Diffuse cutaneous scleroderma: an anaesthetic problem.
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Adolescent↗
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Results for “Scleroderma, Diffuse”
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[Dextran-therapy in diffuse (progressive) scleroderma].
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Adult↗
[Diffuse progressive scleroderma in early childhood].
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Female↗
Scleroderma overlap syndromes.
Overlap features with diffuse scleroderma are rare. More commonly, other connective tissue diseases have features usually seen in the systemic involvement of scleroderma. The limited form of scleroderma (CREST) has interesting associations with primary biliary cirrhosis, whereas mixed connective tissue disease evolves toward a scleroderma-like picture with advancing years.
Autoantibodies↗
A cytogenetic analysis of twenty cases of systemic scleroderma.
Cytogenetic studies were performed on 20 patients with diffuse scleroderma who had not received recent or high doses of irradiation; 1,267 cells were examined. Neither the culture medium composition nor the disease had any significant effect on the frequency of structural chromatidic or chromosomal abnormalities.
Adult↗
[Scleroderma].
Sclerodermas may occur in two basic forms: localized sleroderma (LSc) and systemic scleroderma (SSc). Pseudoscleroderma as well as overlap syndromes have to be differentiated from these two variants. From the clinical point of view, localized scleroderma can be subdivided into type I = plaque-like LSc (= morphea), type II = linear LSc, and type III = deep LSc. According to the degree of the cutaneous involvement, systemic scleroderma can likewise be classified into type I = sclerodactylia, type II = acrosclerosis, and type III = scleroderma with primary involvement of the trunk (diffuse scleroderma). In LSc, we never find systemic involvement; SSc, in contrast, is almost always associated with Raynaud's phenomenon, changes of the esophagus, as well as an increased titer of antinuclear antibodies (Hep-2 cell test). Only 23% of our patients with LSc showed elevated ANA titers. We present and discuss data of 56 patients with LSc and 52 patients with SSc. Evidence in the literature as well as our own findings suggest that the pathogenesis of LSc is different from that of SSc. The influence of various mediators and cytokines on the collagen metabolism might be regarded as a theoretical approach in order to develop new therapeutic regimens. This is even more important since there is still no efficient mode of treatment for neither localized nor systemic scleroderma.
Antibodies, Antinuclear↗
Scleroderma and pregnancy. Anaesthetic considerations.
The case of a pregnant patient with diffuse scleroderma who died following Caesarean section under general anaesthesia is presented. The patient's postoperative course was complicated by pulmonary oedema and pulmonary hypertension, sepsis, thrombocytopenia and renal failure. Aspects of the disease which possess anaesthetic implications are reviewed.
Adult↗
Muscle involvement in the scleroderma syndromes.
Muscle involvement was identified in 14 patients with scleroderma or a connective tissue disease overlap syndrome with predominant features of scleroderma. Patients presented with symmetrical proximal weakness indistinguishable from other inflammatory myopathies. Creatine kinase and electromyography were useful to demonstrate muscle involvement. Muscle histopathology demonstrated primarily the vasculopathy of scleroderma or polymyositis in similar numbers of patients. Scleroderma vasculopathy and polymyositis generally occur without specificity to diffuse scleroderma, the calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia syndrome, or an overlap syndrome with arthritis. Polymyositis also occurs when the vasculopathy of scleroderma involves other organ systems.
Adult↗
Significance of plasma endothelin-1 levels in patients with systemic sclerosis.
Endothelin-1 (ET-1) is a novel potent vasoconstrictor peptide discovered in the supernatant fraction of cultured endothelial cells. We measured plasma levels of ET-1 using a sensitive sandwich enzyme immunoassay. Plasma concentrations of ET-1 in 31 patients with systemic sclerosis (SSc) (1.90 +/- 0.47 pg/ml) were higher than those (1.31 +/- 0.10 pg/ml) in 25 age and sex matched healthy subjects. Patients with SSc with diffuse scleroderma had higher levels of ET-1 compared with those with limited scleroderma. Plasma ET-1 levels correlated inversely with carbon monoxide diffusing capacity (DLco). Measurement of plasma ET-1 levels may be useful as a predictor of prognosis of SSc.
Adult↗
Pulmonary involvement in systemic sclerosis (scleroderma).
One hundred sixty-five nonsmoking systemic sclerosis patients were evaluated by pulmonary function testing. Restrictive lung disease and an isolated reduction of the diffusing capacity of carbon monoxide were the most frequent abnormalities. Patients with the CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias) had a similar frequency and severity of pulmonary involvement compared with the patients who had diffuse scleroderma. CREST syndrome patients with restrictive lung disease rarely had the anticentromere antibody and had more skin and joint involvement of their hands, compared with other CREST syndrome patients. Dyspnea and rales were most commonly found in patients with restrictive lung disease. Fibrosis, shown on chest radiograph, and pulmonary function abnormalities correlated poorly with each other. Dyspnea was associated with restrictive disease, and rales were more commonly found in patients with fibrosis. Patients with a restrictive abnormality had the worst prognosis, with a 5-year survival rate of 58%, although death from pulmonary causes was uncommon. Comparison of these nonsmoking patients with 137 scleroderma patients who smoked, seen during the same time period, revealed more frequent and severe obstructive changes in smokers. Smoking patients with restrictive lung disease had more severe disease than nonsmoking patients. The single breath diffusing capacity for carbon monoxide was significantly decreased in the patients who smoked compared with the nonsmokers. These data confirm that pulmonary function abnormalities are common in patients with systemic sclerosis including CREST syndrome. Smoking appears to have an additive deleterious effect on pulmonary function and should be strongly discouraged.
Calcinosis↗
The analysis of antinuclear and antinucleolar autoantibodies of scleroderma by radioimmunoprecipitation assays.
We have characterized autoantibodies to nuclear and nucleolar antigens in 112 patients with diffuse scleroderma, CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias), scleroderma overlap syndromes, or primary Raynaud's phenomenon by indirect immunofluorescence and radiolabeled immunoprecipitation assays. We noted for the first time that anti-Th RNP antibodies represent a common antibody specificity in scleroderma (occurring in 13% of patients with scleroderma-like illnesses) and that anti-NOR 90 antibodies are quite rare in American patients (none found). In addition, we describe 3 new scleroderma-associated autoantibodies.
Antibodies, Antinuclear↗
[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↗
[Pseudoscleroderma and sclerodermiform states].
Pseudo-scleroderma should not be confused with true scleroderma, the prognosis of which is unpredictable and often serious. Progressive acrosclerosis must be differentiated from Raynaud's disease, congenital or hereditary disorders of unknown aetiology: Werner's syndrome, acrogeria and progeria; Rothmund-Thomson's syndrome, Steinert's disease, phenylketonuria, disorders of glycogen metabolism; metabolic disorders: mutilating acropathies, scleromyxoedema, porphyria cutanea tarda; occupational and iatrogenic disorders: acroosteolysis, toxic epidermic syndrome (Spain), scleroderma-like change induced by bleomycin, chronic graft-versus-host disease; and leprosy. Acute diffuse scleroderma should not be confused with Buschke's scleroedema, sclerema neonatorum, systemic amyloidosis and scleroderma-like changes in hypothyroidism. Linear pseudo-scleroderma is suggested by the following scleroderma-like conditions: facial hemiatrophy, acrodermatitis atrophicans, melorheostosis, pseudo-scleroderma after corticosteroid injection, and cutaneous lesions in carcinoid syndrome. Scleroderma in plaque must be differentiated from hypodermitis sclerotisans, panatrophy and localized lipoatrophies, hypodermitis after vitamin K injection, basal cell carcinoma, necrobiosis lipoidica, vitiligo, chronic radiodermatitis, cutaneous lymphatic invasion. Scleroderma-like changes after drug injection (vitamin B12, progestin), anetoderma barely resemble morphea guttata.
Adipose Tissue↗
[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↗
[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↗
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↗
[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↗
[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↗