Your browser doesn't support javascript.
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 176
Filtrar
1.
Biomarkers ; 24(4): 373-378, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30821519

RESUMO

Objective: The objective was to investigate blood-based biomarkers of type I (PRO-C1), III (PRO-C3) and VI (PRO-C6) collagen formation in systemic sclerosis (SSc) patients and examine their correlation to modified Rodnan skin score (mRSS). Methods: Limited (lSSc, n = 76) and diffuse SSc (dSSc, n = 41) fulfilling the ACR/EULAR 1980 and 2013 classification criteria for SSc and asymptomatic controls (n = 9) were included. PRO-C1, PRO-C3 and PRO-C6 were measured in serum. Results: LSSc compared to dSSc were significantly older, had longer disease duration and lower mRSS. PRO-C3 was higher in early dSSc compared to early lSSc (mean [95 percentile], 27.4 [13.1-39.1] ng/mL vs 14.9 [8.2-28.8] ng/mL, p = 0.006). PRO-C6 levels were higher in early dSSc compared to early lSSc and late dSSc (early dSSc: 28.2 [10.4-92.3] ng/ml vs early lSSc: 11.0 [6.9-28.5] ng/ml; p = 0.006 and late dSSc: 12.6 [6.5-25.3] ng/mL, p = 0.04). No difference was observed with PRO-C1. PRO-C3 and PRO-C6 were moderately correlated with mRSS with R-partials of 0.36 (p < 0.001) and 0.29 (p = 0.002), respectively Conclusion: Measures of type III and VI collagen formation are potential objective biomarkers of fibrosis in systemic sclerosis. These biomarkers could be useful in monitoring the disease and efficacy of treatment.


Assuntos
Colágeno Tipo III/sangue , Colágeno Tipo VI/sangue , Esclerodermia Difusa/diagnóstico , Esclerodermia Limitada/diagnóstico , Adulto , Idoso , Biomarcadores/sangue , Estudos Transversais , Diagnóstico Diferencial , Feminino , Fibrose , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Esclerodermia Difusa/sangue , Esclerodermia Difusa/patologia , Esclerodermia Limitada/sangue , Esclerodermia Limitada/patologia , Índice de Gravidade de Doença , Pele/metabolismo
2.
Int J Rheum Dis ; 21(5): 1082-1092, 2018 May.
Artigo em Inglês | MEDLINE | ID: mdl-29673120

RESUMO

INTRODUCTION: Data regarding the incidence rate (IR) of cardiopulmonary involvement in comparison between late-onset SSc and early-onset SSc are limited. OBJECTIVE: To compare the prevalence of clinical manifestations and the IR of cardiopulmonary involvement compared between the two subgroups. METHODS: An inception cohort of SSc patients seen at the Rheumatology Clinic, Maharaj Nakorn Chiang Mai Hospital, between January 2010 and June 2016, was used. All patients were assessed for clinical manifestations and underwent electrocardiograph, echocardiography and high-resolution computed tomography at the study entry and every 12 months thereafter. RESULT: One hundred and fifteen patients (69 female and 90 diffuse cutaneous SSc [dcSSc]) with a mean (SD) disease duration of 11.6 months (8.8) at cohort entry were enrolled during a mean (SD) observation period of 3.8 years (1.6). Patients were classified into two groups: age ≥ 50 years (late onset) and age < 50 years (early onset). The late-onset group included 78 patients (67.8%). At enrollment, the late-onset group had higher prevalence of digital pitting scars (60.3% vs. 35.1%, P = 0.012), dry eye symptoms (17.9% vs. 2.7%, P = 0.035), and hypertension (20.5% vs. 5.4%, P = 0.037) compared to the early-onset group. In the last visit, it was found that the late-onset group had higher cumulative prevalence of joint contracture (61.5% vs. 37.8%, P = 0.017) compared to the early-onset group. The late-onset group had no significant IR of left ventricular ejection fraction < 50% (3.04 vs. 4.45 per 100 person-years, P = 0.486), right ventricular dysfunction (5.17 vs. 2.73 per 100 person-years, P = 0.269), interstitial lung disease (49.45 vs. 42.03 per 100 person-years, P = 0.462), and systolic pulmonary arterial pressure ≥ 50 mmHg (2.57 vs. 1.07 per 100 person-years, P = 0.267) compared to the early-onset group. CONCLUSION: Our study cohort found that digital pitting scar, xerophthalmia, hypo-hyperpigmentation, joint contracture, and hypertension are more prevalent in late-onset SSc than early-onset SSc. However, no significant differences regarding the IR of cardiopulmonary involvement between the two subgroups, the majority of which were dcSSc, in the early phase of the disease.


Assuntos
Cardiopatias/epidemiologia , Pneumopatias/epidemiologia , Esclerodermia Difusa/epidemiologia , Adolescente , Adulto , Idade de Início , Pressão Arterial , Criança , Estudos de Coortes , Feminino , Cardiopatias/diagnóstico por imagem , Cardiopatias/fisiopatologia , Humanos , Incidência , Pneumopatias/diagnóstico por imagem , Pneumopatias/fisiopatologia , Masculino , Pessoa de Meia-Idade , Prevalência , Prognóstico , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/fisiopatologia , Esclerodermia Difusa/diagnóstico , Volume Sistólico , Tailândia/epidemiologia , Fatores de Tempo , Função Ventricular Esquerda , Função Ventricular Direita , Adulto Jovem
3.
J Clin Pathol ; 71(7): 620-625, 2018 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-29447111

RESUMO

AIM: Humoral immunity and B cells are thought to play an important role in the pathophysiology of the systemic sclerosis (SSc). The production of free light chains (FLC) of immunoglobulins is abnormally high in several pathological autoimmune conditions and reflects B cell activation. Furthermore, FLCs demonstrated different biological activities including their capability to modulate the immune system, proteolytic activity and complement cascade activation. The aims of this study are to determine the FLC levels in patients with SSc compared with healthy controls (HC) and to study their possible association with organ involvement and disease characteristics. METHODS: Sixty-five patients with SSc and 20 HC were studied. Clinical and immunological inflammatory characteristics were assessed for all the patients with SSc. κ-FLC and λ-FLC, interleukin 6 (IL-6) and B cell activating factor levels were measured. RESULTS: The mean serum κ-FLC levels and FLC ratio were significantly higher in patients with SSc compared with HC, while the serum λ-FLC levels were comparable.The levels of FLC were comparable in patients with diffuse skin disease and limited skin involvement, while κ-FLC levels were increased in patients with restrictive lung (forced vital capacity (FVC) <80%) disease (26.4±7.4 mg/L) when compared with patients with FVC ≥80% (19.6±7.3 mg/L, P=0.009). In patients with SSc, the levels of serum κ-FLC level directly correlated with the IL-6 levels (R=0.3, P=0.001) and disease activity (R=0.4, P=0.003). CONCLUSIONS: FLC levels are elevated in SSc and high levels are associated with lung involvement and with a higher degree of inflammation, supporting a possible role of B cell activation in the pathophysiology of the disease.


Assuntos
Cadeias kappa de Imunoglobulina/sangue , Cadeias lambda de Imunoglobulina/sangue , Inflamação/sangue , Pulmão/imunologia , Esclerodermia Difusa/sangue , Esclerodermia Limitada/sangue , Adulto , Idoso , Fator Ativador de Células B/sangue , Fator Ativador de Células B/imunologia , Linfócitos B/imunologia , Linfócitos B/metabolismo , Biomarcadores/sangue , Estudos de Casos e Controles , Feminino , Volume Expiratório Forçado , Humanos , Cadeias kappa de Imunoglobulina/imunologia , Cadeias lambda de Imunoglobulina/imunologia , Inflamação/diagnóstico , Inflamação/imunologia , Interleucina-6/sangue , Interleucina-6/imunologia , Pulmão/fisiopatologia , Ativação Linfocitária , Masculino , Pessoa de Meia-Idade , Esclerodermia Difusa/diagnóstico , Esclerodermia Difusa/imunologia , Esclerodermia Difusa/fisiopatologia , Esclerodermia Limitada/diagnóstico , Esclerodermia Limitada/imunologia , Esclerodermia Limitada/fisiopatologia , Regulação para Cima , Capacidade Vital
4.
Rheumatology (Oxford) ; 57(5): 813-817, 2018 05 01.
Artigo em Inglês | MEDLINE | ID: mdl-29415230

RESUMO

Objective: To gain insight into clinical practice regarding referral, early diagnosis and other aspects of the management of patients with dcSSc in Europe and the USA. Methods: Semi-structured interviews were conducted with 84 rheumatologists (or internal medicine physicians) and 40 dermatologists in different countries (the UK, France, Germany, Italy, Spain and the USA). Physicians were asked to identify key steps in the patient pathway relating to patient presentation, diagnosis and referral, in addition to other treatment and follow-up processes. Results: The interviewed physicians reported that late presentation with dcSSc was common, with some patients presenting to primary care physicians after symptoms had persisted for up to 1 year. Awareness of dcSSc is reported to vary widely among primary care physicians. Final diagnosis, generally following guideline-based recommendations, was by rheumatologists in most cases (or internal medicine physicians in France) and they remained responsible for global patient management, with lesser involvement in diagnosis and management by dermatologists. Specialist centres were not well defined and did not exist in all countries. Conclusion: Patients and primary healthcare providers can be unaware of the symptoms of dcSSc, therefore presentation and referral to specialist care are often late. Thus, improved awareness among patients and primary care physicians is necessary to facilitate earlier referral and diagnosis. Once referred, more consistent use of the modified Rodnan skin score at diagnosis and follow-up may help to monitor disease progression. Furthermore, establishing specialist centres may help to promote such changes and improve patient care.


Assuntos
Competência Clínica , Gerenciamento Clínico , Diagnóstico Precoce , Qualidade da Assistência à Saúde/normas , Encaminhamento e Consulta/tendências , Reumatologistas/normas , Esclerodermia Difusa/diagnóstico , Adulto , Europa (Continente) , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Fatores de Risco , Esclerodermia Difusa/terapia , Inquéritos e Questionários , Fatores de Tempo
5.
Rheumatol Int ; 38(3): 363-374, 2018 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-29322341

RESUMO

OBJECTIVES: To evaluate the clinical characteristics of patients with interstitial lung disease (ILD) in the setting of a large cohort of systemic sclerosis (SSc) patients, and to analyse the differences according to the SSc subtype (following the modification of classification criteria of the American College of Rheumatology for SSc proposed by LeRoy and Medsger), factors are associated with moderate-to-severe impairment of lung function, as well as mortality and causes of death. METHODS: A descriptive study was performed, using the available data from the Spanish Scleroderma Study Group. RESULTS: Twenty-one referral centers participated in the registry. By April 2014, 1374 patients with SSc had been enrolled, and 595 of whom (43%) had ILD: 316 (53%) with limited cutaneous SSc (lcSSc), 240 (40%) with diffuse cutaneous SSc (dcSSc), and 39 (7%) with SSc sine scleroderma (ssSSc). ILD in the lcSSc and the ssSSc subsets tended to develop later, and showed a less impaired forced vital capacity (FVC) and a ground glass pattern on high-resolution computed tomography (HRCT) less frequently, compared with the dcSSc subset. Factors related to an FVC < 70% of predicted in the multivariate analysis were: dcSSc, positivity to anti-topoisomerase I antibodies, a ground glass pattern on HCRT, an active nailfold capillaroscopy pattern, lower DLco, older age at symptoms onset, and longer time between symptoms onset and ILD diagnosis. Finally, SSc-associated mortality and ILD-related mortality were highest in dcSSc patients, whereas that related to pulmonary arterial hypertension was highest in those with lcSSc-associated ILD. CONCLUSIONS: Our study indicates that ILD constitutes a remarkable complication of SSc with significant morbidity and mortality, which should be borne in mind in all three subgroups (lcSSc, dcSSc, and ssSSc).


Assuntos
Doenças Pulmonares Intersticiais , Pulmão , Esclerodermia Difusa , Esclerodermia Limitada , Adulto , Idoso , Causas de Morte , Distribuição de Qui-Quadrado , Feminino , Cardiopatias/mortalidade , Cardiopatias/fisiopatologia , Humanos , Hipertensão Pulmonar/mortalidade , Hipertensão Pulmonar/fisiopatologia , Modelos Logísticos , Pulmão/diagnóstico por imagem , Pulmão/patologia , Pulmão/fisiopatologia , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/mortalidade , Doenças Pulmonares Intersticiais/fisiopatologia , Doenças Pulmonares Intersticiais/terapia , Masculino , Angioscopia Microscópica , Pessoa de Meia-Idade , Análise Multivariada , Razão de Chances , Prevalência , Prognóstico , Sistema de Registros , Fatores de Risco , Esclerodermia Difusa/diagnóstico , Esclerodermia Difusa/mortalidade , Esclerodermia Difusa/fisiopatologia , Esclerodermia Difusa/terapia , Esclerodermia Limitada/diagnóstico , Esclerodermia Limitada/mortalidade , Esclerodermia Limitada/fisiopatologia , Esclerodermia Limitada/terapia , Índice de Gravidade de Doença , Pele/patologia , Espanha/epidemiologia , Tomografia Computadorizada por Raios X , Capacidade Vital
6.
Int J Rheum Dis ; 20(10): 1551-1561, 2017 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-28952189

RESUMO

OBJECTIVE: The aim of the current study was to evaluate if methylation status of CpG sites of interferon regulatory factor 7 (IRF7) promoter in peripheral blood mononuclear cells (PBMCs) of systemic sclerosis (SSc) patients is involved in pathogenesis of the disease. METHODS: PBMCs were isolated from whole blood of 50 SSc patients and 30 controls. After the extraction of total RNA and DNA contents from PBMCs, complementary DNA (cDNA) was synthesized. Afterwards, quantitative analysis of IRF7 messenger RNA (mRNA) was conducted by real-time polymerase chain reaction (PCR). To evaluate the methylation status of the promoter region of IRF7 gene, PCR products of bisulfite-treated DNA from SSc patients and controls were sequenced. RESULTS: The mRNA expression of IRF7 in PBMCs from patients compared with controls was significantly upregulated. While limited cutaneous SSc patients expressed the mRNA of IRF7 higher than controls, the diffuse cutaneous SSc group did not demonstrate significantly increased expression in comparison to controls. Insignificant promoter hypomethylation of IRF7 was observed in SSc patients compared with the control group. However, CpG2 hypomethylation was significantly associated with increased SSc risk. Furthermore, overall promoter methylation and mRNA level of IRF7 were significantly correlated with each other. Nonetheless, none of them correlated with Rodnan score of SSc patients. There was significant difference in IRF7 mRNA expression between CpG8 methylated and unmethylated SSc patients. Moreover, the difference of methylation and expression was not significant between anti-nuclear antibody (ANA)-positive and ANA-negative SSc patients. CONCLUSIONS: It is suggested that hypomethylation of the IRF7 promoter might play a role in SSc pathogenesis, probably through promoting the IRF7 expression in PBMCs of patients with SSc.


Assuntos
Metilação de DNA , Epigênese Genética , Fator Regulador 7 de Interferon/genética , Regiões Promotoras Genéticas , Esclerodermia Difusa/genética , Esclerodermia Limitada/genética , Escleroderma Sistêmico/genética , Adulto , Estudos de Casos e Controles , Ilhas de CpG , Feminino , Estudos de Associação Genética , Humanos , Fator Regulador 7 de Interferon/sangue , Leucócitos Mononucleares/química , Masculino , Pessoa de Meia-Idade , RNA Mensageiro/sangue , RNA Mensageiro/genética , Esclerodermia Difusa/sangue , Esclerodermia Difusa/diagnóstico , Esclerodermia Limitada/sangue , Esclerodermia Limitada/diagnóstico , Escleroderma Sistêmico/sangue , Escleroderma Sistêmico/diagnóstico
7.
BMC Cardiovasc Disord ; 17(1): 187, 2017 07 17.
Artigo em Inglês | MEDLINE | ID: mdl-28716007

RESUMO

BACKGROUND: Systemic sclerosis (SSc) is an autoimmune disease characterized by microvascular abnormalities, inflammation and fibrosis. We hypothesized that myocarditis may be diagnosed in asymptomatic SSc, undergoing routine cardio-vascular magnetic resonance (CMR) for fibrosis assessment, using the Lake Louise criteria: T2 ratio, early (EGE) and late gadolinium enhanced (LGE) images. METHODS: Eighty-two asymptomatic SSc, diagnosed according to American College of Rheumatology criteria, aged 43 ± 5 yrs., 62 with diffuse (dSSc) and 20 with localized (lSSc) systemic sclerosis were evaluated by CMR, performed at 1.5 T scanner, according to Lake Louise criteria. RESULTS: CMR documented normal biventricular function in all SSc. However, 7/62 (11.2%) with dSSc and 2/20 (10%) with lSSc, had CMR signs of myocarditis according to Lake Louise criteria, without any clinical cardiac symptom. In these 9 patients, T2 ratio, EGE ratio and LGE (positive in all 9 SSc) were 2.8 ± 0.5%, 8 ± 3% and 5 ± 3% of LV mass, respectively. No correlation between CMR and blood inflammatory indices (C-reactive protein and erythrocyte sedimentation rate), cardiac troponin T, disease characteristics or type of SSc was identified. A repeat CMR at 6 months, after treatment with prednisone and azathioprine, showed normalisation of the acute inflammation CMR indices. CONCLUSIONS: Silent myocarditis may be diagnosed using the Lake Louise paper criteria in SSc patients without cardiac symptoms, has no correlation with blood inflammatory indices, cardiac troponin or disease characteristics. CMR is a promising tool to diagnose silent myocarditis in SSc and monitor the response to immunosuppressive treatment.


Assuntos
Imagem por Ressonância Magnética , Miocardite/diagnóstico por imagem , Esclerodermia Difusa/complicações , Esclerodermia Limitada/complicações , Adulto , Doenças Assintomáticas , Biomarcadores/sangue , Sedimentação Sanguínea , Proteína C-Reativa/metabolismo , Meios de Contraste/administração & dosagem , Feminino , Fibrose , Gadolínio DTPA/administração & dosagem , Humanos , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Miocardite/tratamento farmacológico , Miocardite/etiologia , Miocardite/fisiopatologia , Miocárdio/patologia , Valor Preditivo dos Testes , Esclerodermia Difusa/diagnóstico , Esclerodermia Difusa/tratamento farmacológico , Esclerodermia Limitada/diagnóstico , Esclerodermia Limitada/tratamento farmacológico , Fatores de Tempo , Resultado do Tratamento , Troponina T/sangue , Função Ventricular Esquerda , Função Ventricular Direita
8.
Microvasc Res ; 114: 41-45, 2017 11.
Artigo em Inglês | MEDLINE | ID: mdl-28602918

RESUMO

BACKGROUND: The angiogenesis in systemic sclerosis (SSc) is impaired. An imbalance of pro-angiogenic factors and angiogenesis inhibitors has been implicated in the progression of peripheral microvascular damage, defective vascular repair and fibrosis. Intrarenal resistance index are considered markers of renal vasculopathy. The aim of the study is to evaluate angiogenic and angiostatic factors (VEGF and endostatin) in SSc patients and to correlate with intrarenal hemodynamic parameters. METHODS: 91 SSc patients were enrolled in this study. Serum VEGF and endostatin levels were determined. All patients underwent a renal Doppler ultrasound RESULTS: A significant positive correlation was observed between endostatin and renal Doppler parameters (p<0.0001). A negative correlation was observed between serum levels of endostatin and eGFR (p<0.01). In SSc patients with high resistive index, serum levels of endostatin were significantly (p<0.01) higher than in SSc patients with normal resistive index. The serum levels of endostatin significantly increased with progression of nailfold videocapillaroscopy damage (p<0.01) and were significantly (p<0.05) higher in SSc patients with digital ulcers than in SSc patients without digital ulcers. CONCLUSION: This is the first study that assess in SSc patients intrarenal hemodynamic parameters and endostatin. In SSc patients, endostatin represents a marker of renal scleroderma-associated vasculopathy.


Assuntos
Endostatinas/sangue , Hemodinâmica , Nefropatias/sangue , Rim/irrigação sanguínea , Circulação Renal , Esclerodermia Difusa/sangue , Esclerodermia Limitada/sangue , Doenças Vasculares/sangue , Fator A de Crescimento do Endotélio Vascular/sangue , Adulto , Idoso , Biomarcadores/sangue , Estudos de Casos e Controles , Feminino , Taxa de Filtração Glomerular , Humanos , Nefropatias/diagnóstico , Nefropatias/fisiopatologia , Masculino , Angioscopia Microscópica , Pessoa de Meia-Idade , Neovascularização Patológica , Esclerodermia Difusa/diagnóstico , Esclerodermia Difusa/fisiopatologia , Esclerodermia Limitada/diagnóstico , Esclerodermia Limitada/fisiopatologia , Ultrassonografia Doppler , Doenças Vasculares/diagnóstico , Doenças Vasculares/fisiopatologia
9.
Ind Health ; 55(3): 275-284, 2017 Jun 08.
Artigo em Inglês | MEDLINE | ID: mdl-28321017

RESUMO

Japanese women now account for 43 percent of the labor force. A number of them are involved in construction, agricultural and forestry jobs. The aim of this study was to establish a non-invasive technique for the evaluation of peripheral circulatory functions in women with Raynaud's phenomenon (RP) and introduce a specific method for the assessment of vascular disturbances in females exposed to hand-transmitted vibration. The subjects of this study were 10 women with primary RP, 7 women with progressive systemic sclerosis (PSS) secondary to RP, and 17 females who were included as the control group. The evaluation of peripheral circulatory functions in all subjects was based on the values of finger blood flow (FBF) and finger skin temperature (FST) measured before, during and following a 5-min recovery period after the hand was immersed in cold water (5°C, 1 min). The measured values of FBF and FST of the primary RP group before and after the immersion test were significantly (p<0.01) lower compared to those of the control group. The technique applied in this study could be used as a non-invasive and tolerable technique to determine the digital circulatory functions in female subjects with RP.


Assuntos
Dedos/irrigação sanguínea , Fluxometria por Laser-Doppler/métodos , Doença de Raynaud/diagnóstico , Temperatura Cutânea , Adulto , Temperatura Baixa/efeitos adversos , Feminino , Mãos , Humanos , Japão , Doença de Raynaud/complicações , Esclerodermia Difusa/complicações , Esclerodermia Difusa/diagnóstico
10.
Int J Rheum Dis ; 20(6): 767-773, 2017 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-28261995

RESUMO

AIM: Cutaneous involvement is an early manifestation of systemic sclerosis (SSc). Localized areas of 'salt and pepper skin' (S&P) may develop. We hypothesize that S&P skin occurs frequently in diffuse cutaneous (dc) SSc which can be used in its early diagnosis and may correlate with joint contractures. METHODS: Sixty-five patients were recruited for this study. The demographic profiles of SSc were ascertained from hospital records. These patients fulfilled the 2013 American College of Rheumatology/European League Against Rheumatism classification criteria. Patients were examined for skin pigmentary changes, modified Rodnan skin score (mRSS), telengiectasias, calcinosis, arthritis and joint contractures and pruritus. RESULTS: Sixty-five patients (59 female) were recruited with median age of 62.87 years. Forty-four had limited cutaneous SSc, 16 dcSSc, five had scleroderma overlap syndrome. Multivariate stepwise logistic regression indicated that mRSS severity and the presence of contractures were independently (P < 0.05) associated with dcSSc. The strong positive association between S&P and mRSS severity may explain the non-significance of S&P in this analysis. If mRSS severity is not included in the logistic regression analysis, the presence of contractures and S&P (odds ratio = 15.1) show significant (P < 0.01) independent associations with the dcSSc subtype. S&P skin and pruritus were similar in patients with Scl-70 and anti-RNA polymerase antibodies. Anti-centromere antibodies were negatively associated with S&P (χ2 = 7.89, P = 0.005). CONCLUSION: Our study demonstrates strong association of S&P skin with dcSSc (69%), increased risk of pruritus and contractures. Its presence can be used as another clinical tool to diagnose dcSSc in early stages. Observing for S&P skin changes does not require much training.


Assuntos
Hiperpigmentação/etiologia , Hipopigmentação/etiologia , Esclerodermia Difusa/complicações , Pigmentação da Pele , Adulto , Idoso , Idoso de 80 Anos ou mais , Distribuição de Qui-Quadrado , Diagnóstico Precoce , Feminino , Humanos , Hiperpigmentação/diagnóstico , Hiperpigmentação/fisiopatologia , Hipopigmentação/diagnóstico , Hipopigmentação/fisiopatologia , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Razão de Chances , Valor Preditivo dos Testes , Prognóstico , Prurido/etiologia , Esclerodermia Difusa/diagnóstico , Esclerodermia Difusa/fisiopatologia , Índice de Gravidade de Doença
11.
Rheumatology (Oxford) ; 56(7): 1111-1122, 2017 07 01.
Artigo em Inglês | MEDLINE | ID: mdl-28340090

RESUMO

Objective: To estimate the effect of disease activity, as measured by the European Scleroderma Research Group Activity Index (EScSG-AI), on the risk of subsequent organ damage in a large systemic sclerosis (SSc) cohort. Methods: Of 421 SSc patients from the Canadian Scleroderma Research Group database with disease duration of ⩽ 3 years, 197 who had no evidence of end-stage organ damage initially and available 3 year follow-up were included. Disease activity was assessed by the EScSG-AI with two variability measures: the adjusted mean EScSG-AI (the area under the curve of the EScSG-AI over the observation period) and persistently active disease/flare. Outcomes were based on the Medsger severity scale and included accrual of a new severity score (Δ â©¾ 1) overall and within organ systems or reaching a significant level of deterioration in health status. Results: After adjustment for covariates, the adjusted mean EScSG-AI was the most consistent predictor of risk across the study outcomes over 3 years in dcSSc: disease progression defined as Δ â©¾ 1 in any major internal organ, significant decline in forced vital capacity and diffusing capacity of carbon monoxide, severity of visceral disease and HAQ Disability Index worsening. In multivariate analysis, progression of lung disease was predicted solely by adjusted mean EScSG-AI, while the severity of lung disease was predicted the adjusted mean EScSG-AI, older age, modified Rodnan skin score (mRSS) and initial severity. The EScSG-AI was associated with patient- and physician-assessed measures of health status and overpowered the mRSS in predicting disease outcomes. Conclusion: Disease activity burden quantified with the adjusted mean EScSG-AI predicted the risk of deterioration in health status and severe organ involvement in dcSSc. The EScSG-AI is more responsive when done repeatedly and averaged.


Assuntos
Avaliação da Deficiência , Progressão da Doença , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/epidemiologia , Adulto , Fatores Etários , Idoso , Canadá , Estudos de Coortes , Estudos Transversais , Europa (Continente) , Feminino , Seguimentos , Humanos , Modelos Lineares , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Valor Preditivo dos Testes , Esclerodermia Difusa/diagnóstico , Esclerodermia Difusa/tratamento farmacológico , Esclerodermia Difusa/epidemiologia , Esclerodermia Localizada/diagnóstico , Esclerodermia Localizada/tratamento farmacológico , Esclerodermia Localizada/epidemiologia , Escleroderma Sistêmico/tratamento farmacológico , Índice de Gravidade de Doença , Fatores Sexuais , Perfil de Impacto da Doença
12.
Int J Rheum Dis ; 20(4): 481-488, 2017 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-28337853

RESUMO

OBJECTIVES: To assess the long-term efficacy and tolerability of mycophenolate mofetil (MMF) in patients with diffuse cutaneous systemic sclerosis (dcSSc). METHODS: Patients enrolled in the Australian Scleroderma Cohort study with dcSSc and baseline modified Rodnan skin score (mRSS) ≥ 12 who were treated for a minimum of 12 months with MMF for the primary indication of skin disease were included and their prospectively collected data retrieved. Change in mRSS, the proportion with a clinically significant improvement (reduction in mRSS ≥ 5 from baseline) and adverse effects due to therapy were determined. RESULTS: Seventy-four participants treated with MMF were identified and of these, 42 met inclusion criteria. The mean age was 53 ± 12 years, with mean disease duration at MMF commencement of 4.8 ± 4.3 years. Twenty-one participants (50%) commenced MMF within 2 years of disease onset and the mean duration of therapy was 2.7 ± 1.7 years. The mean mRSS at baseline was 25.9 ± 9.2 with a reduction of 3.7 ± 7.1 (P = 0.07) after 1 year of therapy, 7.6 ± 8.3 after 2 years (P = 0.01) and 10.5 ± 10.3 after 5 years (P < 0.01). Response to treatment was not affected by disease duration at MMF commencement or baseline skin score. Eighteen participants (43%) demonstrated clinically significant improvement after 1 year, increasing to 92% after 4 years. Two participants (5%) ceased MMF due to adverse effects. CONCLUSION: MMF was associated with a modest improvement in mRSS and was well tolerated in the treatment of dcSSc. Given the natural history of dcSSc where skin involvement can spontaneously improve, randomized, placebo-controlled studies are required to confirm whether improvement can be attributed to MMF therapy.


Assuntos
Imunossupressores/uso terapêutico , Ácido Micofenólico/uso terapêutico , Esclerodermia Difusa/tratamento farmacológico , Adulto , Austrália , Feminino , Humanos , Imunossupressores/efeitos adversos , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Ácido Micofenólico/efeitos adversos , Estudos Prospectivos , Indução de Remissão , Esclerodermia Difusa/diagnóstico , Esclerodermia Difusa/imunologia , Fatores de Tempo , Resultado do Tratamento
13.
J Rheumatol ; 44(6): 791-794, 2017 06.
Artigo em Inglês | MEDLINE | ID: mdl-28298560

RESUMO

OBJECTIVE: To determine the inter/intraobserver reliability of the tender and swollen joint counts (TJC, SJC) and the modified Rodnan Skin Score (mRSS) in diffuse cutaneous systemic sclerosis (dcSSc) and to assess content validity of the TJC/SJC. METHODS: Ten rheumatologists completed the SJC, TJC, and mRSS on 7 patients. Musculoskeletal ultrasound (MSUS) was performed. RESULTS: Interobserver and intraobserver reliability for the TJC was 0.97 and 0.99, for the SJC was 0.24 and 0.71, and for the mRSS was 0.81 and 0.94, respectively. MSUS abnormalities did not correspond with SJC/TJC. CONCLUSION: We demonstrate excellent inter- and intraobserver reliability for the mRSS and TJC in dcSSc. However, the SJC and TJC did not correspond to MSUS.


Assuntos
Articulações/patologia , Esclerodermia Difusa/diagnóstico , Pele/patologia , Adulto , Avaliação da Deficiência , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Exame Físico , Estudos Prospectivos , Sistema de Registros , Reprodutibilidade dos Testes , Esclerodermia Difusa/patologia , Índice de Gravidade de Doença , Adulto Jovem
14.
Int J Rheum Dis ; 20(10): 1572-1581, 2017 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-28296274

RESUMO

OBJECTIVE: Autoantibody profiles in systemic sclerosis (SSc) and their relative clinical association vary between studies. The rate for being anti-topoisomerase-I (ATA) positive and the association with diffuse cutaneous the SSc subset (dcSSc) is higher among Thais than among Caucasians. The objective was to evaluate the relevance of clinical presentation, namely being positive for one or more autoantibodies among Thai SSc patients. METHOD: A retrospective, cohort study was performed among SSc patients over 18 years of age at Srinagarind Hospital, Khon Kaen University, Thailand, during January 2006 to December 2013. Autoantibodies comprising 13 SSc-specific antigens were evaluated using the EUROIMMUN AG (Lübeck, Germany) in order to define their clinical association(s). RESULTS: Two hundred and eighty-five scleroderma patients (200 female; 85 male) were included. The majority (66.7%) were dcSSc subset. ATA was the most common antibody profile in our patients (231 cases; 81.1%), followed by anti-Ro 52 (87 cases; 30.5%). Eleven of our patients (3.9%) were negative for all antibody profiles and 44 cases (15.4%) were negative for ATA and anti-centromere antibody (anti-CENP). Almost 40% (112 cases) were positive for at least two autoantibodies. There was an association between the presence of ATA and hand deformity (odds ratio [OR] 3.94; 95% CI 1.12-13.84), anti-CENP and hand deformity (OR 0.20; 95% CI 0.02-0.90), anti-Ku and scleroderma-polymyositis overlap syndrome (OR 6.58; 95% CI 2.16-19.39) and the absence of both ATA and anti-CENP with female sex (OR 2.90; 95% CI 1.12-7.51), limited cutaneous SSc subset (OR 2.70; 95% CI 1.30-5.55) and scleroderma-polymyositis overlap syndrome (OR 2.53; 95% CI 1.04-6.16). Neither ATA nor anti-CENP were associated with the SSc subset. CONCLUSIONS: ATA and anti-CENP were not helpful in differentiating the SSc subset in Thai SSc patients, albeit they were good for predicting hand function. Coexisting ATA and anti-CENP negativity were associated with less extensive skin tightness and SSc overlap syndrome.


Assuntos
Autoanticorpos/sangue , Proteína Centromérica A/imunologia , Proteína B de Centrômero/imunologia , DNA Topoisomerases Tipo I/imunologia , Esclerodermia Difusa/imunologia , Esclerodermia Limitada/imunologia , Adolescente , Adulto , Idoso , Biomarcadores/sangue , Distribuição de Qui-Quadrado , Diagnóstico Diferencial , Feminino , Humanos , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Razão de Chances , Valor Preditivo dos Testes , Dados Preliminares , Prognóstico , Estudos Retrospectivos , Fatores de Risco , Esclerodermia Difusa/sangue , Esclerodermia Difusa/diagnóstico , Esclerodermia Difusa/epidemiologia , Esclerodermia Limitada/sangue , Esclerodermia Limitada/diagnóstico , Esclerodermia Limitada/epidemiologia , Tailândia/epidemiologia , Adulto Jovem
15.
Wiad Lek ; 69(5): 717-720, 2016.
Artigo em Polonês | MEDLINE | ID: mdl-28033593

RESUMO

Systemic scleroderma is a chronic, autoimmune disease of the connective tissue that involves skin, subcutaneous tissue, muscles and joints, as well as the internal organs: kidneys, lungs, heart. Depending on the extent it can occur as limited or diffuse clinical variant. In 60-80 % of patients with diffuse scleroderma, autopsy studies have shown pathologic changes in the kidneys. About half of the patients with renal involvement the clinical manifestation is limited to a moderate increase in serum creatinine, mild proteinuria, and moderate hypertension. The most serious complication remains sclerodermal renal crisis. It develops in 5-20 % of patients and is characterized by severe hypertension, acute kidney injury with oliguria, proteinuria and erythrocyturia, and microangiopathic hemolytic anemia with thrombocytopenia. In this article pathogenesis, risk factors, symptoms and treatment of scleroderma renal crisis have been reviewed.


Assuntos
Nefropatias/etiologia , Nefropatias/terapia , Rim/patologia , Esclerodermia Difusa/diagnóstico , Esclerodermia Difusa/terapia , Humanos , Fatores de Risco , Esclerodermia Difusa/complicações
16.
Clin Exp Rheumatol ; 34 Suppl 100(5): 142-147, 2016 Sep-Oct.
Artigo em Inglês | MEDLINE | ID: mdl-27749240

RESUMO

OBJECTIVES: To develop a standardized scoring system to assess the severity of DUs in SSc patients and correlate it with functional outcomes. METHODS: In this cross-sectional, longitudinal study in SSc patients with DUs (n=65) we developed a digital ulcers score (DUS) for the assessment of DUs. DUS and the ABILHAND score were measured at each visit and differences were analysed using Tamhane's T2 test. Spearman's Rho test was applied for correlational analysis of DUS and functional outcomes. We calculated a linear regression model using clustered standard errors for correlation analysis between DUS and ABILHAND over time. RESULTS: 117 assessments of DUS were performed in 65 SSc patients. Mean DUS was 11.6±1.9 (range: 0-68). Subgroup analyses showed a higher DUS in patients suffering from diffuse cutaneous SSc when compared to patients with limited cutaneous SSc (12.8±3.0 vs. 9.7±2.2 p=0.18). There was no correlation between the DUS and manual ability using the ABILHAND score (overall: n=106 r=-0.138, p=0.22). We observed a small but significant linear correlation between the DUS and the ABILHAND score for a single patient over time (n=14, R2=0.31, r=0.06, p=0.02). CONCLUSIONS: The DUS is a feasible scoring instrument to assess severity of DUs in SSc patients. In accordance with the literature the severity of DUs correlates with clinical parameters but also severity of the disease. Further study is needed to establish the DUS as a standardized tool for the assessment of DUs.


Assuntos
Esclerodermia Difusa/complicações , Esclerodermia Limitada/complicações , Úlcera Cutânea/diagnóstico , Pele/patologia , Adulto , Idoso , Fármacos Cardiovasculares/uso terapêutico , Estudos Transversais , Avaliação da Deficiência , Estudos de Viabilidade , Feminino , Dedos , Humanos , Imunossupressores/uso terapêutico , Modelos Lineares , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Reprodutibilidade dos Testes , Fatores de Risco , Esclerodermia Difusa/diagnóstico , Esclerodermia Difusa/tratamento farmacológico , Esclerodermia Limitada/diagnóstico , Esclerodermia Limitada/tratamento farmacológico , Índice de Gravidade de Doença , Pele/efeitos dos fármacos , Pele/fisiopatologia , Úlcera Cutânea/tratamento farmacológico , Úlcera Cutânea/patologia , Úlcera Cutânea/fisiopatologia , Inquéritos e Questionários , Resultado do Tratamento , Cicatrização
17.
Reumatol. clín. (Barc.) ; 12(5): 285-287, sept.-oct. 2016.
Artigo em Espanhol | IBECS | ID: ibc-155880

RESUMO

La esclerosis sistémica (ES) en una enfermedad autoinmune, crónica, multifactorial y sistémica que afecta al tejido conectivo. Presentamos este caso clínico dado la baja prevalencia de ES difusa con compromiso cardíaco temprano y progresivo en una paciente joven, y tratamiento con trasplante cardíaco (AU)


Systemic sclerosis (SS) in a multifactorial and systemic, chronic, autoimmune disease that affects the connective tissue. We present this clinical case given the low prevalence of diffuse SS with early and progressive cardiac compromise in a young patient, and treatment with cardiac transplantation (AU)


Assuntos
Humanos , Feminino , Adulto , Esclerodermia Difusa/complicações , Esclerodermia Difusa/diagnóstico , Esclerodermia Difusa/cirurgia , Transplante de Órgãos/métodos , Transplante de Coração/instrumentação , Transplante de Coração/métodos , Doenças Autoimunes/complicações , Análise Multivariada
19.
Rheumatology (Oxford) ; 55(11): 2023-2032, 2016 11.
Artigo em Inglês | MEDLINE | ID: mdl-27550299

RESUMO

OBJECTIVE: DcSSc is associated with high morbidity related to widespread skin disease and poor prognosis due to earlier and more severe organ involvement. The objective of this study is to derive and validate a simple prediction rule for identifying patients at the time of initial diagnosis of SSc who are likely to progress to dcSSc. METHODS: The Nijmegen cohort consists of 619 SSc patients. Logistic regression was used for predictive modelling. A prediction rule was created by rounding regression coefficients. Patients were stratified as being at low risk (<1) or high risk (⩾1) of progression to dcSSc. Performance was analysed in 445 SSc patients from Madrid. RESULTS: One hundred and seventy-four out of 535 patients were classified as dcSSc. The final model consisted of gender, time between RP and non-RP, sclerodactyly (first non-Raynaud symptom) and SSc-specific auto-antibodies. The model performed well in the derivation cohort [area under the curve = 0.78 (95% CI: 0.74, 0.82)] and validation cohort [area under the curve = 0.78 (95% CI: 0.74, 0.83)]. At the optimal cut point (1) for the prediction rule, sensitivity was 87% and specificity 61% in the derivation cohort, compared with 78% and 65% in the validation cohort. Upon application of the prediction rule to 392 lcSSc patients at initial diagnosis, 32 out of 34 patients were correctly classified as dcSSc. CONCLUSION: A simple prediction rule was designed to attribute a low/high risk category for development of dcSSc.This method is suited for assigning intensified screening at the time of initial diagnosis of SSc to patients most at risk for dcSSc. It provides the opportunity for early identification of potential dcSSc patients for enrolment into clinical trials.


Assuntos
Autoanticorpos/metabolismo , Sistemas de Apoio a Decisões Clínicas/normas , Esclerodermia Difusa/diagnóstico , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Fatores de Tempo
20.
Clin Exp Rheumatol ; 34 Suppl 100(5): 148-151, 2016 Sep-Oct.
Artigo em Inglês | MEDLINE | ID: mdl-27463733

RESUMO

OBJECTIVES: The European Scleroderma Study Group (EScSG) activity index meets nearly all the OMERACT-standards of truth, discrimination and feasibility. The sensitivity to change remains to be attested. This study assesses sensitivity to change of the EScSG activity index in patients with early and severe diffuse cutaneous Systemic Sclerosis (dcSSc) treated with rituximab. METHODS: 12-month follow-up (open-label study) of 14 consecutive patients with early dcSSc. Patients received an infusion of two times 1000 mg rituximab at month 0 and 6, together with 100 mg methylprednisolone. Clinical read outs (modified Rodnan skin score [mRSS], lung function and echocardiography) and EScSG activity index were performed at month 0, 3, 6 and 12. Mixed models analyses (MMA) were used to evaluate changes in parameters over time. RESULTS: There was a clinically significant change in skin score with a mean (SD) mRSS of 24.8 (4.44) at baseline and 10.4 (3.12) at month 12 (MMA p<0.001). Also the EScSG activity index decreased significantly, with a mean (SD) of 4.3 (1.79) at baseline and 0.7 (0.83) at month 12 (MMA p<0.001). The estimated mean change of the EScSG activity index was -3.6 (95%CI -4.9; -2.4) over 12 months. Indices of internal organ involvement remained stable throughout the study. CONCLUSIONS: A significant improvement of the EScSG activity index was observed, in line with the significant improvement of the mRSS and the stabilisation of internal organ involvement. To our knowledge, this is the first study to attest sensitivity to change of the EScSG activity index in the subset of 'early' dcSSc. TRIAL REGISTRATION: ClinicalTrials.gov Registration, http://clinicaltrials.gov, number NCT00379431.


Assuntos
Imunossupressores/administração & dosagem , Rituximab/administração & dosagem , Esclerodermia Difusa/tratamento farmacológico , Administração Intravenosa , Adulto , Idoso , Bélgica , Esquema de Medicação , Quimioterapia Combinada , Ecocardiografia , Feminino , Cardiopatias/diagnóstico , Cardiopatias/tratamento farmacológico , Cardiopatias/etiologia , Humanos , Imunossupressores/efeitos adversos , Pneumopatias/diagnóstico , Pneumopatias/tratamento farmacológico , Pneumopatias/etiologia , Masculino , Metilprednisolona/administração & dosagem , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Indução de Remissão , Reprodutibilidade dos Testes , Testes de Função Respiratória , Fatores de Risco , Rituximab/efeitos adversos , Esclerodermia Difusa/complicações , Esclerodermia Difusa/diagnóstico , Índice de Gravidade de Doença , Fatores de Tempo , Resultado do Tratamento
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA