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Ciclofosfamida: eficaz no tratamento do quadro cutâneo grave da esclerose sistêmica; Cyclophosphamide: effective in the treatment of severe cutaneous involvement in systemic sclerosis

MACEDO, Patrícia Andrade de; BORGES, Cláudia Teresa Lobato; SOUZA, Romy Beatriz Christmann de
Fonte: Elsevier Editora Ltda Publicador: Elsevier Editora Ltda
Tipo: Artigo de Revista Científica
POR
Relevância na Pesquisa
66.25%
INTRODUÇÃO: A esclerose sistêmica (ES) é uma doença reumatológica caracterizada por inflamação autoimune, vasculopatia e fibrose da pele e de vários órgãos. Poucos tratamentos são considerados eficazes para a doença com intenso envolvimento de pele como a ES na forma difusa. Portanto, avaliamos a eficácia da ciclofosfamida no tratamento de pacientes com ES difusa grave. PACIENTES E MÉTODOS: Nove pacientes com ES difusa (Critérios do Colégio Americano de Reumatologia [ACR]) com escore de Rodnan de pele modificado (ERM) > 30 (0-51) foram submetidos ao tratamento com ciclofosfamida (CFM) EV na dose de 0,5 a 1,0 g/m² mensal por 18 meses, totalizando 18 pulsos. Além da realização do ERM a cada seis meses, exames laboratoriais e avaliação de efeitos adversos foram também analisados. Pacientes com doença pulmonar e envolvimento cardíaco foram excluídos. RESULTADOS: A maioria dos pacientes era do sexo feminino (77%), com média de idade de 41,7 anos e tempo de doença de 2,2 anos. Observou-se redução significativa do ERM de 37,7 ± 4,08 para 29,1 ± 8,13 após 12 meses de tratamento (P = 0,009). Sete pacientes completaram 18 meses de CFM e com redução persistente do ERM (média ERM = 26,4; P = 0,01). Houve também uma redução da proteína C-reativa (PCR) (média inicial PCR = 8...

Diffuse systemic sclerosis and autoimmune hepatitis: a unique association

RODRIGUES, Carlos Ewerton Maia; BORGES, Claudia Lobato; CARVALHO, Jozelio Freire de
Fonte: SPRINGER LONDON LTD Publicador: SPRINGER LONDON LTD
Tipo: Artigo de Revista Científica
ENG
Relevância na Pesquisa
66.38%
Autoimmune hepatitis (AIH) is a chronic hepatitis of unknown etiology characterized by continuing hepatocellular necrosis and inflammation that afflicts 100,000 to 200,000 persons in the United States. It is a rare manifestation of systemic sclerosis. Only about nine reports of this association have been previously reported in the literature. Importantly, all cases had the limited clinical form of systemic. The authors describe herein the first report of a patient with diffuse systemic sclerosis who was diagnosed with AIH with positive antimitochondrial antibody and had an excellent response to immunosuppressive drugs. We also briefly review the literature regarding this issue.

Central Corneal Thickness in Patients With Systemic Sclerosis: A Controlled Study

GOMES, Beatriz de A. F.; SANTHIAGO, Marcony R.; KARA-JUNIOR, Newton; NOE, Rosangela A. M.; AZEVEDO, Mario N. L. de; MORAES JR., Haroldo V.
Fonte: LIPPINCOTT WILLIAMS & WILKINS Publicador: LIPPINCOTT WILLIAMS & WILKINS
Tipo: Artigo de Revista Científica
ENG
Relevância na Pesquisa
66.3%
Purpose: To compare the central corneal thickness (CCT) of patients with systemic sclerosis (SSc) and control subjects. Methods: The study group comprised 37 consecutive patients with SSc, and the control group comprised 23 healthy individuals similar in age and sex. CCT value was measured by ultrasound pachymetry. Results: In the SSc group, the mean CCT in the right eye was 534.9 +/- 33.5 mu m and 536.9 +/- 32.4 mu m in the left eye. In the control group, the mean CCT was 533.0 +/- 32.9 mu m in the right eye and 533.1 +/- 33.6 mu m in the left eye. The mean CCT was not significantly different in the SSc group compared with the control group for both the right (P = 0.83) and left (P = 0.67) eyes. Conclusions: CCT measurements do not significantly differ in patients with SSc compared with healthy control subjects.; Fundacao de Amparo a Pesquisa - RJ (FAPERJ)

Altered adhesion molecules expression on peripheral blood mononuclear cells from patients with systemic sclerosis and clinical correlations

SAWAYA, Heloisa Helena Braga; SOUZA, Romy Beatriz Christmann de; CARRASCO, Solange; GOLDENSTEIN-SCHAINBERG, Claudia
Fonte: SPRINGER Publicador: SPRINGER
Tipo: Artigo de Revista Científica
ENG
Relevância na Pesquisa
66.25%
The aim of the study was to evaluate the expressions of adhesion molecules (AM) on peripheral blood mononuclear cells (PBMNC) from systemic sclerosis (SSc) patients. Thirty-one SSc patients (ACR) and 20 normal subjects were selected for the study. PBMNC were analyzed for LFA-1 alpha, LFA-1 beta, ICAM-3, ICAM-1, and l-selectin expressions. ICAM-3 expression was decreased while ICAM-1 was increased on SSc PBMNC, compared to controls (p = 0.04 and 0.003, respectively). A positive association was found between LFA-1 alpha (r = 0.37, p = 0.03), LFA-1 beta (r = 0.38, p = 0.002), ICAM-3 (r = 0.42, p = 0.01), and l-selectin (r = 0.38, p = 0.03) expressions and greater number of immunosuppressive drugs taken by SSc patients. Also, anti-centromeric positive SSc patients had lower expressions of LFA-1 alpha, LFA-1 beta, ICAM-3, and l-selectin. Lower expression of ICAM-3 and higher expression of ICAM-1 suggest that AMs may be involved in the pathogenesis of scleroderma.

Centrilobular Fibrosis: An Underrecognized Pattern in Systemic Sclerosis

SOUZA, Romy B. C. de; BORGES, Claudia T. L.; CAPELOZZI, Vera L.; PARRA, Edwin R.; JATENE, Fabio B.; KAVAKAMA, Jorge; KAIRALLA, Ronaldo A.; BONFA, Eloisa
Fonte: KARGER Publicador: KARGER
Tipo: Artigo de Revista Científica
ENG
Relevância na Pesquisa
66.25%
Background: The impressive association of lung involvement and gastroesophageal reflux in scleroderma raises the possibility of a cause-effect relationship. Objectives: To determine clinical, radiological and histopathological features of systemic sclerosis (SSc) patients according the presence or absence of centrilobular fibrosis (CLF). Methods: Twenty-eight SSc patients with lung involvement were submitted to open lung biopsy and the specimens classified for the presence of CLF (bronchocentric distribution of the lesions and intraluminal matter according to the classification of idiopathic interstitial pneumonia). HRCT, pulmonary function tests and esophageal analysis were also performed. Subsequently, cyclophosphamide was introduced for the nonspecific interstitial pneumonia subgroup and antireflux treatment was intensified for isolated CLF patients. Results: Isolated CLF was found in 21% of the biopsies and also found associated to nonspecific interstitial pneumonia in 84% of these patients. The other 3 cases had usual interstitial pneumonia, pulmonary hypertension and respiratory bronchiolitis-associated interstitial lung disease. The histopathological analysis revealed that all 6 patients with isolated CLF had the bronchocentric distribution and intraluminal basophilic content...

Mandibular Function is Severely Impaired in Systemic Sclerosis Patients

FERREIRA, Edna Livia A.; CHRISTMANN, Romy B.; BORBA, Eduardo F.; BORGES, Claudia T. L.; SIQUEIRA, Jose T. T.; BONFA, Eloise
Fonte: QUINTESSENCE PUBLISHING CO INC Publicador: QUINTESSENCE PUBLISHING CO INC
Tipo: Artigo de Revista Científica
ENG
Relevância na Pesquisa
66.25%
Aims: To evaluate the presence of temporomandibular disorders (TMD) in systemic sclerosis (SSc) patients and its possible association with the severity of skin involvement. Methods: The presence of TMD was evaluated in 35 SSc women and 30 age- and sex-matched healthy controls by means of the anamnestic (A(i)) and clinical (D(i)) Helkimo indices; the jaw mobility was further analyzed (M(I)). Skin involvement was scored by the Modified Rodnan Skin Score (MRSS). Results: Signs and symptoms of TMD were more frequent in SSc patients than in controls, the frequency distribution of the different clinical dysfunction indices differing significantly (P < .001) between patients (D(i)0 8.6%, D(i)I 48.6%, D(i)II 22.8%, and D(i)III 20%) and controls (D(i)0 50%, D(i)I 33.3%, and D(i)II 16.7%). Cyclophosphamide for severe and rapidly progressive cutaneous fibrosis was prescribed in six out of seven patients with severe signs (D(i)III), in contrast this treatment was indicated for only two out of 25 patients with mild to moderate signs (D(i)I and D(i)II, P <. 001). Impaired jaw mobility was more frequent in SSc patients than controls (P < .001). It was severe in 77.1% (M(I)II) and mild in 22.9% (M(1)I) of the cases, in contrast to controls (M(I)0 33.4%...

Collagen V and vascular injury promote lung architectural changes in systemic sclerosis

PARRA, Edwin Roger; AGUIAR JR., Armando Costa; TEODORO, Walcy Rosolia; SOUZA, Romy de; YOSHINARI, Natalino Hajime; CAPELOZZI, Vera Luiza
Fonte: WILEY-BLACKWELL PUBLISHING, INC Publicador: WILEY-BLACKWELL PUBLISHING, INC
Tipo: Artigo de Revista Científica
ENG
Relevância na Pesquisa
66.35%
Background: Systemic sclerosis (SSc) is a multisystem disorder characterized by inflammation, fibrosis and vascular damage. The aim of this study was to evaluate the interactions between basement membrane disruption, endothelial injury and collagen V deposition on the vascular wall, as well as their association with pulmonary function tests in patients with SSc. Method: The endothelial apoptosis was assessed by TUNEL and electron microscopy, and quantified through the point-counting technique. To evaluate basement membrane integrity, laminin immunostaining and electron microscopy were used. Immunofluorescence and morphometric analysis were used to determine the amount of collagen V in the vascular walls in 23 open lung biopsies of patients with SSc without pulmonary hypertension. Normal lung tissue was obtained from five individuals who had died of traumatic injuries. Results: The apoptosis index in SSc was higher in the endothelial cells (13.83 +/- 6.83) when compared with the control (2.51 +/- 2.06) group (P < 0.001) and confirmed by electron microscopy. We observed an important disruption of the basement membrane on the vascular wall shown by discontinuous laminin immunostaining and electron microscopy. An increase in collagen V on the vascular wall of the SSc group was observed (45.28 +/- 13.21)...

Arterial and interstitial remodelling processes in non-specific interstitial pneumonia: systemic sclerosis versus idiopathic

CARVALHO, E. F. de; PARRA, E. R.; SOUZA, R. de; SABER, A. M. A`b; MACHADO, J. de Carvalho; CAPELOZZI, V. L.
Fonte: WILEY-BLACKWELL Publicador: WILEY-BLACKWELL
Tipo: Artigo de Revista Científica
ENG
Relevância na Pesquisa
66.25%
Aims: To compare septal and vascular matrix remodelling, vascular occlusion, Pulmonary function tests and survival between two groups: one with idiopathic non-specific interstitial pneumonia (NSIP) and one with NSIP associated with systemic sclerosis (SSc). Methods and results: Pulmonary biopsy specimens were examined from 40 patients, 22 with NSIP and 18 with NSIP associated with SSc. The content of septal collagen and elastic fibres, as well as the elastic fibres in the vascular interstitium, were higher in the SSc group (P = 0.01, P = 0.001 and P < 0.0001, respectively). Among pulmonary function tests. the diffusing capacity for carbon monoxide/alveolar volume was affected to a greater extent in the SSc group (59%) of the predicted value in SSc and 97% in the idiopathic group). There were no differences in collagen content of the vascular interstitium, arterial occlusion, or survival between the two groups. Conclusions: Although the fibrotic process is more intense in the SSc group. it, does not affect the prognosis of these patients. Because the elastotic process is higher in the SSc group, this might suggest that autoimmune inflammatory mechanisms affecting the elastic fibre system play a greater role in the pathogenesis and pulmonary remodelling process of SSc NSIP than in idiopathic NSIP.; National Council for Scientific and Technological Development [CNPq]; Foundation for the Support of Research of the State of Sao Paulo[FAPESP 2000/14336-0]; Universidade de São Paulo - Laboratories for Medical Research[LIM 05] - FM/USP; Clinicas Hospital...

Estudo da segurança e eficácia do exercício aeróbico em pacientes com esclerose sistêmica; Study of safety and efficacy of aerobic exercise in systemic sclerosis patients

Silva, Natália Cristina de Oliveira Vargas e
Fonte: Biblioteca Digitais de Teses e Dissertações da USP Publicador: Biblioteca Digitais de Teses e Dissertações da USP
Tipo: Tese de Doutorado Formato: application/pdf
Publicado em 02/06/2009 PT
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OBJETIVO: Diversos estudos demonstraram que pacientes com Esclerose Sistêmica possuem capacidade aeróbia reduzida. É relevante avaliar se o exercício aeróbio é seguro e eficaz para estes pacientes. MÉTODO: Sete pacientes com Esclerose Sistêmica e sete controles saudáveis participaram de um programa de oito semanas, que consistiu em atividade aeróbia de intensidade moderada duas vezes por semana. RESULTADOS: Pacientes e controles apresentaram aumento significativo no pico de consumo de oxigênio, e foram capazes de executar uma intensidade de exercício significativamente maior em relação ao pré-teste. O grupo Esclerose Sistêmica demonstrou aumento na saturação de oxigênio no pico do exercício. O escore de Rodnan foi similar antes e após a intervenção, e as úlceras digitais e o fenômeno de Raynaud permaneceram estáveis. CONCLUSÃO: O exercício aeróbio foi seguro e eficaz para pacientes com Esclerose Sistêmica, portanto, aumentar a capacidade aeróbia é uma meta possível no tratamento desta doença.; OBJECTIVES: Several studies have established that Systemic Sclerosis patients have a reduced exercise capacity. It is relevant to evaluate whether aerobic exercise consists in a safe and effective intervention for these patients. METHODS: Seven Systemic Sclerosis patients and seven healthy sedentary controls were enrolled in an eight-week program consisting of moderate intensity aerobic exercise twice a week. RESULTS: Systemic Sclerosis patients and controls had a significant improvement in their peak oxygen consumption and were able to perform a significantly higher exercise intensity when compared to baseline. Systemic sclerosis group improved peak exercise oxygen saturation. Rodnan score was similar before and after the intervention. Digital ulcers and Raynauds phenomenon remained stable. CONCLUSIONS: Aerobic exercise was safe and effective in patients with Systemic Sclerosis...

Esclerose sistêmica e níveis séricos elevados de organoclorado : uma associação possível?; Systemic sclerosis and elevated organochlorine blood levels : a possible association?

Monticielo, Odirlei André; Palominos, Penelope E.; Chakr, Rafael Mendonça da Silva; Bortoli, Rodrigo; Brenol, João Carlos Tavares; Xavier, Ricardo Machado
Fonte: Universidade Federal do Rio Grande do Sul Publicador: Universidade Federal do Rio Grande do Sul
Tipo: Artigo de Revista Científica Formato: application/pdf
POR
Relevância na Pesquisa
66.3%
A patogênese da esclerose sistêmica (ES) é complexa e pouco conhe¬cida. Há a participação de ativação imune, dano vascular e excessiva produção de matriz extracelular com deposição de colágeno estru¬turalmente normal. Fatores genéticos e ambientais contribuem para a expressão da doença nos pacientes. Dentre os fatores ambientais, diversos agentes químicos já foram associados à doença. Descrevemos o caso de uma adolescente de 16 anos, com história prévia de hepa¬tite criptogênica e câncer de lábio, que desenvolveu quadro atípico e rapidamente progressivo de esclerose sistêmica. Inicialmente o quadro era compatível com esclerodermia em placas, porém rapida¬mente evoluiu para uma forma sistêmica com grave acometimento cardiopulmonar e óbito. Durante a investigação de possíveis fatores etiológicos que pudessem estar relacionados, foram encontrados níveis séricos extremamente elevados de oxiclordano, um agrotóxico organoclorado. Uma possível correlação entre a intoxicação por esse agente químico e o surgimento de ES foi aventada.; The pathogenesis of systemic sclerosis (SS) is complex and not completely understood. Autoimmune mechanisms, vas¬cular damage, and excessive extracelular matrix with collagen deposition play a significant role. Genetic and environmental factors also are decisive to the disease expression. Among en¬vironmental factors many chemical agents have been associated with the development of SS. A 16-year-old white woman...

Correlation between serum E-selectin levels and panoramic nailfold capillaroscopy in systemic sclerosis

Valim,V.; Assis,L.S.S.; Simões,M.F.J.; Trevisani,V.F.M.; Pucinelli,M.L.C.; Andrade,L.E.C.
Fonte: Associação Brasileira de Divulgação Científica Publicador: Associação Brasileira de Divulgação Científica
Tipo: Artigo de Revista Científica Formato: text/html
Publicado em 01/09/2004 EN
Relevância na Pesquisa
66.41%
E-selectin is expressed by the activated endothelium and its plasma levels are increased in patients with systemic sclerosis. Eighteen patients fulfilling the American Rheumatism Association criteria for systemic sclerosis, 15 females and 3 males, 42-70 years old, 9 with diffuse and 9 with limited forms, were sequentially recruited for this study. Serum E-selectin levels were determined by commercially available ELISA and their association with nailfold capillaroscopic abnormalities was investigated. Nailfold capillaries were analyzed by 16X magnification wide-field capillaroscopy. Two parameters on capillaroscopy were used to correlate to serum E-selectin: deletion and ectasia. Data were analyzed statistically by the Student t-test and Spearman correlation. Two-tailed P values below 0.05 were considered significant. E-selectin range was 38 to 200 ng/ml (80 ± 39.94). There was a correlation between serum E-selectin levels and the deletion capillaroscopic score (r = 0.50, P < 0.035). This correlation was even stronger within the first 48 months of diagnosis (r = 0.63, P < 0.048). On the other hand, no association was observed between selectin and ectasia. Patients with diffuse disease presented higher serum E-selectin levels than patients with limited disease...

Angiotensin II type 1 and 2 receptors and lymphatic vessels modulate lung remodeling and fibrosis in systemic sclerosis and idiopathic pulmonary fibrosis

Parra,Edwin Roger; Ruppert,Aline Domingos Pinto; Capelozzi,Vera Luiza
Fonte: Faculdade de Medicina / USP Publicador: Faculdade de Medicina / USP
Tipo: Artigo de Revista Científica Formato: text/html
Publicado em 01/01/2014 EN
Relevância na Pesquisa
66.5%
OBJECTIVE: To validate the importance of the angiotensin II receptor isotypes and the lymphatic vessels in systemic sclerosis and idiopathic pulmonary fibrosis. METHODS: We examined angiotensin II type 1 and 2 receptors and lymphatic vessels in the pulmonary tissues obtained from open lung biopsies of 30 patients with systemic sclerosis and 28 patients with idiopathic pulmonary fibrosis. Their histologic patterns included cellular and fibrotic non-specific interstitial pneumonia for systemic sclerosis and usual interstitial pneumonia for idiopathic pulmonary fibrosis. We used immunohistochemistry and histomorphometry to evaluate the number of cells in the alveolar septae and the vessels stained by these markers. Survival curves were also used. RESULTS: We found a significantly increased percentage of septal and vessel cells immunostained for the angiotensin type 1 and 2 receptors in the systemic sclerosis and idiopathic pulmonary fibrosis patients compared with the controls. A similar percentage of angiotensin 2 receptor positive vessel cells was observed in fibrotic non-specific interstitial pneumonia and usual interstitial pneumonia. A significantly increased percentage of lymphatic vessels was present in the usual interstitial pneumonia group compared with the non-specific interstitial pneumonia and control groups. A Cox regression analysis showed a high risk of death for the patients with usual interstitial pneumonia and a high percentage of vessel cells immunostained for the angiotensin 2 receptor in the lymphatic vessels. CONCLUSION: We concluded that angiotensin II receptor expression in the lung parenchyma can potentially control organ remodeling and fibrosis...

Assessment of light touch sensation in the hands of systemic sclerosis patients

Silva,Paula Gabriel; Jones,Anamaria; Araujo,Pola Maria Poli; Natour,Jamil
Fonte: Faculdade de Medicina / USP Publicador: Faculdade de Medicina / USP
Tipo: Artigo de Revista Científica Formato: text/html
Publicado em 01/09/2014 EN
Relevância na Pesquisa
66.46%
INTRODUCTION: Systemic sclerosis is a relatively rare connective tissue disorder characterized by severe and progressive fibrosis of the skin. Due to the current lack of available information on this subject, the aim of the present study was to assess light touch sensations in the hands of patients with systemic sclerosis. METHODS: We completed a cross-sectional comparative study. Light touch sensations were evaluated in 30 individuals, including 15 patients with systemic sclerosis who exhibited changes in the dermis of their hands without loss of the distal phalanx and 15 subjects who did not exhibit changes in the upper limbs (control group). The groups were age- and sex-matched. Tactile sensory evaluations were performed using the Semmes-Weinstein monofilament test and the two-point discrimination test. RESULTS: Statistically significant differences were found between groups in the monofilament test. The study group had lower scores across all points of the hand when compared with the control group. Differences were also found when dominant and non-dominant hands were compared (p<0.05). Statistically significant differences were found between groups for a subset of the assessed points in the two-point discrimination test. CONCLUSIONS: The results of a monofilament test showed that tactile sensation...

Tanshinone IIA attenuates interleukin-17A-induced systemic sclerosis patient-derived dermal vascular smooth muscle cell activation via inhibition of the extracellular signal-regulated kinase signaling pathway

Liu,Mengguo; Yang,Ji; Li,Ming
Fonte: Faculdade de Medicina / USP Publicador: Faculdade de Medicina / USP
Tipo: Artigo de Revista Científica Formato: text/html
Publicado em 01/04/2015 EN
Relevância na Pesquisa
66.53%
OBJECTIVE: Salvia miltiorrhiza has long been used to treat systemic sclerosis. Tanshinone IIA, one of the phytochemicals derived from the roots of Salvia miltiorrhiza, exhibits multiple biological activities. The present study aimed to investigate whether tanshinone IIA has an effect on the interleukin-17A-induced functional activation of systemic sclerosis patient-derived dermal vascular smooth muscle cells. METHODS: Systemic sclerosis patient-derived dermal vascular smooth muscle cells were incubated with various dosages of tanshinone IIA in the presence of interleukin-17A or the serum of systemic sclerosis patients. Cell proliferation was assessed using Cell Counting Kit-8. The expression of collagen 1 and 3 in cells was evaluated by immunofluorescence. Cell migration was measured using a transwell assay. The expression of phospho-extracellular signal-regulated kinase was detected by Western blotting. RESULTS: Our data demonstrate that tanshinone IIA exerts an inhibitory effect on interleukin-17A-induced systemic sclerosis patient-derived dermal vascular smooth muscle cell proliferation, collagen synthesis and migration. CONCLUSION: These findings suggest that tanshinone IIA might serve as a promising therapeutic agent for the treatment of systemic sclerosis.

Breast cancer and systemic sclerosis: A clinical description of 21 patients in a population-based cohort study

Lu, T.; Hill, C.; Pontifex, E.; Roberts-Thomson, P.
Fonte: Springer-Verlag Publicador: Springer-Verlag
Tipo: Artigo de Revista Científica
Publicado em //2008 EN
Relevância na Pesquisa
66.41%
Previous studies have demonstrated an increased risk of breast cancer among patients with systemic sclerosis (scleroderma). To describe the clinical characteristics of 21 patients with both systemic sclerosis and breast cancer, and compare their risk factors to female scleroderma patients without breast cancer, in a population-based cohort study of South Australia. Subjects with scleroderma and breast cancer were identified from the South Australian Scleroderma Register with cross-linking to the South Australian Cancer Registry, last updated to the end of December 2005. Clinical information was obtained from standardised self-administered questionnaires and case note reviews. Odds ratios for the risk factors for breast cancer in scleroderma were determined, and clinical variables were analysed using chi square, Fisher’s exact, Mann–Whitney and t tests. At the end of December 2005 there were a total of 389 female patients with scleroderma. Of these, 21 (5.4%) had been diagnosed with breast cancer. The mean age of onset of scleroderma was 43.5 years, and the mean age of breast cancer was 60.5 years in those with scleroderma and breast cancer. The majority (71.4%) had limited scleroderma, with anti-centromere antibody being the most prevalent serological abnormality. In 16 (76%) patients the diagnosis of breast cancer occurred on an average of 22.3 years after the onset of their first scleroderma symptom. When compared to 48 controls...

Assessing the Activity of Agonistic Autoantibodies in Systemic Sclerosis and their Effects on Cultured Vascular Smooth Muscle Cells

Chokr, Nidaa
Fonte: Université de Montréal Publicador: Université de Montréal
Tipo: Thèse ou Mémoire numérique / Electronic Thesis or Dissertation
EN
Relevância na Pesquisa
66.3%
La sclérose systémique (ScS) est une maladie auto-immune dévastatrice d'étiologie inconnue. Le dysfonctionnement immunitaire, la fibrose et la vasculopathie sont les trois principales caractéristiques de cette maladie. Une récente étude a révélé un nouveau lien entre l'auto-immunité et la fibrose, par la présence d'auto-anticorps stimulant le récepteur du facteur de croissance dérivé des plaquettes (PDGFR) des fibroblastes. Ces auto-anticorps sont capables de stimuler les espèces réactives de l'oxygène et d’activer la kinase régulée par un signal extracellulaire (ERK1/2). L’hypothèse que nous formulons est que les cellules musculaires lisses vasculaires (VSMCs) exprimant conjointement les PDGFR, répondront elles aussi aux autoanticorps anti-PDGF-R. Le travail présenté ici vise à valider la présence d'auto-anticorps PDGFR dans les sérums de patients ScS, et à caractériser ensuite la réponse de VSMCs exposées à de l'immunoglobuline G (IgG) de ces sérums, en mesurant l’activation des cascades de signalisation spécifiques, ainsi que l'induction des gènes impliqués dans la réponse fibrotique. Nos résultats démontrent la présence d'une fraction IgG stimulant une réponse phénotypique dans les cultures de VSMCs. Notamment...

Systemic sclerosis sine scleroderma associated with antiphospholipid syndrome

Leite, Pollyanna d'Avila; de Carvalho, Jozelio Freire
Fonte: SPRINGER HEIDELBERG; HEIDELBERG Publicador: SPRINGER HEIDELBERG; HEIDELBERG
Tipo: Artigo de Revista Científica
ENG
Relevância na Pesquisa
66.3%
The antiphospholipid syndrome (APS) can be primary, when it occurs alone, or secondary, when it is associated with another autoimmune disease, mainly systemic lupus erythematosus and rarely other autoimmune diseases. Cases described in literature (Medline 1966 to December 2009) associate the presence of antiphospholipid antibodies with the presence of APS and systemic sclerosis (SS). Currently, however, no cases of the SS variant sine scleroderma with APS have been described. In this study, the authors describe the case of a patient with APS characterised by thrombosis of the retinal veins, in May 2006, the presence of lupus anticoagulant and an anticardiolipin IgG antibody. In May 2007, this patient developed Raynaud's phenomenon, a lack of oesophageal motility and nailfold capillaroscopy with a scleroderma pattern. The patient was positive for the anti-centromere antibody but lacked any evidence of cutaneous thickening or involvement. In summary, the authors describe the first case of a patient with APS associated with SS sine scleroderma.; Federico Foundation; Federico Foundation; CNPq; CNPq [300665/2009-1]

Ocular findings in patients with systemic sclerosis

GOMES, Beatriz de A. F; SANTHIAGO, Marcony R; MAGALHÃES, Priscilla; KARA-JUNIOR, Newton; AZEVEDO, Mário N. L. de; MORAES JR, Haroldo V
Fonte: Faculdade de Medicina / USP Publicador: Faculdade de Medicina / USP
Tipo: Artigo de Revista Científica
ENG
Relevância na Pesquisa
66.52%
OBJECTIVE: To evaluate the frequency and characteristics of ocular manifestations in outpatients with systemic sclerosis. METHODS: In this cross-sectional study, 45 patients with systemic sclerosis were enrolled. Data regarding demographics, disease duration and subtype, age at diagnosis, nailfold capillaroscopic pattern and autoantibody profile were collected, and a full ophthalmic examination was conducted. Parametric (Student's t-test) and nonparametric (Mann-Whitney U test) tests were used to compare continuous variables. Fisher's exact test was used to compare categorical data. P values < 0.05 were considered significant. RESULTS: Twenty-three subjects (51.1%) had eyelid skin changes; 22 (48.9%) had keratoconjunctivitis sicca, 19 (42.2%) had cataracts, 13 (28.9%) had retinal microvascular abnormalities and 6 (13.3%) had glaucoma. Eyelid skin changes were more frequent in patients with the diffuse subtype of systemic sclerosis and were associated with a younger age and an earlier age at diagnosis. Cataracts were presumed to be age-related and secondary to corticosteroid treatment. There was no association between demographic, clinical or serological data and keratoconjunctivitis sicca. The retinal microvascular abnormalities were indistinguishable from those related to systemic hypertension and were associated with an older age and a severe capillaroscopic pattern. CONCLUSIONS: Eyelid skin abnormalities and keratoconjunctivitis sicca were the most common ocular findings related to systemic sclerosis. Some demographic and clinical data were associated with some ophthalmic features and not with others...

Ocular findings in patients with systemic sclerosis

Gomes,Beatriz de A. F; Santhiago,Marcony R; Magalhães,Priscilla; Kara-Junior,Newton; Azevedo,Mário N. L. de; Moraes Jr,Haroldo V
Fonte: Faculdade de Medicina / USP Publicador: Faculdade de Medicina / USP
Tipo: Artigo de Revista Científica Formato: text/html
Publicado em 01/01/2011 EN
Relevância na Pesquisa
66.52%
OBJECTIVE: To evaluate the frequency and characteristics of ocular manifestations in outpatients with systemic sclerosis. METHODS: In this cross-sectional study, 45 patients with systemic sclerosis were enrolled. Data regarding demographics, disease duration and subtype, age at diagnosis, nailfold capillaroscopic pattern and autoantibody profile were collected, and a full ophthalmic examination was conducted. Parametric (Student's t-test) and nonparametric (Mann-Whitney U test) tests were used to compare continuous variables. Fisher's exact test was used to compare categorical data. P values < 0.05 were considered significant. RESULTS: Twenty-three subjects (51.1%) had eyelid skin changes; 22 (48.9%) had keratoconjunctivitis sicca, 19 (42.2%) had cataracts, 13 (28.9%) had retinal microvascular abnormalities and 6 (13.3%) had glaucoma. Eyelid skin changes were more frequent in patients with the diffuse subtype of systemic sclerosis and were associated with a younger age and an earlier age at diagnosis. Cataracts were presumed to be age-related and secondary to corticosteroid treatment. There was no association between demographic, clinical or serological data and keratoconjunctivitis sicca. The retinal microvascular abnormalities were indistinguishable from those related to systemic hypertension and were associated with an older age and a severe capillaroscopic pattern. CONCLUSIONS: Eyelid skin abnormalities and keratoconjunctivitis sicca were the most common ocular findings related to systemic sclerosis. Some demographic and clinical data were associated with some ophthalmic features and not with others...

Ocular findings in patients with systemic sclerosis

Gomes, Beatriz de A. F; Santhiago, Marcony R; Magalhães, Priscilla; Kara-Junior, Newton; Azevedo, Mário N. L. de; Moraes Jr, Haroldo V
Fonte: Universidade de São Paulo. Faculdade de Medicina Publicador: Universidade de São Paulo. Faculdade de Medicina
Tipo: info:eu-repo/semantics/article; info:eu-repo/semantics/publishedVersion; ; ; ; ; ; Formato: application/pdf
Publicado em 01/01/2011 ENG
Relevância na Pesquisa
66.52%
OBJECTIVE: To evaluate the frequency and characteristics of ocular manifestations in outpatients with systemic sclerosis. METHODS: In this cross-sectional study, 45 patients with systemic sclerosis were enrolled. Data regarding demographics, disease duration and subtype, age at diagnosis, nailfold capillaroscopic pattern and autoantibody profile were collected, and a full ophthalmic examination was conducted. Parametric (Student's t-test) and nonparametric (Mann-Whitney U test) tests were used to compare continuous variables. Fisher's exact test was used to compare categorical data. P values < 0.05 were considered significant. RESULTS: Twenty-three subjects (51.1%) had eyelid skin changes; 22 (48.9%) had keratoconjunctivitis sicca, 19 (42.2%) had cataracts, 13 (28.9%) had retinal microvascular abnormalities and 6 (13.3%) had glaucoma. Eyelid skin changes were more frequent in patients with the diffuse subtype of systemic sclerosis and were associated with a younger age and an earlier age at diagnosis. Cataracts were presumed to be age-related and secondary to corticosteroid treatment. There was no association between demographic, clinical or serological data and keratoconjunctivitis sicca. The retinal microvascular abnormalities were indistinguishable from those related to systemic hypertension and were associated with an older age and a severe capillaroscopic pattern. CONCLUSIONS: Eyelid skin abnormalities and keratoconjunctivitis sicca were the most common ocular findings related to systemic sclerosis. Some demographic and clinical data were associated with some ophthalmic features and not with others...