De subitaneis mortibus. VIII. Coronary arteries and conduction system in scleroderma heart disease.
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Adolescent↗
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Results for “Scleroderma, Systemic”
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Tissue specificity of antinuclear antibodies in scleroderma.
Studies of antinuclear antibodies (ANA) were carried out in 39 cases of systemic scleroderma and for comparison in 19 cases of systemic lupus erythematosus (SLE) using indirect immunofluorescence (IF) methods under standard conditions. The results on three different substrates--monkey esophagus, guinea pig lip and rat liver--are reported. In 48.7% of scleroderma cases ANA showed a substrate specificity. The highest percentage of positive results in scleroderma was obtained on monkey esophagus (97.4%) and the lowest on rat liver (61.5%). In SLE, in contrast, only about 13% of the sera displayed such specificity. If only sera with substrate specificity are considered, the positive results on monkey esophagus and rat liver are 94.7% and 21.1%, respectively. Titers of sera reacting positively on 2 or 3 substrates were mostly in agreement, although some sera both in systemic scleroderma and SLE showed higher titers on monkey esophagus. The IF pattern was usually the same regardless of the substrate. Tests for ANA in scleroderma should be performed on at least 2 substrates simultaneously.
Animals↗
Presence of antinucleosome autoantibodies in a restricted set of connective tissue diseases: antinucleosome antibodies of the IgG3 subclass are markers of renal pathogenicity in systemic lupus erythematosus.
OBJECTIVE: To study the frequency and disease specificity of antinucleosome antibody reactivity in diverse connective tissue diseases (CTD), and to determine factors, such as antibody subclass, that may influence the pathogenicity of these antibodies in relation to disease activity. METHODS: IgG and IgM antinucleosome activities on nucleosome core particles from 496 patients with 13 different CTD and 100 patients with hepatitis C were measured by enzyme-linked immunosorbent assay (ELISA). Of the patients with CTD, 120 had systemic lupus erythematosus (SLE), 37 had scleroderma (systemic sclerosis; SSc), 20 had mixed connective tissue disease (MCTD), and 319 had other CTD, including Sjögren's syndrome, inflammatory myopathy, rheumatoid arthritis, primary antiphospholipid syndrome, Wegener's granulomatosis, Takayasu arteritis, giant cell arteritis, relapsing polychondritis, Behçet's syndrome, and sarcoidosis. Antinucleosome-positive sera were further analyzed, by isotype-specific ELISA, for antinucleosome and anti-double-stranded DNA (anti-dsDNA) IgG subclasses. RESULTS: SLE, SSc, and MCTD were the only 3 CTD in which antinucleosome IgG were detected (71.7%, 45.9%, and 45.0% of patients, respectively). Antinucleosomes of the IgG3 subclass were present at high levels in patients with active SLE and were virtually absent in those with SSc, MCTD, or inactive SLE, and their levels showed a positive correlation with SLE disease activity. Of note, an increase in levels of antinucleosome of the IgG3 isotype was observed during SLE flares, and this increase was found to be closely associated with active nephritis. Levels of antinucleosome of the IgG1 subclass showed a trend toward an inverse correlation with SLE disease activity. No significant fluctuation in the anti-dsDNA isotype profile was observed in relation to SLE severity or clinical signs. CONCLUSION: Our data suggest that IgG antinucleosome is a new marker that may help in the differential diagnosis of CTD; antinucleosome of the IgG3 isotype might constitute a selective biologic marker of active SLE, in particular, of lupus nephritis.
Antibodies, Antinuclear↗
Epidermal nucleolar IgG deposition in clinically normal skin: clinical and serologic features of eight patients.
Epidermal nucleolar IgG deposition in clinically normal skin reflects high serum concentrations of antibody to nucleolar antigen. This immunopathologic finding occurred in six patients with scleroderma and in two patients with a scleroderma-systemic lupus erythermatosus overlap syndrome.
Adult↗
Substrate specificity of antinuclear antibodies in scleroderma.
Studies of antinuclear antibodies (ANA) were carried out in 39 cases of systemic scleroderma and for comparison in 19 cases of systemic lupus erythematosus (SLE) and 4 of mixed connective tissue disease (MCTD) using indirect immunofluorescence (IF) methods under standard conditions. The results on three different substrates--monkey esophagus, guineapig lip and rat liver--are reported. In 48.7% of scleroderma cases ANA showed a substrate specificity. The highest percentage of positive results in scleroderma was obtained on monkey esophagus (97.4%) and the lowest on rat liver (61.5%). In SLE and MCTD, in contrast, only about 13% of the sera displayed such specificity. If only sera with substrate specificity are considered, the positive results on monkey esophagus and rat liver are 94.7% and 21.1%, respectively. Titers of sera reacting positively on 2 or 3 substrates were mostly in agreement, although some sera both in systemic scleroderma and SLE showed higher titers on monkey esophagus. The IF pattern was usually the same regardless of the substrate, Tests for ANA in scleroderma should be performed on at least 2 substrates simultaneously.
Adult↗
Localized scleroderma in breast cancer patients treated with supervoltage external beam radiation: radiation port scleroderma.
BACKGROUND: An association between systemic scleroderma, radiation, and breast cancer has been recognized. However, localized scleroderma in the radiation port of breast cancer patients has been rarely described. OBJECTIVE: Our purpose was to describe the concurrence of localized scleroderma, supervoltage radiation, and breast carcinoma. METHODS: Patients were prospectively evaluated in a tertiary care cancer center, and the literature was reviewed. RESULTS: We describe six patients with breast cancer in whom localized scleroderma developed within their radiation port. CONCLUSION: Radiation port localized scleroderma can be a sequela of supervoltage radiation therapy in patients with breast cancer. Recognition is important because localized scleroderma can clinically mimic recurrent breast carcinoma.
Breast Neoplasms↗
[Scleroderma, dermatomyositis].
Systemic sclerosis (scleroderma, SSc) is a progressive and often fatal disorder characterized clinically by sclerotic changes in the skin, joints and internal organ systems such as the gastrointestinal tract, heart, lungs and kidneys, moreover pathologically by abnormalities of mucopolysaccharides, fibrous tissue deposition, atrophy of parenchymal structures in skin and various internal organs, and by vascular insufficiency. Little is known of its etiology and pathogenesis. Transforming growth factor-beta (TGF-beta), platelet derived growth factor-AA (PDGF-AA) and PDGF-alpha receptor interaction may play an important role in the pathogenesis of scleroderma. Furthermore, many of the proteoglycans act as modulators of growth factor activities. Dermatomyositis is also a complex connective tissue disease of unknown etiology, in which inflammatory change in the skin, muscle, and lung in association with vascular insufficiency and internal malignancy.
Blood Vessels↗
[Treatment of localized scleroderma with systemic administration of a corticosteroid hormone].
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Adolescent↗
Collagen synthesis in scleroderma: selection of fibroblast populations during subcultures.
In progressive systemic scleroderma, excessive deposition of collagen leads to fibrosis of several tissues including the skin. It has been found that different populations of fibroblasts are present in scleroderma skin; these can be obtained by establishing cell cultures from different layers of the involved skin. Excessive overproduction of collagen was noted in primary cultures of cells obtained from deeper layers of the skin of patients in an early stage of the disease, whereas control fibroblasts did not manifest significant variations dependent on the layers of skin used to initiate the cultures. The synthesis of type-I and -III collagen was found to be altered concomitantly. The production of collagen and collagenous proteins was then followed during subcultivations of overproducing fibroblasts. In many cell strains, increased synthesis of collagen and/or non-collagenous proteins had already been lost after the first subcultivation, whereas overproduction was stable in others. However, after five passages, most of the cultures showed normal collagen synthesis, which probably indicates a loss of phenotype due to successive subcultures or overgrowth by another population of fibroblasts.
Adult↗
[Value of digestive manometry in scleroderma of childhood].
The authors report a case of systemic scleroderma in a 7 year-old boy, with digestive, cardiac and cutaneous involvement. They stress for the first time the pediatric significance of esophageal and recto-anal manometry from an etiologic topographic point of view.
Child↗
Intestinal absorption of L-tryptophan in scleroderma.
The purpose of this investigation was to study the intestinal absorption of L-tryptophan and to assess the absorptive function of the intestine in scleroderma. The oral L-tryptophan loading test was performed in 31 cases of systemic scleroderma (progressive systemic sclerosis, PSS) and 3 cases of localized scleroderma. Serum levels of tryptophan and urinary excretion of indole-acetic acid (IAA) and indican (IS) were determined in order to assess intestinal absorption of tryptophan. In 10 cases the D-xylose test and in 4 cases Schilling's test was also performed. Furthermore, in vitro binding of L-tryptophan by plasma proteins in PSS and in other skin diseases as controls was studied. The normal increase in serum tryptophan after loading was noted in 17 cases (in 14 cases of PSS with a mild, slow progression in 3 cases of PSS with a severe, rapidly progressing course). In 10 of these cases, urinary excretion of IAA was higher than normal and in normal and in 3 cases excretion of urinary IS was also above normal. On the other hand, in 14 cases of severe, rapidly progressing PSS and in 2 of 3 cases of widespread linear scleroderma, serum levels of tryptophan were markedly depressed after loading, while urinary excretion of IAA and IS was normal. In all 4 cases studied, Schilling's test was normal, and only in 2 of 10 cases of PSS was the D-xylose test abnormal. It is concluded that in the majority of cases of PSS, intestinal absorpiton of tryptophan is normal as also is the absorptive function of the intestine. The slight rise in serum tryptophan after loading in some cases of PSS may be a result of increased binding of tryptophan by albumin.
Adolescent↗
Rehabilitation in joint and connective tissue diseases. 1. Systemic diseases.
This self-directed learning module highlights new advances in this topic area. It is part of the chapter on rehabilitation in joint and connective tissue diseases in the Self-Directed Physiatric Education Program for practitioners and trainees in physical medicine and rehabilitation. This article discusses treatment and outcome in rheumatoid arthritis, musculoskeletal involvement in human immunodeficiency virus infection, scleroderma, systemic lupus erythematosus, and intraarticular injection of corticosteroids.
Adrenal Cortex Hormones↗
[Eosinophilia-myalgia syndrome and L-tryptophan intake].
We present the case of a 52-year-old woman who developed diffuse induration of the skin and severe edema of the subcutaneous tissue involving the extremities and the trunk, sparing hands, feet and face after 10 years of almost constant oral tryptophan medication. The skin manifestations were similar to those of eosinophilic fasciitis (Shulman syndrome). The patient complained of severe muscle pain and weakness. Laboratory studies revealed an elevated Westergren erythrocyte sedimentation rate and eosinophilia. There were no signs of internal organ involvement and no immunological parameters of progressive systemic scleroderma. Eosinophilia and myalgia resolved in response to intermittent systemic therapy with glucocorticosteroids, whereas the progressive scleroderma and the edema showed only slight improvement after the discontinuation of L-tryptophan.
Biopsy↗
[Immunology and clinical picture of progressive scleroderma].
The interrelations between clinic and immunology of systemic scleroderma serve the pathogenetic differentiation of the disease, on one hand, and the nosological classification, on the other. The primary cause of the pathogenesis of progressive scleroderma cannot be localized in the immune system. The fibroblast's activity, the ability of being stimulated and the metabolism are changed. The endothelial cell seems to be selectively influenced by serum proteases acting cytotoxically. The incidence of antinuclear antibodies reaches up to 90%, there is no correlation between titer and clinical activity. Circulating immune complexes are found with 30% of the patients, immunoglobulin and complementary deposits are found with 75%. The T-helper cell rate is increased, the T-suppressor cell population is decreased. Immunity phenomena permit the classification of three types, the definition of overlapping syndromes, as well as the estimation of visceral manifestations.
Antibodies, Antinuclear↗
Diagnosis of systemic lupus erythematosus. Importance of antinuclear antibody titers and peripheral staining patterns.
Titers and patterns of antinuclear antibodies (ANA) in sera from 134 normal blood donors, 20 patients with rheumatoid arthritis, 15 patients with systemic scleroderma, and 32 patients with diagnosed or suspected systemic lupus erythematosus (SLE) were studied. The difference between the findings with sera of patients with SLE and normal subjects in terms of high (greater than 160) titers of ANA was greater than in terms of peripheral staining patterns. However, in comparing sera from patients with SLE with sera from patients with other connective tissue diseases, greater differences were found in the incidence of peripheral patterns of ANA compared to differences in the frequency of high ANA titers. Maximum specificity in the diagnosis of SLE was achieved when both titers and patterns of ANA were considered.
Adult↗
Nodular scleroderma.
We describe a 40-year-old woman with systemic scleroderma who had hundreds of firm nodules that developed on the trunk and upper extremities during several months. We briefly review previously reported cases of this rare variant of scleroderma.
Adult↗
Hypersensitivity of scleroderma cutaneous vascular smooth muscle to 5-hydroxytryptamine.
Vascular smooth muscle strips from subcutaneous wrist vessels of patients with scleroderma (systemic sclerosis) were studied in an isolated system. No difference between catecholamine response of scleroderma or control vessel strips was observed. Three of four patients demonstrated significant hypersensitivity to 5-hydroxytryptamine as compared to control values. Serotonin hypersensitivity may play a role in the evolution of Raynaud's phenomenon and vascular scleroderma.
Adenosine Triphosphate↗
[Normotensive scleroderma renal crisis].
A 60 year old male patient having systemic scleroderma and normotensive scleroderma renal crisis was admitted in our hospital. He presented polyarticular, esophagic, lung and skin compromise. Before admission he had been treated with high doses of corticosteroids. We believe corticosteroids led to the worsening of renal damage with renal failure, microangiopathic hemolytic anemia without high blood pressure. The 10% of these cases have normal blood pressure. The patient was treated with enalapril and hemodialysis. There was no favourable response to this treatment and he died seven days after admission.
Acute Kidney Injury↗