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PubMed1995

Chromosome changes in lymphocytes of patients with scleroderma.

The authors have analyzed cytogenetically 28 cultured lymphocytes from females with Diffuse Scleroderma and 28 female controls between 30 and 70 years of age. Recurrent chromosome abnormalities were +8, +X, -X, and the PCD(X) phenomenon. Triplo X cells were significatively more frequent in patients than in controls. The incidence of +X and PCD(X) was significatively higher in the patients between 30 and 50 years of age, while the frequency of -X cells was higher in controls than in patients. None of these chromosome changes was correlated with the presence of anticentromere antibodies (ACA) in the patients' serum. Random structural chromosome abnormalities were also observed in the patients, but no break point clustering was observed. The incidence of chromosome breaks was significatively higher in patients than in controls. These data suggest a general tendency of females with Scleroderma to develop X polisomies and +X and the PCD(X) phenomenon may be considered Scleroderma related in younger patients.

Adult↗

PubMed1990

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↗

PubMed1992

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↗

PubMed1984

[Prognosis and treatment of scleroderma].

The prognosis and treatment of scleroderma depend on the clinicopathological form of the disease: superficial, localised scleroderma is only a minor cosmetic problem; circumscribed, deep forms of scleroderma may cause troublesome symptoms which justify steroid therapy. Circumscribed and diffuse scleroderma are similar to systemic scleroderma for which prognostic criteria have been identified. There is a poor prognosis in men, in extensive skin involvement and in intestinal, cardiac or renal involvement. Limited skin disease and anti-centromere antibodies are associated with a good prognosis. The absence of an animal model and the uncertainty as to the underlying cause, explain the therapeutic problems. The authors discuss the respective value of a number of "specific" drugs, including D-penicillamine which seems to be the most hopeful line of treatment. Continuous follow-up of this chronic condition remains essential for the diagnosis and symptomatic management of visceral complications.

Adrenal Cortex Hormones↗

PubMed1993

Large vessel occlusive disease associated with CREST syndrome and scleroderma.

OBJECTIVES: To report the cases of three patients with CREST syndrome and one patient with diffuse scleroderma who had severe macrovascular disease and only minimal vascular risk factors. METHODS: The medical histories, physical examinations, and results of clinical investigations were reviewed in four patients. RESULTS: These four patients had severe morbidity from macrovascular disease of the arms and legs in the presence of minimal underlying vascular risk factors. These patients represent 11% of the women with scleroderma seen at our hospital since 1974. This is a greater than threefold increase above the expected proportion of symptomatic vascular disease seen in population studies. In the patients with CREST syndrome, large vessel disease was first seen more than 10 years after the onset of Raynaud's phenomenon, which was the first manifestation of the disease. A pathological specimen of the ulnar artery from one patient showed severe luminal narrowing by an acellular material with no evidence of atheroma. CONCLUSIONS: These cases suggest an association of both the CREST syndrome and scleroderma with macrovascular disease.

Aged↗

PubMed1985

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↗

PubMed1990

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↗

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↗

PubMed1993

[Correlation between various autoantibodies in scleroderma patients and their clinical features].

We studied the antibodies of cell structural localized antibody and the correlation between the new classifications and their clinical features. The results suggest that antikinetochore antibody is the apparent indicator of CREST syndrome, anticentrosome antibody the apparent indicator of local scleroderma with mild clinical features and better prognosis, antinucleous antibody the apparent indicator of diffuse scleroderma with most severe clinical features, high mortality and poor prognosis. New classifications 13 of diagnostic value in clinical grouping and prognosis estimation.

Adolescent↗

PubMed1994

Scleroderma and silicone breast implants.

A 43-yr-old caucasian female presented in July 1992 with an explosive onset of diffuse scleroderma following general anaesthesia for orthopaedic surgery. Her environmental exposures included silicone breast prosthetic surgery and silica exposure, both preceding development of scleroderma. She was DR5-negative, DRw52 positive. All currently available literature relating to breast implants and scleroderma is reviewed and the conclusion drawn that, for currently documented US data, there is no evidence to suggest that patients having had breast augmentation have rates of scleroderma higher than expected to chance alone. However the cause-effect relationship between silicone and scleroderma/other connective tissue disease requires, not just anecdotal reports, but a rigorously designed study.

Adult↗

PubMed1984

[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↗

PubMed1995

[Systemic sclerosis. A rare connective tissue disease with manifestations in many organs].

Systemic sclerosis (scleroderma) is a rare connective tissue disease which can affect most human organs. Systemic sclerosis is divided into two groups: one diffuse scleroderma form, which is often more serious, with extensive organ involvement, and a limited scleroderma form with good prognosis. During a period of 20 years from 1974 to 1994, 14 patients were diagnosed as having systemic sclerosis in a population of approximately 150,000. Five were classified as having the diffuse form, and nine as having the limited form. Ten patients were found to have antinuclear antibodies. All patients had Raynaud's phenomena and scleroderma. Involvement of the gastrointestinal tract/lungs and joint-tendon sheets were found in six and eight patients respectively. Muscles, heart and kidneys were involved in three of the patients. Our results correspond with those described in the literature.

Adult↗

PubMed1990

[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↗

PubMed1995

Variability of skin scores and clinical measurements in scleroderma.

OBJECTIVE: To determine the variability of several clinical outcome measurements commonly used in scleroderma clinical trials. METHODS: Ten researchers, members of a multicenter placebo controlled trial of methotrexate in early diffuse scleroderma, studied the intraobserver and interobserver variability of variables used to assess efficacy in scleroderma trials. RESULTS: For most measures, the variability within an observer was less than that found between observers, and therefore the intraobserver reliability was better than the interobserver reliability. The reliability of the modified Rodnan skin score exceeded the Rodnan skin score. Measures with inherent interpretation such as global assessments and skin scores had more variability than easily performed measurements such as grip strength and oral opening. CONCLUSION: Some of our variability was higher than variability previously reported; this could be due to the large number of examiners and patients in our study.

Female↗

PubMed1990

[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↗

PubMed1983

D-penicillamine treatment of progressive systemic sclerosis (scleroderma): a comparison of clinical and in vitro effects.

Punch biopsies of skin were obtained from the forearms of 3 patients with progressive systemic sclerosis with diffuse scleroderma before and after treatment for 1 year or more with D-penicillamine. While on therapy, each patient studied had demonstrated a marked reduction in skin thickening. Collagenase-sensitive protein and glycosaminoglycan accumulation were measured in fibroblast cultures derived from these biopsies. No differences were observed pre- and post-treatment. We conclude that although D-penicillamine may exert its effect in vivo on connective tissue synthesis, maturation and/or turnover, fibroblasts remaining in the thinned dermis retain their potential for increased synthesis of connective tissue.

Adult↗

PubMedDate not supplied

[Telangiectasias in progressive systemic sclerosis (generalized scleroderma). Observation on 120 cases].

120 patients with progressive systemic sclerosis (PSS) were studied and subdivided into five groups according to the PSS classification of Giordano et al. ("acute diffuse scleroderma", "intermediate syndrome", "acrosclerosis sensu stricto", "sclerodactylia" and "sclerosis sine scleroderma"). In all five subgroups telangiectasias incidence was high (from 75% to 100%). The "ramosus" and "telangiectatic mats" types occur more frequently than other forms of telangiectasias. The former particularly involves the face, neck and chest; whereas the latter more usually involves the upper extremities and is the only type which appears at the lips. Cuticular telangiectasia is a third type, as important as the others but less frequent. The incidence of telangiectasias is related to disease duration. Similar telangiectasias have been observed in rarer patients with other connective tissue diseases (SLE, RA, dermatomyositis and undifferentiated connective tissue diseases).

Arthritis, Rheumatoid↗

PubMed1979

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↗