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1.
Semin Arthritis Rheum ; 48(4): 694-700, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-29685482

RESUMO

BACKGROUND: To estimate patient acceptable symptom state (PASS) and minimal clinically important difference (MCID) for patient-reported outcomes in systemic sclerosis (SSc). METHODS: We conducted a secondary analysis of the SCLEREDUC trial, a 12-month randomized controlled trial comparing the efficacy of physical therapy to usual care in 220 SSc patients followed-up from September 2005 to October 2010. Self-rated state and change in patient health at 12 months were assessed by using 2 external anchors extracted from the Medical Outcomes Study 36-Item Short-Form. Patients who self-rated their health as "excellent", "very good" or "good" were the PASS group and those who self-rated their health change as "somewhat better" were the MCID group. Main outcomes were the estimates of PASS by using the 75th percentile method and of MCID by using the mean change in scores method for pain and activity limitation. RESULTS: PASS (95% confidence interval) and mean (SD) MCID estimates at 12 months were 53.75 (34.00 to 68.00) and -6.74 (32.02) for the joint-pain visual analog scale (range 0-100), 1.41 (1.13 to 1.63) and -0.21 (0.48) for the Health Assessment Questionnaire (HAQ, range 0-3), 1.27 (1.07 to 1.62) and -0.13 (0.45) for the scleroderma HAQ (range 0-3), 26.00 (17.00 to 37.00) and -3.38 (9.87) for the Cochin Hand Function Scale (range 0-90), and 19.40 (17.20 to 21.90) and -5.69 (6.79) for the McMaster-Toronto Arthritis Patient Preference Disability Questionnaire (range 0-30), respectively. CONCLUSIONS: We provide, for the first time, the PASS and MCID estimates for pain and activity limitation in SSc. TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT00318188. First Posted: April 26, 2006.


Assuntos
Modalidades de Fisioterapia , Escleroderma Sistêmico/terapia , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Diferença Mínima Clinicamente Importante , Medidas de Resultados Relatados pelo Paciente , Satisfação do Paciente , Escleroderma Sistêmico/tratamento farmacológico
2.
J Rheumatol ; 45(2): 242-247, 2018 02.
Artigo em Inglês | MEDLINE | ID: mdl-29142028

RESUMO

OBJECTIVE: Clara cell secretory protein (CC16) is a sensitive marker of bronchial epithelial cell damage. The CC16 serum level is elevated in patients with pulmonary fibrosis, but its predictive value on lung disease progression has not yet been studied. We aimed to assess the value of serum CC16 concentration in predicting lung disease deterioration in patients with systemic sclerosis (SSc). METHODS: We prospectively analyzed and followed 106 patients with SSc during a 4-year period for the risk of developing combined deleterious event, defined as a 10% decrease in total lung capacity or forced vital capacity from baseline, or death, according to serum CC16 at inclusion. Receiver-operating characteristic (ROC) curve analysis was performed for prediction of events during the first 2 years after inclusion. Cumulative risks of combined events were computed by Kaplan-Meier analysis. RESULTS: The best cutoff level of serum CC16 for prediction of a combined event was 33 ng/ml, with 76% sensitivity and 65% specificity (area under the ROC curve: 0.71, 95% CI 0.61-0.81, p < 0.0001). Progression of lung disease evaluated by a mean time-to-event differed between patients with high baseline serum CC16 (42.8 mos, 36.3-49.3) and those with low serum CC16 (56.3 mos, 50.9-61.7; log-rank test, p < 0.001). After adjustment for age, duration of disease, clinical and lung function measures, the risk of combined event occurrence in patients with high serum CC16 was significantly higher than in those with low CC16 (HR 2.9, 1.2-6.75, p < 0.05). CONCLUSION: High baseline serum CC16 predicts lung disease worsening in patients with SSc.


Assuntos
Progressão da Doença , Pneumopatias/sangue , Pneumopatias/patologia , Escleroderma Sistêmico/sangue , Escleroderma Sistêmico/patologia , Uteroglobina/sangue , Adolescente , Adulto , Biomarcadores/sangue , Brônquios/patologia , Criança , Pré-Escolar , Ecocardiografia , Células Epiteliais/metabolismo , Feminino , Seguimentos , França , Humanos , Lactente , Estimativa de Kaplan-Meier , Pneumopatias/complicações , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Modelos de Riscos Proporcionais , Estudos Prospectivos , Curva ROC , Escleroderma Sistêmico/complicações , Sensibilidade e Especificidade , Estatísticas não Paramétricas , Tomografia Computadorizada por Raios X , Capacidade Vital , Adulto Jovem
3.
J Med Internet Res ; 19(8): e293, 2017 08 23.
Artigo em Inglês | MEDLINE | ID: mdl-28835354

RESUMO

BACKGROUND: Assessing the satisfaction of patients about the health care they have received is relatively common nowadays. In France, the satisfaction questionnaire, I-Satis, is deployed in each institution admitting inpatients. Internet self-completion and telephone interview are the two modes of administration for collecting inpatient satisfaction that have never been compared in a multicenter randomized experiment involving a substantial number of patients. OBJECTIVE: The objective of this study was to compare two modes of survey administration for collecting inpatient satisfaction: Internet self-completion and telephone interview. METHODS: In the multicenter SENTIPAT (acronym for the concept of sentinel patients, ie, patients who would voluntarily report their health evolution on a dedicated website) randomized controlled trial, patients who were discharged from the hospital to home and had an Internet connection at home were enrolled between February 2013 and September 2014. They were randomized to either self-complete a set of questionnaires using a dedicated website or to provide answers to the same questionnaires administered during a telephone interview. As recommended by French authorities, the analysis of I-Satis satisfaction questionnaire involved all inpatients with a length of stay (LOS), including at least two nights. Participation rates, questionnaire consistency (measured using Cronbach alpha coefficient), and satisfaction scores were compared in the two groups. RESULTS: A total of 1680 eligible patients were randomized to the Internet group (n=840) or the telephone group (n=840). The analysis of I-Satis concerned 392 and 389 patients fulfilling the minimum LOS required in the Internet and telephone group, respectively. There were 39.3% (154/392) and 88.4% (344/389) responders in the Internet and telephone group, respectively (P<.001), with similar baseline variables. Internal consistency of the global satisfaction score was higher (P=.03) in the Internet group (Cronbach alpha estimate=.89; 95% CI 0.86-0.91) than in the telephone group (Cronbach alpha estimate=.84; 95% CI 0.79-0.87). The mean global satisfaction score was lower (P=.03) in the Internet group (68.9; 95% CI 66.4-71.4) than in the telephone group (72.1; 95% CI 70.4-74.6), with a corresponding effect size of the difference at -0.253. CONCLUSIONS: The lower response rate issued from Internet administration should be balanced with a likely improved quality in satisfaction estimates, when compared with telephone administration, for which an interviewer effect cannot be excluded. TRIAL REGISTRATION: Clinicaltrials.gov NCT01769261 ; http://clinicaltrials.gov/ct2/show/NCT01769261 (Archived by WebCite at http://www.webcitation.org/6ZDF5lA41).


Assuntos
Satisfação do Paciente , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Pacientes Internados , Internet , Masculino , Pessoa de Meia-Idade , Medidas de Resultados Relatados pelo Paciente , Inquéritos e Questionários , Telefone , Adulto Jovem
4.
Arthritis Care Res (Hoboken) ; 69(7): 1050-1059, 2017 07.
Artigo em Inglês | MEDLINE | ID: mdl-27696703

RESUMO

OBJECTIVE: To compare a physical therapy program to usual care of systemic sclerosis (SSc) patients on disability. METHODS: A 12-month followup, parallel-group randomized controlled trial involving a modified Zelen design was conducted in 4 tertiary-care hospitals. Patients were enrolled if they had a disability rating ≥0.5 on the Health Assessment Questionnaire disability index (HAQ DI) or symptoms of decreased mouth opening or limited range of motion of at least 1 joint. The experimental intervention (n = 112, of which 110 were analyzed) was a 1-month personalized supervised physical therapy program provided by trained care providers followed by home sessions. The comparator (n = 108, and all 108 were analyzed) was usual care that could include ambulatory physical therapy. The primary outcome was the HAQ DI score. RESULTS: There was no statistically significant difference in disability at 12 months (HAQ DI score between-group difference -0.01 [95% confidence interval (95% CI) -0.15, 0.13]; P = 0.86). Disability was reduced at 1 month for patients in the physical therapy group (HAQ DI between-group difference -0.14 [95% CI -0.24, -0.03]; P = 0.01); at 6 months the HAQ DI score between-group difference was -0.12 (95% CI -0.23, 0.01); P = 0.054. There was a statistically significant difference for hand mobility and function, and for pain, at 1 month. Microstomia was lower in the physical therapy group at 1, 6, and 12 months (between-group difference at 12 months 1.62 [95% CI 0.32, 2.93]; P = 0.01). No differences in adverse effects were observed. CONCLUSION: A personalized physical therapy program did not reduce disability at 12 months but had short-term benefits for patients with SSc.


Assuntos
Serviços de Assistência Domiciliar/normas , Modalidades de Fisioterapia/normas , Medicina de Precisão/normas , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/terapia , Adulto , Idoso , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Assistência ao Paciente/métodos , Assistência ao Paciente/normas , Medicina de Precisão/métodos
5.
PLoS One ; 11(9): e0160283, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-27617966

RESUMO

In a pilot ProtoArray analysis, we identified 6 proteins out of 9483 recognized by autoantibodies (AAb) from patients with systemic sclerosis (SSc). We further investigated the 6 candidates by ELISA on hundreds of controls and patients, including patients with Systemic Lupus Erythematosus (SLE), known for high sera reactivity and overlapping AAb with SSc. Only 2 of the 6 candidates, Ephrin type-B receptor 2 (EphB2) and Three prime Histone mRNA EXonuclease 1 (THEX1), remained significantly recognized by sera samples from SSc compared to controls (healthy or with rheumatic diseases) with, respectively, 34% versus 14% (P = 2.10-4) and 60% versus 28% (P = 3.10-8). Above all, EphB2 and THEX1 revealed to be mainly recognized by SLE sera samples with respectively 56%, (P = 2.10-10) and 82% (P = 5.10-13). As anti-EphB2 and anti-THEX1 AAb were found in both diseases, an epitope mapping was realized on each protein to refine SSc and SLE diagnosis. A 15-mer peptide from EphB2 allowed to identify 35% of SLE sera samples (N = 48) versus only 5% of any other sera samples (N = 157), including SSc sera samples. AAb titers were significantly higher in SLE sera (P<0.0001) and correlated with disease activity (p<0.02). We could not find an epitope on EphB2 protein for SSc neither on THEX1 for SSc or SLE. We showed that patients with SSc or SLE have AAb against EphB2, a protein involved in angiogenesis, and THEX1, a 3'-5' exoribonuclease involved in histone mRNA degradation. We have further identified a peptide from EphB2 as a specific and sensitive tool for SLE diagnosis.


Assuntos
Autoanticorpos/sangue , Exorribonucleases/imunologia , Lúpus Eritematoso Sistêmico/imunologia , Receptor EphB2/imunologia , Escleroderma Sistêmico/imunologia , Ensaio de Imunoadsorção Enzimática , Mapeamento de Epitopos , Feminino , Humanos , Lúpus Eritematoso Sistêmico/diagnóstico , Masculino , Pessoa de Meia-Idade , Sensibilidade e Especificidade
6.
Rheumatology (Oxford) ; 54(10): 1852-7, 2015 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-26001634

RESUMO

OBJECTIVES: Tracheobronchial stenosis (TBS) is noted in 12-23% of patients with granulomatosis with polyangiitis (GPA), and includes subglottic stenosis and bronchial stenosis. We aimed to analyse the endoscopic management of TBS in GPA and to identify factors associated with the efficacy of endoscopic interventions. METHODS: We conducted a French nationwide retrospective study that included 47 patients with GPA-related TBS. RESULTS: Compared with patients without TBS, those with TBS were younger, more frequently female and had less frequent kidney, ocular and gastrointestinal involvement and mononeuritis multiplex. Endoscopic procedures included 137 tracheal and 50 bronchial interventions, mainly endoscopic dilatation, local steroid injection and conservative laser surgery, and less frequently stenting. After the first endoscopic procedure, the cumulative incidence of endoscopic treatment failure was 49% at 1 year, 70% at 2 years and 80% at 5 years. Factors significantly associated with a higher cumulative incidence of treatment failure were a shorter time from GPA diagnosis to endoscopic procedure [hazard ratio (HR) 1.08 (95% CI 1.01, 1.14); P = 0.01] and a bronchial stenosis [HR 1.96 (95% CI 1.28, 3.00); P = 0.002]. A prednisone dose ≥30 mg/day at the time of the procedure was associated with a lower cumulative incidence of treatment failure [HR 0.53 (95% CI 0.31, 0.89); P = 0.02]. CONCLUSION: TBS represents severe and refractory manifestations with a high rate of restenosis. High-dose systemic CSs at the time of the procedure and increased time from GPA diagnosis to bronchoscopic intervention are associated with a better event-free survival. In contrast, bronchial stenoses are associated with a higher rate of restenosis than subglottic stenosis.


Assuntos
Broncopatias/etiologia , Broncopatias/terapia , Endoscopia/métodos , Granulomatose com Poliangiite/complicações , Estenose Traqueal/etiologia , Estenose Traqueal/terapia , Adolescente , Adulto , Idoso , Constrição Patológica/etiologia , Constrição Patológica/terapia , Dilatação/métodos , Feminino , Humanos , Injeções , Terapia a Laser/métodos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Stents , Esteroides/administração & dosagem , Esteroides/uso terapêutico , Falha de Tratamento , Resultado do Tratamento , Adulto Jovem
7.
Autoimmun Rev ; 13(12): 1189-94, 2014 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-25151977

RESUMO

INTRODUCTION: The purpose of this study was to estimate the prevalence of monoclonal immunoglobulin (MIg) among patients with systemic sclerosis (SSc) according to the capillary electrophoresis or immunofixation method of detection and to search for any related clinical correlations. PATIENTS AND METHODS: Retrospective multicenter comparison of capillary electrophoresis and immunofixation results in SSc patients and of the characteristics of patients with and without MIg. RESULTS: The study included 244 SSc patients (216 women and 28 men, mean age: 55±14 years). Median time since SSc diagnosis was 51 months [0-320]; disease was diffuse in 48% of cases. Ten percent of patients had cancer, including Waldenström macroglobulinemia (n=1) and multiple myeloma (n=3). Capillary electrophoresis showed a γ-globulin anomaly in 41% of cases, and immunofixation in 18%: MIg (13.5%) and restriction of heterogeneity (4.5%). Capillary electrophoresis failed to detect 60% of the 33 MIg patients. Measurable MIg concentrations were obtained from 7 patients. MIg patients were significantly older at SSc diagnosis than those without MIg (p=0.002), had a lower diffusing capacity (p=0.002), a higher prevalence of pulmonary hypertension and cancer (p=0.002) and were more frequently positive for anti-mitochondrial and anti-beta2-glycoprotein-I antibodies (p=0.03 and p=0.02, respectively). Multivariate analyses showed that only age at test [hazard ratio 1.03 (95% CI, 1.00-1.07, p=0.04)] and presence of cancer [hazard ratio 4.46 (95% CI, 1.6-12.4, p=0.004)] were associated with MIg. CONCLUSION: Immunofixation detected a high prevalence of MIg among SSc patients especially in patients aged 50-years or older. MIg was not detected by the standard capillary electrophoresis in 60% of cases and was significantly associated with cancer.


Assuntos
Anticorpos Monoclonais/imunologia , Escleroderma Sistêmico/imunologia , Anticorpos Monoclonais/análise , Capilares , Humanos , Fenótipo , Prevalência , Estudos Retrospectivos , Escleroderma Sistêmico/epidemiologia
8.
Nitric Oxide ; 40: 17-21, 2014 Aug 31.
Artigo em Inglês | MEDLINE | ID: mdl-24831352

RESUMO

Cyclophosphamide (CYC) is not always effective in patients with scleroderma-related interstitial lung disease (SSc-ILD), hence the need for biomarkers able to predict beneficial responses to CYC therapy. We therefore assessed whether baseline alveolar concentration of nitric oxide (CANO) could predict the favourable response to CYC therapy in patients with SSc-ILD. Nineteen non-smoker patients with SSc-ILD, were enrolled and treated with 6 courses of CYC (0.75 g/m2/monthly) for lung function decline the year before inclusion, and followed-up for 2 years period. We assessed the proportion of favourable response to CYC, defined as improvement of forced vital capacity (FVC) or total pulmonary capacity (TLC) more than 10% between the inclusion and each following visit, according to the validated cut-off of CANO at 8.5 ppb identifying progressive SSc-ILD subset. At inclusion, 7 patients out of 19 had CANO >8.5 ppb. Clinical parameters were comparable between patients with high (>8.5 ppb) and low level of CANO (≤8.5 ppb). After CYC therapy, and during the follow-up, 9 out of 19 patients had favourable response to CYC therapy, 10 did not meet responder's criteria, from whom 4 patients died from respiratory failure. Six out of 7 patients with CANO >8.5 ppb at inclusion had favourable response to CYC therapy, while only 3 out of 12 patients with CANO ≤8.5 ppb responded favourably to CYC therapy (p=0.001). High level of CANO >8.5 ppb reflecting alveolar inflammation identify SSc patients with a greater chance to benefit from CYC treatment with a significant lung function improvement.


Assuntos
Ciclofosfamida/uso terapêutico , Doenças Pulmonares Intersticiais/tratamento farmacológico , Óxido Nítrico/uso terapêutico , Escleroderma Sistêmico/tratamento farmacológico , Administração Intravenosa , Adulto , Ciclofosfamida/administração & dosagem , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Óxido Nítrico/administração & dosagem , Estudos Prospectivos , Testes de Função Respiratória
10.
J Rheumatol ; 41(1): 99-105, 2014 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-24293584

RESUMO

OBJECTIVE: To estimate the prevalence, determine the subgroups at risk, and the outcomes of patients with systemic sclerosis (SSc) and gastric antral vascular ectasia (GAVE). METHODS: We queried the European League Against Rheumatism Scleroderma Trials and Research (EUSTAR) network for the recruitment of patients with SSc-GAVE. Each case was matched for cutaneous subset and disease duration with 2 controls with SSc recruited from the same center, evaluated at the time the index case made the diagnosis of GAVE. SSc characteristics were recorded at the time GAVE occurred and the last observation was collected to define the outcomes. RESULTS: Forty-nine patients with SSc and GAVE were included (24 with diffuse cutaneous SSc) and compared to 93 controls with SSc. The prevalence of GAVE was estimated at about 1% of patients with SSc. By multivariate analysis, patients with SSc-GAVE more frequently exhibited a diminished (< 75%) DLCO value (OR 12.8; 95% CI 1.9-82.8) despite less frequent pulmonary fibrosis (OR 0.2; 95% CI 0.1-0.6). GAVE was also associated with the presence of anti-RNA-polymerase III antibodies (OR 4.6; 95% CI 1.2-21.1). SSc-GAVE was associated with anemia (82%) requiring blood transfusion (45%). Therapeutic endoscopic procedures were performed in 45% of patients with GAVE. After a median followup of 30 months (range 1-113 months), survival was similar in patients with SSc-GAVE compared to controls, but a higher number of scleroderma renal crisis cases occurred (12% vs 2%; p = 0.01). CONCLUSION: GAVE is rare and associated with a vascular phenotype, including anti-RNA-polymerase III antibodies, and a high risk of renal crisis. Anemia, usually requiring blood transfusions, is a common complication.


Assuntos
Ectasia Vascular Gástrica Antral/epidemiologia , Escleroderma Sistêmico/epidemiologia , Adulto , Idoso , Transfusão de Sangue , Estudos de Casos e Controles , Comorbidade , Endoscopia Gastrointestinal , Feminino , Ectasia Vascular Gástrica Antral/diagnóstico , Ectasia Vascular Gástrica Antral/cirurgia , Hemostasia Cirúrgica , Humanos , Masculino , Pessoa de Meia-Idade , Prevalência , Risco , Adulto Jovem
11.
Nitric Oxide ; 28: 65-70, 2013 Jan 15.
Artigo em Inglês | MEDLINE | ID: mdl-23099297

RESUMO

Alveolar concentration of nitric oxide (C(A)NO) is a non invasive prognostic marker of systemic sclerosis (SSc) lung disease. There is, however, as yet no direct evidence showing concomitant increase of C(A)NO and the presence of inflammatory cells in alveoli. We have therefore measured C(A)NO and performed broncho-alveolar lavage (BAL) in SSc patients. Exhaled NO was measured, by the means of two different models, the two-compartment model (2CM) and the trumpet model with axial diffusion (TMAD), in 22 SSc patients and compared with 15 healthy controls. BAL was performed in all SSc patients. Alveolitis was defined as lymphocytes >14%, polymorphonuclears >4%, or eosinophils >3% on cell count in BAL fluid. Comparisons of C(A)NO levels were made between SSc patients with, and without, alveolitis. Levels of C(A)NO were significantly higher in SSc patients as compared with controls (p<0.001). Median C(A)NO was significantly higher in SSc patients with alveolitis as compared with SSc patients without alveolitis (8.4ppb; 1st and 3rd interquartile range: 6.0-10.5 vs 3.3ppb; 2.2-3.5; p=0.004 for 2CM and 5.4ppb; 3.2-9.2 vs 3.2ppb; 1.4-3.3, p=0.02 for TMAD), while bronchial airway output of NO (J'awNO, p=0.19), and fractional exhaled NO (F(E)NO, p=0.12) were comparable. C(A)NO was consistently high in SSc patients with alveolitis irrespective of the methods chosen (TMAD or 2CM). Our findings showed that increased C(A)NO was associated with alveolitis in patients with SSc. We submit that C(A)NO could be used as a reliable non-invasive surrogate biomarker of alveolitis in scleroderma lung disease.


Assuntos
Pneumopatias/metabolismo , Óxido Nítrico/análise , Óxido Nítrico/metabolismo , Escleroderma Sistêmico/metabolismo , Adulto , Feminino , Humanos , Pneumopatias/diagnóstico , Masculino , Pessoa de Meia-Idade , Escleroderma Sistêmico/diagnóstico
12.
Presse Med ; 42(2): 151-9, 2013 Feb.
Artigo em Francês | MEDLINE | ID: mdl-22552044

RESUMO

A sub-clinical inflammatory aortitis is very frequent in patients with giant cell arteritis, and can be the only localization of the disease. In most patients, this aortitis is asymptomatic and is of no consequence on the patient's survival. The relative risk of developing an aortic dissection or aneurysm is 17.3. Evolution towards an aneurysm or an aortic dissection is unpredictable and rare; and seems independent of the disease activity and the associated vascular risk factors. Isolated aortitis treatment is not consensual, but often similar to the treatment of giant cell arteritis and adapted to clinical and biological markers of disease activity. Screening for sub-clinical aortitis with FDG-PET should not be prescribed in patients with typical presentation of giant cell arteritis. A systematic screening of aortic complications in giant cell arteritis patients could be done with a chest X-ray and an abdominal ultrasound possibly completed with an aortic CT-scan at time of diagnosis, in order to look for aneurysms with possible surgical indication.


Assuntos
Aortite/complicações , Arterite de Células Gigantes/complicações , Aortite/diagnóstico , Aortite/epidemiologia , Aortite/terapia , Diagnóstico por Imagem/métodos , Progressão da Doença , Arterite de Células Gigantes/diagnóstico , Arterite de Células Gigantes/epidemiologia , Arterite de Células Gigantes/terapia , Humanos , Inflamação/complicações , Inflamação/diagnóstico , Inflamação/epidemiologia , Inflamação/terapia , Prognóstico
13.
Arthritis Res Ther ; 14(3): R152, 2012 Jun 22.
Artigo em Inglês | MEDLINE | ID: mdl-22726824

RESUMO

INTRODUCTION: Myopathy related to systemic sclerosis (Myo-SSc) is a disabling and unpredictable complication of SSc. We assessed the predictive value of serum aldolase, creatine kinase (CK), alanine transaminase (ALT), aspartate transaminase (AST) and C-reactive protein (CRP) to estimate the risk of developing Myo-SSc. METHODS: We enrolled 137 SSc patients without proximal muscle weakness in a prospective monocentric study to follow them longitudinally over a four-year period. The risk of occurrence of Myo-SSc was ascertained according to the European NeuroMuscular Centre criteria and was analyzed according to levels of plasma aldolase, CK, transaminase enzymes and CRP at inclusion. Performance of each parameter to predict Myo-SSc occurrence was assessed and compared with the others. RESULTS: The area under the receiver operating characteristic curves (ROC) of plasma aldolase for Myo-SSc occurrence prediction was 0.80 (95% CI: 0.67 to 0.94, P < 0.001), which was higher than that of plasma CK (0.75, P = 0.01), and that of ALT (0.63, P = 0.04). AST and CRP had no predictive value for Myo-SSc occurrence. The best cut-off of aldolase for prediction of Myo-SSc occurrence within three years after inclusion was 9 U/L and higher than the upper normality limit (7 U/L), unlike that of CK and ALT. Myo-SSc occurred more frequently in patients whose plasma aldolase was higher than 9 U/L. Adjusted Hazard Ratio for patients with aldolase > 9 U/L was 10.3 (95% CI: 2.3 to 45.5), P < 0.001. CONCLUSIONS: Increased plasma aldolase level accurately identified SSc patients with high risk to develop subsequent Myo-SSc. This could help initiate appropriate treatment when the disabling muscle damage is still in a reversible stage.


Assuntos
Frutose-Bifosfato Aldolase/sangue , Doenças Musculares/sangue , Escleroderma Sistêmico/enzimologia , Área Sob a Curva , Biomarcadores/sangue , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doenças Musculares/enzimologia , Doenças Musculares/etiologia , Valor Preditivo dos Testes , Curva ROC , Fatores de Risco , Escleroderma Sistêmico/sangue , Escleroderma Sistêmico/complicações
14.
PLoS One ; 7(5): e36870, 2012.
Artigo em Inglês | MEDLINE | ID: mdl-22615829

RESUMO

Although many studies have analyzed HLA allele frequencies in several ethnic groups in patients with scleroderma (SSc), none has been done in French Caucasian patients and none has evaluated which one of the common amino acid sequences, (67)FLEDR(71), shared by HLA-DRB susceptibility alleles, or (71)TRAELDT(77), shared by HLA-DQB1 susceptibility alleles in SSc, was the most important to develop the disease. HLA-DRB and DQB typing was performed for a total of 468 healthy controls and 282 patients with SSc allowing FLEDR and TRAELDT analyses. Results were stratified according to patient's clinical subtypes and autoantibody status. Moreover, standardized HLA-DRß1 and DRß5 reverse transcriptase Taqman PCR assays were developed to quantify ß1 and ß5 mRNA in 20 subjects with HLA-DRB1*15 and/or DRB1*11 haplotypes. FLEDR motif is highly associated with diffuse SSc (χ(2) = 28.4, p<10-6) and with anti-topoisomerase antibody (ATA) production (χ(2) = 43.9, p<10-9) whereas TRAELDT association is weaker in both subgroups (χ(2) = 7.2, p = 0.027 and χ(2) = 14.6, p = 0.0007 respectively). Moreover, FLEDR motif- association among patients with diffuse SSc remains significant only in ATA subgroup. The risk to develop ATA positive SSc is higher with double dose FLEDR than single dose with respectively, adjusted standardised residuals of 5.1 and 2.6. The increase in FLEDR motif is mostly due to the higher frequency of HLA-DRB1*11 and DRB1*15 haplotypes. Furthermore, FLEDR is always carried by the most abundantly expressed ß chain: ß1 in HLA DRB1*11 haplotypes and ß5 in HLA-DRB1*15 haplotypes.In French Caucasian patients with SSc, FLEDR is the main presenting motif influencing ATA production in dcSSc. These results open a new field of potential therapeutic applications to interact with the FLEDR peptide binding groove and prevent ATA production, a hallmark of severity in SSc.


Assuntos
Epitopos/genética , Antígenos HLA/genética , Antígenos HLA/imunologia , Escleroderma Sistêmico/genética , Escleroderma Sistêmico/imunologia , População Branca/genética , Alelos , Epitopos/imunologia , Feminino , Frequência do Gene , Predisposição Genética para Doença , Cadeias beta de HLA-DQ/genética , Cadeias beta de HLA-DQ/imunologia , Cadeias HLA-DRB1/genética , Cadeias HLA-DRB1/imunologia , Cadeias HLA-DRB5/genética , Cadeias HLA-DRB5/imunologia , Haplótipos , Humanos , Desequilíbrio de Ligação , Masculino , Pessoa de Meia-Idade , RNA Mensageiro/genética
15.
Rheumatology (Oxford) ; 51(3): 460-7, 2012 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-22087012

RESUMO

OBJECTIVE: Scleroderma renal crisis (SRC) is a severe manifestation of SSc, whose prognosis remains severe, despite treatment with angiotensin-converting-enzyme inhibitor and dialysis. This study was undertaken to describe SRC characteristics, prognosis and outcome, and evaluate the responsibility of CSs in its occurrence. METHODS: Analysis concerned 91 SSc patients with SRC who were compared with 427 non-SRC-SSc patients taken as controls. RESULTS: Among the 91 SRC patients, 71 (78.0%) had high blood pressure, 53 (58.2%) hypertensive encephalopathy and 51 (56.0%) thrombotic microangiopathy; 64 (70.3%) had received CSs before or concomitantly with SRC vs 156 (36.5%) non-SRC-SSc patients (P < 0.001). Treated SRC patients also received more prednisone 29.3 (28.4) vs 3.6 (9.9) mg than controls (P < 0.001). SRC clinical outcomes were poor: 49 (53.8%) patients required dialysis, which was definitive for 38. Thirty-seven (40.7%) SRC patients died vs 10.8% of the controls (P < 0.001). Death was most frequent among dialysed patients who never recovered renal function (22 vs 2) and 13 never-dialysed SRC patients died. CONCLUSIONS: Although SRC prognosis has improved markedly, SRC remains a severe manifestation of SSc, despite treatment with angiotensin-converting enzyme inhibitor and dialysis. CSs contributed significantly to SRC occurrence.


Assuntos
Injúria Renal Aguda/etiologia , Inibidores da Enzima Conversora de Angiotensina/uso terapêutico , Glucocorticoides/uso terapêutico , Prednisona/uso terapêutico , Diálise Renal , Escleroderma Sistêmico/complicações , Injúria Renal Aguda/mortalidade , Injúria Renal Aguda/terapia , Adulto , Idoso , Estudos de Casos e Controles , Terapia Combinada , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Escleroderma Sistêmico/mortalidade , Escleroderma Sistêmico/terapia , Taxa de Sobrevida , Resultado do Tratamento
16.
Thorax ; 67(2): 157-63, 2012 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-22026971

RESUMO

BACKGROUND: Respiratory failure is a life-threatening and unpredictable complication of systemic sclerosis (SSc). A study was undertaken to assess the value of alveolar nitric oxide (NO) in predicting the risk of lung function deterioration leading to respiratory failure or death in patients with SSc. METHODS: 105 patients with SSc were enrolled in this prospective cohort and were followed longitudinally over a 3-year period during which the risk of occurrence of deleterious events was analysed according to alveolar concentration (C(A)NO), conducting airway output (J'(aw)NO) and fractional concentration (F(E)NO(0.05)) of exhaled NO measured at inclusion. Comparison was made between each NO parameter to predict the occurrence of deleterious events, defined as a 10% decrease in total lung capacity or forced vital capacity from baseline, or death. RESULTS: The area under the receiver operating characteristic curve of C(A)NO to predict the occurrence of the combined events was 0.84 (95% CI 0.76 to 0.92; p<0.001), which was significantly higher than those of J'(aw)NO and F(E)NO(0.05) (p<0.001). A cut-off of C(A)NO of 5.3 ppb had a sensitivity of 88% and a specificity of 62% for the prediction of the occurrence of combined events during follow-up, and was validated in an independent cohort of patients with SSc. Combined events occurred more frequently in patients whose C(A)NO was >5.3 ppb. The adjusted HR for patients with C(A)NO >5.3 ppb was 6.06 (95% CI 2.36 to 15.53; p<0.001). C(A)NO accurately predicted the occurrence of combined events irrespective of forced vital capacity values or the presence of interstitial lung disease at baseline. CONCLUSIONS: Increased C(A)NO accurately identifies patients with SSc with a high risk of developing lung function deterioration and may help to initiate early appropriate treatment.


Assuntos
Doenças Pulmonares Intersticiais/diagnóstico , Óxido Nítrico/metabolismo , Alvéolos Pulmonares/metabolismo , Escleroderma Sistêmico/complicações , Adulto , Idoso , Biomarcadores/metabolismo , Testes Respiratórios/métodos , Progressão da Doença , Métodos Epidemiológicos , Feminino , Humanos , Doenças Pulmonares Intersticiais/etiologia , Doenças Pulmonares Intersticiais/metabolismo , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Escleroderma Sistêmico/metabolismo , Escleroderma Sistêmico/fisiopatologia , Capacidade Vital/fisiologia
17.
BMC Musculoskelet Disord ; 12: 258, 2011 Nov 11.
Artigo em Inglês | MEDLINE | ID: mdl-22078002

RESUMO

BACKGROUND: Fibromyalgia (FM) is a heterogeneous syndrome and its classification into subgroups calls for broad-based discussion. FM subgrouping, which aims to adapt treatment according to different subgroups, relies in part, on psychological and cognitive dysfunctions. Since motor control of gait is closely related to cognitive function, we hypothesized that gait markers could be of interest in the identification of FM patients' subgroups. This controlled study aimed at characterizing gait disorders in FM, and subgrouping FM patients according to gait markers such as stride frequency (SF), stride regularity (SR), and cranio-caudal power (CCP) which measures kinesia. METHODS: A multicentre, observational open trial enrolled patients with primary FM (44.1 ± 8.1 y), and matched controls (44.1 ± 7.3 y). Outcome measurements and gait analyses were available for 52 pairs. A 3-step statistical analysis was carried out. A preliminary single blind analysis using k-means cluster was performed as an initial validation of gait markers. Then in order to quantify FM patients according to psychometric and gait variables an open descriptive analysis comparing patients and controls were made, and correlations between gait variables and main outcomes were calculated. Finally using cluster analysis, we described subgroups for each gait variable and looked for significant differences in self-reported assessments. RESULTS: SF was the most discriminating gait variable (73% of patients and controls). SF, SR, and CCP were different between patients and controls. There was a non-significant association between SF, FIQ and physical components from Short-Form 36 (p = 0.06). SR was correlated to FIQ (p = 0.01) and catastrophizing (p = 0.05) while CCP was correlated to pain (p = 0.01). The SF cluster identified 3 subgroups with a particular one characterized by normal SF, low pain, high activity and hyperkinesia. The SR cluster identified 2 distinct subgroups: the one with a reduced SR was distinguished by high FIQ, poor coping and altered affective status. CONCLUSION: Gait analysis may provide additional information in the identification of subgroups among fibromyalgia patients. Gait analysis provided relevant information about physical and cognitive status, and pain behavior. Further studies are needed to better understand gait analysis implications in FM.


Assuntos
Transtornos Cognitivos/diagnóstico , Avaliação da Deficiência , Fibromialgia/classificação , Fibromialgia/diagnóstico , Transtornos Neurológicos da Marcha/diagnóstico , Exame Físico/métodos , Adulto , Biomarcadores , Catastrofização/diagnóstico , Catastrofização/psicologia , Transtornos Cognitivos/epidemiologia , Comorbidade , Feminino , Fibromialgia/epidemiologia , Transtornos Neurológicos da Marcha/epidemiologia , Transtornos Neurológicos da Marcha/psicologia , Humanos , Pessoa de Meia-Idade , Medição da Dor/métodos , Inquéritos e Questionários/normas , Adulto Jovem
19.
Clin Res Hepatol Gastroenterol ; 35(6-7): 475-81, 2011 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-21550330

RESUMO

Among adults, liver involvement is relatively frequent in Langerhans' cell histiocytosis (LCH), even though it is often overlooked. In fact, the liver involvement may be missed in apparently localized LCH or when it is the sole site of involvement. We present 23 cases of liver involvement in LCH out of a cohort study of 85 adult patients included in the French Histiocytosis Study Group Registry. The most frequent clinical setting was multiorgan involvement (87% of our cases). The main histological pattern in liver LCH was sclerosing cholangitis (56% of the cases). The symptoms included hepatomegaly (48%) and/or liver biochemistry abnormalities (61%, including cholestasis associated with increased transaminases levels in 35% of cases, cholestasis only in 22% and increased transaminases levels only in 4% of the cases). Particularly suggestive of the diagnosis was the observation of biliary tree abnormalities through magnetic resonance imaging (MRI). The natural history of liver LCH fits into two stages: early infiltration by histiocytes and late sclerosis of the biliary tree. We found that liver involvement had a significant impact on survival. Thus we suggest that clinical and biological liver evaluation must be performed regularly onwards to screen every LCH adult patient from the time of the initial diagnosis. MRI and liver biopsy should be considered as soon as the data point to a possible liver localization. If this diagnosis is confirmed, we suggest a treatment with ursodesoxycholic acid, as in other cholestatic diseases, together with treatments specifically directed towards LCH. However, the ideal treatment of liver LCH remains to be found, and in advanced cases transplantation is the sole option.


Assuntos
Colangite Esclerosante/etiologia , Colestase/etiologia , Hepatomegalia/etiologia , Histiocitose de Células de Langerhans/complicações , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Antineoplásicos/uso terapêutico , Biópsia , Criança , Pré-Escolar , Colangite Esclerosante/tratamento farmacológico , Colestase/tratamento farmacológico , Estudos de Coortes , Diagnóstico por Imagem , Feminino , Hepatomegalia/tratamento farmacológico , Histiocitose de Células de Langerhans/diagnóstico , Histiocitose de Células de Langerhans/tratamento farmacológico , Humanos , Lactente , Fígado/patologia , Testes de Função Hepática , Masculino , Pessoa de Meia-Idade , Sistema de Registros , Estudos Retrospectivos , Transaminases/análise , Adulto Jovem
20.
Arthritis Care Res (Hoboken) ; 63(8): 1126-33, 2011 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-21485023

RESUMO

OBJECTIVE: To review the literature and collect expert advice for proposing preventive and curative treatments of mouth and dental involvement in patients with systemic sclerosis (SSc; scleroderma). METHODS: The literature pertaining to mouth and/or dental involvement related to SSc was reviewed, and recommendations were developed according to the suggestions of a French multidisciplinary working group of experts and validated by a lecture committee. RESULTS: Dentists face 3 main issues in caring for SSc patients: oral mucosa involvement, manducatory apparatus and mouth involvement responsible for limitations in mouth opening, and treatment-related adverse events. An increased risk of tongue carcinoma has been noted. In patients with severe limitation in mouth opening (<30 mm), recommended treatments are a specific mouth-opening rehabilitation program, flexible sectional dentures, and splint therapy. Indications for dental implants depend on the severity of SSc, comorbidities, and/or ongoing bisphosphonate treatment. Prevention of mouth infections and caries implies patient education and teaching about mouth and dental hygiene, periodontal maintenance, and treatment of sicca syndrome. Cessation of tobacco use is mandatory. Patient-tailored rehabilitation may improve limitations in mouth opening. Systematic dental panoramic radiography allows for the early detection of dental caries. CONCLUSION: Prevention of oral and dental complications is a major issue in patients with SSc. Dental treatment should be tailored to limitations in mouth opening, disease severity, and ongoing treatments.


Assuntos
Assistência Odontológica para Doentes Crônicos , Doenças da Boca/prevenção & controle , Escleroderma Sistêmico/complicações , Doenças Dentárias/prevenção & controle , Humanos , Doenças da Boca/complicações , Escleroderma Sistêmico/terapia , Doenças Dentárias/complicações
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