological manifestations of toxic agents.

A great variety of cutaneous disorders can be seen as reactions to toxic substances. To limit the extent of this review, cutaneous manifestations known to have an immunological mechanism are simply listed with pertinent references. The report has been focused to cover the cutaneous manifestations of toxic agents of non-immunological and unknown mechanisms. Entities discussed include acne, alopecia, dermatitis caused by spider bites, pityriasis rosea, and chemically-induced scleroderma.

Humans↗
PubMed2004

Ethnic differences in cytotoxic T lymphocyte associated antigen 4 genotype associations with systemic sclerosis.

OBJECTIVE: To determine the role of cytotoxic T lymphocyte associated antigen 4 (CTLA-4) genetic polymorphisms in susceptibility to systemic sclerosis (SSc, scleroderma). METHODS: The study population consisted of 293 African American and Caucasian patients with SSc and matched controls. Subjects were genotyped for allelic determinants at 4 polymorphic sites: 3 in the promoter region (positions -318, -1661, -1722) and one in the first exon (position +49) of the CTLA-4 gene, using polymerase chain reaction (PCR) restriction fragment length polymorphism (RFLP) methods. Genotype frequencies were compared using Pearson's chi-square or Fisher's exact test. RESULTS: In African American patients, the frequency of AG heterozygotes at position +49 was significantly higher than in controls (71% vs 36%, p = 0.003; OR = 4.37), while the frequency of AA homozygotes was significantly lower in patients than in controls (29% vs 61%, p = 0.007; OR = 0.26). The distribution of CTLA-4 alleles at other loci did not differ significantly between patients and controls. CTLA-4 genotypes were not associated with SSc in Caucasians. No differences in CTLA-4 genotype distributions were observed between patients with the limited and diffuse forms of the disease. CONCLUSION: Our data show that the exon 1 (+49) polymorphism of the CTLA-4 gene is associated with systemic sclerosis in African Americans.

Black or African American↗
PubMed1995

Electrophysiological evidence of "nerve entrapment syndromes" and subclinical peripheral neuropathy in progressive systemic sclerosis (scleroderma).

We report the electrophysiological findings and the management of 5 subjects with progressive systemic sclerosis (PSS) and clinical evidence of nerve entrapment. Three had carpal tunnel syndrome (CTS), 1 bilateral CTS and right tarsal tunnel syndrome (TTS) and 1 Guyon's canal syndrome. Only 1 patient (with CTS) showed significant clinical improvement after surgical decompression; the other 4 demonstrated a slight recovery of conduction without lasting clinical relief after conventional treatment. To explain these failures we hypothesized that these entrapment syndromes were the clinical expression of underlying diffuse damage to the peripheral nervous system (PNS). The conduction values of nerves unaffected by entrapment syndromes were within normal limits, but almost all distal velocities were below the mean of controls. Such subclinical distal peripheral neuropathy was also verified in a selected sample of 17 patients with PSS, without clinical symptoms or signs of PNS involvement. In these 17 cases the mean distal sensory and motor conduction findings of the median, ulnar, sural and tibial nerves were significantly lower than those of a control group, while no significant differences were found in the more proximal tracts of the same nerves. Furthermore, 3 of the 17 patients showed classical electrophysiological evidence of TCS and TTS without any clinical symptoms. We conclude that the subjects with PSS had subclinical polyneuropathy which may become plain polyneuropathy or nerve entrapment syndromes perhaps induced by other risk factors.

Adult↗
PubMed2002

[Pathogenesis of systemic scleroderma: immunological aspects].

Systemic sclerosis (SSc) is a connective tissue disorder that is characterized by excessive collagen synthesis by fibroblasts and by vascular hyperreactivity and obliteration phenomena. Excessive collagen production is the consequence of abnormal interactions between endothelial cells, fibroblasts and mononuclear cells. Immunological abnormalities are present very early in the development of SSc. Mononuclear cells, particularily macrophages and T lymphocytes play a prominent role in fibroblast activation and collagen synthesis through the cytokines they produce. Thus, lymphocytic infiltrates in the skin and in the lung are preferentially composed of CD8+ T lymphocytes, that produce important amounts of interleukin 4 (IL-4). The effects of IL-4 are added to these of transforming growth factor B (TGF-B) and connective tissue growth factor (CTGF) that stimulate collagen synthesis by fibroblasts. T lymphocytes produce important amounts of gamma interferon (INF-gamma) that is the best inhibitor of collagen synthesis by fibroblasts. However, the inhibitory effect of INF-gamma on collagen synthesis is diminished in SSc patients. Numerous autoantibodies can be evidenced in the serum of SSc patients. Three of them are specific for SSc and mutually exclusive: anti-centromere antibodies (Ab) in limited SSc, anti-Scl70 Ab in diffuse SSc and anti-RNA polymerase III Ab in diffuse SSc with renal involvement. These autoantibodies are good prognosis markers but their pathogenic role remains uncertain.

Autoantibodies↗
PubMed1983

Antinuclear and anticytoplasmic antibodies. Concepts and misconceptions.

Skin involvement is a common feature of several diseases in which circulating autoantibodies to nuclear and cytoplasmic antigens can be found. Patients with these diseases can be managed more efficiently if the physician is fully aware of the diagnostic and prognostic value of these various antinuclear and anticytoplasmic antibodies. The dermatologist, therefore, not infrequently, must face the bewildering task of ordering and interpreting the results of assays for an ever-increasing number of circulating autoantibodies. In this report, we have attempted to make this less burdensome for the clinician by pointing out the basis for some of the confusion that has arisen in this area. The confusion has resulted primarily from a failure to recognize the limitations of the various assays involved.

Antibodies, Antinuclear↗
PubMed1975

[Xero-radiography of the thorax(author's transl)].

In over 150 patients, comparative chest examinations were carried out using films and xerography. The following results were obtained: Using xerography, image quality depends significantly on the size and shape of the imaging structure and on its marginal sharpness. Using a sectional technique and xerography, pulmonary vessels, bronchi, hilar structures and various linear changes are more clearly demonstrated than with ordinary film. Xerography has no advantage in showing changes occupying a large area. Routine use of xerography for chest examinations is limited by the size of the selenium plates and the higher radiation dose required.

Bone Neoplasms↗
PubMed1988

Small opacities among dental laboratory technicians in Copenhagen.

After a case of advanced pneumoconiosis occurred in a dental laboratory technician, 31 other dental technicians and 30 control subjects controlled for smoking habits, sex, and age were investigated. More technicians (55%) than controls (30%) had at least grade 1 dyspnoea (p greater than 0.05). Multiple regression analysis showed that 13 technicians who had produced dental prostheses for at least 15 years had consistently lower lung function (FVC, FEV1, FEV1/FVC, MEF50, and DCO single breath), although the differences were not statistically significant. All mean lung function values for technicians and controls were within normal limits. Increases in MEF50 after breathing 80% helium and 20% O2 failed to show small airways dysfunction among the technicians. Of the six with radiological pneumoconiosis (5 simple, 1 advanced) four had symptoms. All three biopsy specimens showed varying degrees of pulmonary fibrosis. DCO single breath was diminished in four of the six. One male dental technician had scleroderma and possibly Erasmus syndrome. Blind readings showed an increased number of suspicious chest x rays films (greater than or equal to category 0/1) among older smokers and ex-smokers (p = 0.013) regardless of occupation. Our results support other evidence that dental technicians are at risk of developing pneumoconiosis. Therefore, adequate hygienic control of dental laboratories is indicated.

Adolescent↗