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Results for “Scleroderma, Limited”

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At least 397 records · Page 22Linked to original sources
PubMed1999

Serum xylosyltransferase: a new biochemical marker of the sclerotic process in systemic sclerosis.

UDP-D-xylose:proteoglycan core protein beta-D-xylosyltransferase (EC2.4.2.26) is the initial enzyme in the biosynthesis of chondroitin sulfate and dermatan sulfate proteoglycans in fibroblasts and chondrocytes. Secretion of xylosyltransferase into the extracellular space was determined in cultured human dermal fibroblasts. A more than 6-fold accumulation of xylosyltransferase activity in cell culture supernatant was observed (day 1, 0.6 microU per 106 cells; day 9, 4.1 microU per 106 cells); however, intracellular xylosyltransferase activity remained at a constant level (0.4 microU per 106 cells). Exposure of human chondrocytes to colchicine led to a 3-fold decreased level of xylosyltransferase and chondroitin-6-sulfate concentration in cell culture. Specific xylosyltransferase activity and chondroitin-6-sulfate concentration decreased in a concentration-dependent manner and in parallel in culture medium and accumulated 5-fold in cell lysates indicating that xylosyltransferase is secreted simultaneously into the extracellular space with chondroitin sulfate proteoglycans. Xylosyltransferase activities were determined in serum samples of 30 patients with systemic sclerosis. Xylosyltransferase activities in female (mean value 1.28 mU per liter, 90% range 1.10-1.55 mU per liter) and male patients (mean 1.39 mU per liter, 90% range 1.16-1. 57 mU per liter) with systemic sclerosis were significantly increased in comparison with blood donors of a corresponding age. Furthermore, xylosyltransferase activity was correlated with the clinical classification of systemic sclerosis. Female patients with diffuse cutaneous systemic sclerosis showed higher serum xylosyltransferase activities than patients with limited systemic sclerosis. These results confirm that the increase of proteoglycan biosynthesis in sclerotic processes of scleroderma is closely related to an elevated xylosyltransferase activity in blood and demonstrate the validity of xylosyltransferase as an additional diagnostic marker for determination of sclerotic activity in systemic sclerosis.

Adult↗
PubMed2006

Does cyclically iloprost infusion prevent severe isolated pulmonary hypertension in systemic sclerosis? Preliminary results.

The objective of this study was to present our clinical experience about scleroderma-related pulmonary hypertension in the patients treated with intermittent iloprost infusions. Eighty-one patients affected by systemic sclerosis (12 men, 69 women; 30 with diffuse pattern and 51 with limited pattern; mean age 55.1 years; mean duration of disease 105.3 months) have been treated with cyclic iloprost infusions for at least 15 months (range 15-126 months). During the last 4 months all patients underwent Doppler echocardiography in order to estimate the value of systolic pulmonary artery pressure. In 14 subjects (17.2%) systolic pulmonary artery pressure was = or > 35 mmHg. Four patients presented high systolic pulmonary artery pressure associated with pulmonary fibrosis (mean value 40.5 +/- 4.5 mmHg). Ten women (one with diffuse pattern of disease and nine with limited form) showed isolated high systolic pulmonary artery pressure; one of these patients underwent right heart catheterization which resulted normal. The remaining nine patients (mean age of 67.1 years; age at the onset of scleroderma 52.2 years) showed estimated systolic pulmonary artery pressure values between 35 and 50 mmHg. Among these patients affected by isolated pulmonary hypertension only one has been receiving bosentan in association with iloprost infusions. None of our scleroderma patients treated with cyclic iloprost infusions developed severe isolated pulmonary hypertension. In systemic sclerosis the multiple effects of iloprost on endothelium, platelets and cytokine network may counteract the vasospastic profile of lung microvasculature in pulmonary arterial hypertension and the consequent vascular wall remodelling, thus preventing the development of severe illness.

Adult↗
PubMed2004

Drug eruption caused by azathioprine: value of using the drug-induced lymphocytes stimulation test for diagnosis.

Azathioprine (AZA) is an immunosuppressant commonly used for organ transplantation and autoimmune diseases. Allergic side effects of AZA are rare, and reported allergic skin eruptions from AZA are very limited in Japan. We report AZA-induced drug eruption that developed in two cases of systemic scleroderma with polymyositis. One case presented with Stevens-Johnson syndrome, and the other had systemic papular erythema. The stimulation indices of the drug-induced lymphocyte stimulation test (DLST) for AZA in these two patients were as high as 2,180% and 430%, respectively, but those of healthy volunteers were under 120% without nonspecific suppression of lymphocyte proliferation. Other drugs used simultaneously were ruled out by patch and challenge tests. The challenge test for Stevens-Johnson syndrome type drug allergy is very risky. DLST is a good diagnostic tool for AZA allergy, especially for severe drug allergy cases.

Azathioprine↗
PubMed1999

CREST syndrome and periodontal surgery: a case report.

CREST syndrome is a slowly progressive form of systemic scleroderma. It is characterized by calcinosis cutis, Raynaud's phenomenon, esophageal involvement, sclerodactyly, and telangiectasia. There are limited reports of dental treatment for patients with this syndrome, and no reports of periodontal surgical procedures. This paper presents a case report of periodontal surgical treatment in a 38-year-old female patient with CREST syndrome, and a discussion of the clinical manifestations of the syndrome as they relate to dental treatment.

Adult↗
PubMed2001

Recombinant relaxin: a review of pharmacology and potential therapeutic use.

Use of recombinant relaxin in the treatment of systemic sclerosis (or scleroderma) has been explored and determined as ineffective. However, continued research has revealed that relaxin is not limited to its role as a hormone. Relaxin has also been shown to decrease collagen formation and secretion, increase collagenase production, influence renal vasodilation, increase vascular endothelial growth factor expression and angiogenesis, promote dilation of blood vessels, and inhibit release of histamine. Further studies to discover other potential uses of relaxin are well-justified.

Animals↗
PubMed2005

Phototherapy and photochemotherapy of sclerosing skin diseases.

The treatment of sclerosing skin diseases [systemic sclerosis, localized scleroderma, lichen sclerosus et atrophicus, sclerodermoid graft-vs.-host disease, scleredema adultorum (Buschke), scleromyxedema and necrobiosis lipoidica] is difficult and remains a great challenge. Numerous treatments, some with potentially hazardous side effects, are currently used with only limited success. The introduction of phototherapy and photochemotherapy for sclerosing skin diseases has considerably enriched the therapeutic panel and proven useful in a number of sclerosing skin diseases especially in localized scleroderma. Two phototherapeutic modalitites are used for the treatment of sclerosing skin diseases, long-wave ultraviolet A and psoralen plus ultraviolet A (PUVA). This article reviews current knowledge about the application of phototherapy and photochemotherapy to various sclerosing skin disorders.

Humans↗
PubMed1999

Watermelon-stomach as a cause of chronic iron deficiency anemia in a patient with systemic sclerosis.

Watermelon-stomach is a rare cause of gastrointestinal bleeding. There has been an increasing number of reports on the association of this lesion with diseases of the scleroderma group, causing chronic, sometimes severe gastrointestinal blood loss. The present report presents the case of a 75-year-old female with limited cutaneous systemic sclerosis and watermelon-stomach, which was the cause of her long-standing sideropenic anemia.

Aged↗
PubMedDate not supplied

Outcome of patients with scleroderma admitted to intensive care unit. A report of nine cases.

OBJECTIVE: Patients with systemic rheumatic disease constitute a small percentage of admissions to the medical intensive care units (ICUs). Systemic sclerosis (SSc) is one of the rheumatic diseases that together with secondary complications may lead to a critical illness requiring hospitalization in the ICU. We present the features, clinical course and outcome of critically ill patients with scleroderma that were admitted to the ICU. METHODS: The medical records of nine patients with diagnosis of scleroderma (8 female, 1 male), admitted to the intensive care unit of Sheba Medical Center during the 11-year interval between 1991 and 2002, were reviewed. RESULTS: The mean age of the patients at the time of admission to the ICU was 48 +/- 13 [SD] years. The mean duration of SSc from diagnosis to the ICU admission was 8 +/- 8 years. Six patients had diffuse SSc, two patients had limited SSc and one patient had juvenile diffuse morphea. The main reasons for admission to the ICU were: infection/ septic syndrome (n = 4), scleroderma renal crisis (SRC) with pulmonary congestion (n = 2), acute renal failure associated with diffuse alveolar hemorrhage namely scleroderma- pulmonary - renal syndrome (SPRS) (n = 1), iatrogenic pericardial tamponade (n = 1), mesenteric ischemia (n = 1). The patients had high severity illness score (mean APACHE II 25 +/- 3). Eight out of nine patients (89%) that were admitted to the ICU died during the hospitalization, six (66.6%) of them died in the ICU. Septic complications as the main cause of death were determined in five patients (62.5%), while four of them had pneumonia and acute respiratory failure along with underlying severe pulmonary fibrosis. Lungs and kidneys were the most common severely affected organs by SSc in our patients. CONCLUSION: The outcome of scleroderma patients admitted to the ICU was extremely poor. Infectious complication was the most common cause of death in our patients. Although infections are treatable, the high mortality rate for this group of patients was dependent on the severity of the underlying visceral organ involvement, particularly severe pulmonary fibrosis. The severity of this involvement is a poor outcome predictor. An early diagnosis and an appropriate treatment of such complications may help to reduce the mortality in scleroderma patients.

Adult↗
PubMed1991

Current topics in the diagnosis and treatment of scleroderma.

Scleroderma has been viewed as one of the most discouraging diffuse connective tissue diseases to manage. In the last 10 years, we have seen major advances in our understanding of its pathogenesis. New techniques for earlier diagnosis widen the window of opportunity for therapeutic intervention. A multitude of possible disease-modifying therapies are under study. Advances in molecular biology, including increased understanding of the cellular messages controlling the process of fibrosis, provide additional hope for better treatment. Clinical research in this disease is inhibited by limited numbers of patients and the difficulties encountered in evaluating progression and response to treatment. All patients with suspected or newly diagnosed scleroderma should be evaluated at a medical center actively engaged in the research of this disease. Such research is likely to accelerate the gains made during the last decade.

Adult↗
PubMed1984

Oral and gingival changes in systemic sclerosis (scleroderma).

Nineteen patients with systemic sclerosis, including two with CREST syndrome variant, were clinically evaluated for oral and gingival manifestations of their disease. Subjectively over 50% complained of xerostomia, limited opening, recurrent "mouth sores" and dysphagia. Four patients indicated that they had been refused dental care due to limited accessibility to the oral cavity. Objectively, physical examination disclosed prominent lingual and buccal mucosal crenations and loss of tongue mobility with fibrotic induration in 25% of the subjects examined. Three of the patients with advanced disease exhibited foci of severe gingival recession due to fibrous strictures and attached gingiva stripping. None of the patients showed any indication of uncontrolled dental disease secondary to xerostomia.

Adult↗
PubMed1979

Enzymes of collagen biosynthesis in skin and serum and dermatological diseases. II. Serum enzymes.

Serum immunoreactive prolyl hydroxylase protein and galactosylhydroxylysyl glucosyltransferase activity were measured in 54 patients wtih various dermatological diseases and compared with corresponding values in 32 control subjects. These serum enzymes were at the control level in the great majority of the patients, and no correlation was found between serum and skin enzymes, except for one weak correlation in lichen ruber planus. Some patients with psoriasis, lichen ruber planus, keloids, erythema nodosum or chronic discoid lupus erythematosus, however, did have at least one of these enzymes elevated in the serum, and a significant correlation (P less than 0.01) between the two enzymes was found in the total disease material. Thus it does seem that diseases limited only to the skin can sometimes raise these serum enzyme levels. The mean levels of serum immunoreactive prolyl hydroxylase and galactosylhydroxylysyl glucosyltransferase activity were significantly elevated (P less than 0.001) in active systemic connective tissue diseases such as systemic lupus erythematosus, scleroderma or dermatomyositis compared with the controls, and 4 out of 7 values for immunoreactive prolyl hydroxylase and 3 out of 7 for galactosylhydroxylysyl glucosyltransferase activity were above the 95% confidence limit of the controls. Since the levels of the skin enzymes were not elevated in most of these patients, however, the main sources for the elevated serum enzymes were probably tissues other than the skin.

Adolescent↗
PubMed1995

Intracerebral involvement in scleroderma en coup de sabre: report of a case with neuropathologic findings.

Linear scleroderma en coup de sabre (LScs) is a rare disorder not infrequently associated with neurologic symptoms, notably epilepsy. However, histopathologic documentation of intracerebral lesions in LScs is very limited and the etiology of the central nervous system symptoms has therefore never been convincingly established. We describe a 27-year-old woman with LScs and a longstanding history of epilepsy. Radiographic studies demonstrated a focal, intraparenchymal lesion in the left frontal lobe directly subjacent to the area of scleroderma on the forehead and scalp. The resected cerebral lesion revealed localized band-like sclerosis of the leptomeninges and associated vessels, as well as intraparenchymal calcifications and anomalous, ectatic vessels. These findings suggest that LScs may represent a neurocutaneous syndrome of vascular dysplasia similar to the Sturge-Weber syndrome, rather than a localized form of collagen vascular disease, as suggested by some.

Adult↗
PubMed1984

Neuromuscular disorders in a new toxic syndrome: electrophysiological study--a preliminary report.

The electrophysiological features in 145 patients with a new toxic syndrome related to ingestion of adulterated oil are described. Myalgia, joint limitation, weight loss, cramps, progressive weakness and wasting, sensory disturbances that can be asymmetrical or patchy, and scleroderma-like changes were the main clinical features. The electrophysiological findings suggest that the neuromuscular impairment in the new toxic syndrome is a slowly progressive mixed axonal neuropathy, which starts asymmetrically in some patients, with involvement of proximal and distal muscles as well as paraspinal and respiratory muscles. Muscle and nerve biopsies confirm the neuropathy, and show severe perineuritis and perineurial and perimysial fibrosis. The single fiber electromyography (SFEMG) study showed increased motor unit fiber density directly related to the time after onset of the illness. Unstable complex potentials were found after 6 months of evolution, which suggests that an effective collateral reinnervation was delayed following a long period of progressive denervation.

Adolescent↗
PubMed1979

[D-Penacillamine. From constituent of penicillins to significant drug].

D-Penicillamine is used against a variety of diseases. For many years it has been successful in treating Wilson's disease, cystinuria and heavy-metal poisonings. It also proved to be effective against rheumatoid arthritis, scleroderma, chronic active hepatitis, pulmonary fibrosis and multiple sclerosis. However, the use of D-penicillamine is still limited owing to the frequent occurrence of considerable, though generally reversible, side effects. This article deals with the history of D-penicillamine as well as the methods of its synthesis, its pharmacokinetics, effects and side effects. In addition, the significance of the stereo isomeric L-penicillamine is discussed.

Arthritis, Rheumatoid↗
PubMed1996

Use of recombinant proteins in the diagnosis of autoimmune connective tissue diseases.

The use of recombinant proteins has been an invaluable tool for the study of the structure and function of cell components, and the analysis of autoimmune-mediated responses. Furthermore, it has also been instrumental in facilitating the development of reliable assays for the detection of a number of autoantibodies. This article discusses basic aspects of recombinant DNA technology, as well as its applications and limitations in the diagnosis of autoimmune connective tissue diseases such as systemic lupus erythematosus, Sjogren's syndrome, systemic scleroderma, mixed connective tissue disease, autoimmune inflammatory myopathies and primary vasculitides.

Autoantibodies↗
PubMed1997

Cost-of-illness of scleroderma: the case for rare diseases.

OBJECTIVE: To determine the societal costs of scleroderma (SSc), a rare chronic connective tissue disease that affects approximately 98,000 Americans. Lack of reliable national databases limit rare disease cost studies, and this study suggests methods of using multiple data sources to assess the costs of rare diseases. METHODS: Primary and secondary data sources were used to calculate direct and indirect costs of SSc, including discounted lifetime mortality and morbidity costs. A prevalence-based, human capital approach was used. Sensitivity analyses were used to vary parameters that are uncertain, such as prevalence, mortality, and labor costs. RESULTS: Annual direct and indirect costs of SSc in the United States are $1.5 billion. Morbidity represents the major cost burden, with costs of $819 million (56%) of total costs. The current value of lifetime earnings lost was $179 million (12%) or $300,000 per death. Direct costs were $462 million (32%) or $4,731 per person annually, indicating that costs are spread over the long disease duration. CONCLUSIONS: This study provides one model for the assessment of rare disease costs. Triangulation of data sources and sensitivity analyses are important for determining the costs of rare diseases. The high cost of SSc, despite its low prevalence, suggests that the burden of rare chronic diseases can be high. The high morbidity costs reflect the young age of onset of the disease as well as the need for treatments to decrease morbidity costs. Local shared databases and national surveys are needed to improve cost estimates of rare diseases.

Adolescent↗
PubMed1995

Juvenile scleroderma: report of a case.

Scleroderma is a rare connective tissue disease in children. A 12-year-old boy suffered from progressive increasing skin tension with erythematous changes in his left leg for a period of 3 months. This limited the range of motion in his left first and second metatarsophalangeal joints. A skin biopsy showed hypertrophic collagen bundles with atrophic skin appendages and lymphocytic infiltration. Based on the clinical manifestations and typical histopathologic findings, juvenile linear scleroderma was diagnosed. He was successfully treated with a short course of oral prednisolone in addition to long-term therapy with D-penicillamine and a topical emollient.

Child↗