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PubMedDate not supplied

[Scleroderma and occupational factors: a case-control study and analysis of literature].

The scientific literature concerning occupational factors associated with scleroderma consists mostly of case report type studies. We therefore undertook a case-control study in the Province of Trento using as data source the Hospital Records System. The annual rate of scleroderma incidence in the period considered (1976-1991) was 5.8 per million in females and 1.5 per million in males. A statistically significant association was observed (OR = 9.28, p = 0.039) in the population under study for occupational exposure to solvents. Also, an excess risk was observed in male subjects exposed to silica dust, even though the simultaneous exposure to other risk factors, such as hand-arm vibrations, makes it difficult to establish the specific contribution of silica dust exposure.

Adult↗
PubMed1995

Sample size calculations in scleroderma: a rational approach to choosing outcome measurements in scleroderma trials.

Subjects with both diffuse and limited scleroderma were studied to calculate the baseline characteristics of several commonly used outcome measurements in order to provide parameters for sample size calculations for scleroderma clinical trials. From these estimates, outcome measurements were chosen as potentially responsive to change in clinical trials if their sample sizes were not prohibitively large. Forty-five patients with scleroderma were systematically assessed to determine the means and standard deviations whereby sample size calculations can be performed using this information. Examples of sample sizes were determined for the entire group, and for 2 subsets: those with diffuse scleroderma and those with diffuse disease of recent onset. Many baseline characteristics were significantly different in patients with diffuse compared to limited systemic sclerosis. The baseline values were different for the Health Assessment Questionnaire (HAQ) disability score, Functional Index, grip strength, oral aperture, finger-to-palm distance, skin score, and physician global assessment. Sample sizes can vary widely depending upon the outcome measurement chosen and the range of deltas used within the different scleroderma subsets. Sample size requirements for many outcome measures are extremely large due to marked variability in the baseline measures. Primary outcome measures in scleroderma trials should be chosen which have adequate power to detect a minimal clinically relevant change in the primary outcome measurements at the sample sizes employed. All other outcome measures should be ranked as secondary. Skin scores, global assessments, and grip strength measurements require smaller sample sizes than the other outcome measurements which were studied. Sample sizes in future trials will vary depending upon the proportion of patients with diffuse and limited scleroderma who are included.

Female↗
PubMed1995

Circulating interleukin 1 beta and soluble interleukin 2 receptor: evaluation as markers of disease activity in scleroderma.

OBJECTIVES: To investigate whether circulating levels of interleukin 1 beta (IL-1 beta) or soluble interleukin 2 receptor (sIL-2R) reflect clinical disease status and response to therapy in scleroderma. METHODS: Plasma IL-1 beta and serum sIL-2R were measured by ELISA in 19 patients with limited cutaneous scleroderma (9 with extraesophageal internal organ involvement), 5 patients with diffuse cutaneous scleroderma and internal organ involvement, and 11 healthy controls, as well as serially over 12 months in 4 patients with scleroderma treated with cyclosporine. RESULTS: IL-1 beta levels were similar in scleroderma and control subject groups. sIL-2R levels were significantly higher in subjects with scleroderma involving internal organs (elevated in 93%), and correlated with erythrocyte sedimentation rate. sIL-2R levels decreased over 12 months in 2 of 4 patients taking cyclosporine in whom other variables remained unchanged. CONCLUSIONS: Elevated serum sIL-2R is a marker of internal organ involvement in scleroderma and warrants further investigation in assessing disease prognosis and response to therapy.

Adult↗
PubMed1994

[Systemic sclerosis].

Systemic sclerosis is a generalised connective tissue disorder characterised clinically by thickening and fibrosis of the skin and by the involvement of such internal organs as the kidneys, lungs, heart and gastro-intestinal tract. Although the aetiology of systemic sclerosis is unknown, its prevalence has been reported to be increased in workers exposed to silica. Most pathogenic findings in patients with systemic sclerosis involve changes in the vascular system, immunological anomalies and disturbances in the regulation of fibroblast function. Two subsets are recognised according to the degree of skin involvement: the limited cutaneous form, usually confined to acral features; and diffuse cutaneous scleroderma, manifesting involvement both of the trunk and extremities. The latter subset is characterised by more rapid progression of skin and visceral involvement, poorer prognosis and manifestly reduced survival. No adequate therapy has been found, and optimal use of the available therapies necessitates accurate subset assignment of the patient in order to be able to assess the stage of disease in relation to vascular, immunological and fibroblast function changes.

Adjuvants, Immunologic↗
PubMedDate not supplied

Prognosis and management of scleroderma and scleroderma-like disorders in children.

Scleroderma is a spectrum of disorders of which juvenile scleroderma forms a part. Fortunately, most children develop one of the localised forms and systemic disease is late. The prognosis of any individual type of scleroderma is dependent upon the subset of the disease, its extent, and internal organ involvement. In childhood, the problem is usually not one of survival, but of morbidity due to atrophy of skin, muscle and bone, with the potential for eventual growth defects and deformity. Drug treatment, when required, is usually directed towards suppressing inflammation and collagen deposition; vascular therapy does not play a major role, but physical therapy is crucial in certain forms of the disease.

Child↗
PubMedDate not supplied

Aggressive treatment in childhood rheumatic diseases.

Much remains to be learned about the optimal therapy for children with rheumatic diseases. Current therapies remain inexact and are aimed at either the inflammatory or the immune responses of patients. There have been a few advances, for example in the treatment of children with dermatomyositis, lupus and, in particular, Kawasaki disease. Progress in the treatment of juvenile rheumatoid arthritis (JRA) and the spondylarthropathies has lagged behind, however, although methotrexate does appear to be promising in the short-term treatment of JRA. Another urgent problem which remains to be resolved is the identification of those children who will have poor outcomes and who warrant early, aggressive treatment. A number of interesting alternative therapies for adults have been proposed, but their applicability to children remains an open question. Further investigation of combined therapies with various drugs also warrants exploration.

Arthritis, Juvenile↗
PubMed1994

Comparison of aminobenzoate potassium and placebo in the treatment of scleroderma.

OBJECTIVE: To determine the safety and efficacy of aminobenzoate potassium (KPAB) in treating the skin manifestations of scleroderma. METHODS: Via a 48-week prospective, randomized, double blind, placebo controlled trial we compared the efficacy of KPAB 12 g/day with matching placebo. Outcome measures included skin mobility and thickening scores, patient and physician global assessments and, measurements of maximal oral aperture and hand range of motion. RESULTS: Of 146 patients who entered the study, 76 (52%) completed. Demographics of the study population included age 49 +/- 13 years, 83% women, mean (range) disease duration was 104 (7-600) months. There were no differences in the demographics of the KPAB vs placebo nor the group that completed the study compared with the withdrawal group. There were no clinical or statistically significant differences between the KPAB and the placebo treated groups in any of the outcome measures. Subgroup analyses of skin mobility and skin thickening based on age, extent of disease, severity of disease, duration of disease and involved vs uninvolved skin were performed, but no differences were noted. The overall compliance to the medical regimen was > or = 75% in 93% of patients completing the study. Eighteen patients in the KPAB group and 6 placebo patients withdrew due to adverse drug reactions (ADR). The most common withdrawals for ADR were gastrointestinal intolerance and headaches. All ADR resolved following withdrawal of medication. CONCLUSION: KPAB did not alter the skin changes of scleroderma in a group of patients with relatively longstanding stable disease. KPAB was reasonably well tolerated in this group of patients.

4-Aminobenzoic Acid↗
PubMed1994

Genetic and immunological differences between Japanese patients with diffuse scleroderma and limited scleroderma.

OBJECTIVE: To study the association between HLA-DR and scleroderma (SSc), subsets of SSc, and autoantibodies in SSc. METHODS: HLA-DR antigens were determined in 45 Japanese patients with SSc. The association between HLA-DR and SSc, subsets of SSc, and autoantibodies was analyzed in 22 patients with SSc excluding mixed connective tissue disease (MCTD)/overlap syndrome (OL). RESULTS: When the 20 patients with MCTD and 3 patients with OL were excluded from the original patient group, a significant increase of HLA-DR2 was observed (59 vs 29% of controls, p < 0.01). The frequency of DR2 increased to 69% in patients with diffuse SSc (p < 0.01). DR1, which was not found in diffuse SSc, was found in 2 of 9 patients with limited SSc. The frequency of DR2 was significantly higher in patients with antitopoisomerase I (10/12, 83%, p < 0.05). In contrast, DR1 was found only in 2 patients with anticentromere antibodies (ACA), and all 5 patients with ACA had no HLA-DR2 (p < 0.01). CONCLUSION: Our results suggest that different HLA-DR markers may be associated with the production of distinct autoantibodies in diffuse SSc and limited SSc.

Antibodies↗
PubMed1993

[Types of anticellular structure antisera in scleroderma patients].

The sera of 338 scleroderma patients were cytologically examined with indirect immunofluorescence method. The sera were classified according to the cellular structure, to which they were against respectively. Among these sera, six main types of cellular structure antiserum were found: antikinetochore, antinucleolus, antichromatin, antiheterochromatin, antichromosome and anticentrosome. Some of these main types can further be subtyped with respect to the substructure or the chemical components (antigens) of a particular cellular structure. These types of antiserum may possibly be correlated with the clinical signs of scleroderma patients. For exploring the possibility to use for diagnosis.

Adolescent↗
PubMed1993

Scleroderma.

Research into the pathogenesis of scleroderma, which may ultimately yield a cure, is continuing. In the meantime much can be done for patients presenting with scleroderma today. In mild cases reassurance to allay unjustified fears is one of the most important aspects of management. In severe cases, the use of penicillamine and some of the other available therapies has improved quality of life and chances of survival.

Female↗
PubMed1993

Connective tissue diseases.

The classic connective tissue diseases (lupus erythematosus, dermatomyositis, and scleroderma) have been described with respect to their pathogenesis, clinical picture, laboratory diagnosis, and management. Because the aim of this volume is to provide an interface between plastic surgery and dermatology, the cutaneous manifestations, both shared and pathognomonic for each of these diseases, have been emphasized. An attempt has been made to point out opportunities for surgical management of some of the problems associated with these diseases.

Dermatomyositis↗
PubMed1993

Demonstration of interleukin 8 and autoantibodies to interleukin 8 in the serum of patients with systemic sclerosis and related disorders.

BACKGROUND AND DESIGN: Interleukin 8 (IL-8), a chemotactic cytokine produced by various cell types, displays structural homology to the connective tissue-activating peptide III. Little is known of the possible role of IL-8 in connective tissue disorders. We therefore determined serum concentrations of IL-8 and autoantibodies to IL-8 in 134 patients with systemic sclerosis (SSc) and related connective tissue disorders, as well as in pooled serum from 28 healthy control subjects by a sensitive enzyme-linked immunosorbent assay. RESULTS: Interleukin 8 was undetectable in the pooled serum from 28 healthy controls, but detectable in serum samples from 24 of the 134 patients described above. It was detected in 13 of 60 patients with limited SSc and in eight of 48 patients with diffuse SSc. It was also detectable in one of three patients with eosinophilic fasciitis and in two of 10 patients with Raynaud's syndrome without skin involvement. In contrast, none of the three patients with morphea or the 10 patients with eosinophilia-myalgia syndrome had detectable IL-8 levels. We further determined the concentration of autoantibodies to IL-8 in the same serum samples. The values in healthy controls were 6.7 +/- 0.2 ng/mL (mean +/- SEM). Significantly elevated autoantibody levels were detected in patients with limited SSc (21.5 +/- 1.7), diffuse SSc (23.4 +/- 2.2), and Raynaud's syndrome (20.5 +/- 3.7). Elevated levels were also detected in patients with eosinophilic fasciitis (43.7 +/- 8.6) and morphea (14.7 +/- 3.2). Normal levels (7.5 +/- 2.0) were found in patients with eosinophilia-myalgia syndrome. Analysis of variance between the levels of autoantibodies to IL-8 and duration of the disease, extent of skin involvement, drug therapy, or serologic findings failed to show a significant correlation. CONCLUSIONS: These results suggest that increased production of IL-8 may relate to activation of mononuclear phagocytes, fibroblasts, or endothelial cells, among other cell types, in patients with SSc, but not in those with eosinophilia-myalgia syndrome. This activation could be related to the production of autoantibodies to IL-8.

Adult↗
PubMed1979

Biogenic amines derived from tryptophan in systemic and cutaneous scleroderma.

Blood levels of serotonin (5-HT) and urinary excretion of 5-hydroxyindole-acetic acid (5-HIAA), tryptamine (T), and indole-acetic acid (IAA) were determined in 39 cases of systemic scleroderma and in 7 cases of very severe cutaneous scleroderma. The T/IAA ratio was normal and serotonin elevated in mild and rather severe acrosclerosis alike, i.e. in systemic scleroderma with pronounced vascular involvement. The T/IAA ratio was increased and serotonin normal in severe acroscleroderma, representing an intermediate form or transition to diffuse scleroderma, in severe diffuse scleroderma, and in severe cutaneous scleroderma. The T/IAA ratio and serotonin level were both elevated in a few cases of systemic scleroderma and in severe generalized morphea with concomitant vascular changes. The findings suggest impaired monoamine oxidase (MAO) activity in scleroderma with consequent accumulation in the organism of biogenic amines derived from tryptophan. An increased T/IAA ratio seems to be of prognostic significance in scleroderma, suggesting an adverse course of the disease.

Colorimetry↗
PubMed1995

Mortality in systemic sclerosis: a comparison with the general population.

OBJECTIVE: To compare the mortality rate in patients with systemic sclerosis (SSc) with that of the general population. METHODS: Standardized mortality ratios (SMR) were calculated for 237 patients with SSc followed prospectively, using age and sex specific mortality rates in Ontario for the period 1976-1990. RESULTS: The overall SMR for the SSc cohort was 4.69. The mortality rate was greater with diffuse than with limited scleroderma (SMR 6.18 and 3.80, respectively), but not different between men and women. CONCLUSION: The mortality rate in SSc is increased compared to that of the general population, especially in the subset with diffuse disease.

Adult↗
PubMed1996

Cutaneous manifestations of selected rheumatologic diseases.

Systemic lupus erythematosus, chronic cutaneous lupus erythematosus and subacute and acute cutaneous lupus erythematosus are associated with distinct cutaneous manifestations, and each disease has a particular clinical course and prognosis. Dermatomyositis, an immune-mediated disorder of unknown etiology that is often associated with a malignancy, consists of an inflammatory myopathy combined with characteristic cutaneous findings. Some patients with dermatomyositis also have clinical features that overlap with those of systemic lupus erythematosus or scleroderma. Manifestations of scleroderma range from cutaneous involvement alone to multisystem internal disease. Morphea, progressive systemic sclerosis, eosinophilic fascitis, eosinophilia myalgia syndrome and mixed connective tissue disease are all within the spectrum of scleroderma.

Dermatomyositis↗