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2.
Lakartidningen ; 1162019 Sep 26.
Artigo em Sueco | MEDLINE | ID: mdl-31573670

RESUMO

Systemic sclerosis is an autoimmune systemic disease with an annual incidence in Sweden of only 20 cases per million and a standardised mortality rate of 3-4. Disease onset is usually preceded by a period with Raynaud's phenomenon, combined with structurally abnormal nailbed capillaries and accompanied by presence of scleroderma related autoantibodies. The presenting symptoms are skin thickness, puffy fingers, digital ulcers, dysphagia, joint stiffness and pain, and pruritus. Optimal management involves a number of specialists including allied health professionals. Early recognition, diagnosis and treatment are important. The dominating causes of death are cardiopulmonary.


Assuntos
Escleroderma Sistêmico , Autoanticorpos/imunologia , Humanos , Atenção Primária à Saúde , Doenças Raras/complicações , Doenças Raras/diagnóstico , Doenças Raras/patologia , Doenças Raras/terapia , Doença de Raynaud/etiologia , Encaminhamento e Consulta , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/patologia , Escleroderma Sistêmico/terapia
3.
PLoS One ; 14(8): e0221021, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31433819

RESUMO

BACKGROUND: Impaired myocardial deformation has been sporadically described in cardiac asymptomatic systemic sclerosis (SSc). We aimed to study myocardial deformation indices in cardiac asymptomatic SSc patients using cardiac magnetic resonance feature tracking (CMR-FT) and correlate these findings to the phenotypic and autoimmune background. METHODS: Fifty-four cardiac asymptomatic SSc patients (44 females, 56±13 years), with normal routine cardiac assessment and CMR evaluation, including cine and late gadolinium enhancement (LGE) images, were included. SSc patients were compared to 21 sex- and age- matched healthy controls (17 females; 54±19 years). For CMR-FT analysis, a mid-ventricular slice for LV peak systolic radial and circumferential strain and a 4-chamber view for LV/RV peak systolic longitudinal strain were used. RESULTS: Twenty-four patients had diffuse cutaneous SSc and 30 limited cutaneous SSc. Thirteen patients had digital ulcers. Median disease duration was 3.6 years. LV ejection fraction was higher in SSc patients compared to controls (62±6% vs. 59±5%, p = 0.01). Four patients had no LGE examination; in the remaining patients LGE was absent in 74%, while 18% had RV insertion fibrosis and 8% evidence of subendocardial infarction. LV longitudinal strain differed in those with insertion fibrosis (-18.0%) and infarction (-16.7%) compared to no fibrosis (-20.3%, p = 0.04). Patients with SSc had lower RV longitudinal strain and strain rate compared to controls (p<0.001 and p = 0.01, respectively). All other strain and strain rate measurements were non-significant between patients and controls. CONCLUSIONS: In cardiac asymptomatic SSc patients with normal routine functional indices, CMR-FT identifies subclinical presence of insertion fibrosis and/or myocardial infarction by impaired LV longitudinal strain. RV derived longitudinal indices were impaired in the patient group. CMR FT indices did not correlate to the patients' phenotypic and autoimmune features.

4.
Lakartidningen ; 1162019 May 01.
Artigo em Sueco | MEDLINE | ID: mdl-31192407

RESUMO

For patients living with chronic conditions, the daily life will change due to symptoms, drug treatment, and psychosocial factors caused by the disease. Our studies show that Swedish patients with PAH or CTEPH are satisfied with the information they have received, but still have a wish for more. Therefore, it is important to repeat the information on a regular basis and to make sure that patients have understood and remember the messages. In order to increase the knowledge about their disease, improve compliance with treatment and quality of life, patients and next of kin should continuously be informed by the multidisciplinary team at the PAH/CTEPH-specialist care centers.


Assuntos
Hipertensão Pulmonar/psicologia , Educação de Pacientes como Assunto , Embolia Pulmonar/psicologia , Adolescente , Adulto , Distribuição por Idade , Idoso , Idoso de 80 Anos ou mais , Criança , Doença Crônica , Feminino , Conhecimentos, Atitudes e Prática em Saúde , Humanos , Hipertensão Pulmonar/epidemiologia , Masculino , Adesão à Medicação , Pessoa de Meia-Idade , Equipe de Assistência ao Paciente , Satisfação do Paciente , Embolia Pulmonar/epidemiologia , Qualidade de Vida , Distribuição por Sexo , Inquéritos e Questionários , Suécia/epidemiologia , Adulto Jovem
5.
Ann Rheum Dis ; 78(7): 979-987, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-30967395

RESUMO

OBJECTIVE: To assess the safety and efficacy of rituximab in systemic sclerosis (SSc) in clinical practice. METHODS: We performed a prospective study including patients with SSc from the European Scleroderma Trials and Research (EUSTAR) network treated with rituximab and matched with untreated patients with SSc. The main outcomes measures were adverse events, skin fibrosis improvement, lung fibrosis worsening and steroids use among propensity score-matched patients treated or not with rituximab. RESULTS: 254 patients were treated with rituximab, in 58% for lung and in 32% for skin involvement. After a median follow-up of 2 years, about 70% of the patients had no side effect. Comparison of treated patients with 9575 propensity-score matched patients showed that patients treated with rituximab were more likely to have skin fibrosis improvement (22.7 vs 14.03 events per 100 person-years; OR: 2.79 [1.47-5.32]; p=0.002). Treated patients did not have significantly different rates of decrease in forced vital capacity (FVC)>10% (OR: 1.03 [0.55-1.94]; p=0.93) nor in carbon monoxide diffusing capacity (DLCO) decrease. Patients having received rituximab were more prone to stop or decrease steroids (OR: 2.34 [1.56-3.53], p<0.0001). Patients treated concomitantly with mycophenolate mofetil had a trend for better outcomes as compared with patients receiving rituximab alone (delta FVC: 5.22 [0.83-9.62]; p=0.019 as compared with controls vs 3 [0.66-5.35]; p=0.012). CONCLUSION: Rituximab use was associated with a good safety profile in this large SSc-cohort. Significant change was observed on skin fibrosis, but not on lung. However, the limitation is the observational design. The potential stabilisation of lung fibrosis by rituximab has to be addressed by a randomised trial.

7.
Clin Physiol Funct Imaging ; 39(3): 215-225, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30597705

RESUMO

PURPOSE: Patients with pulmonary arterial hypertension (PAH) due to systemic sclerosis (SSc) have high mortality. Left ventricular (LV) peak global longitudinal strain (GLS) is decreased in SSc. It is unknown whether low GLS is due to SSc or PAH. Therefore, our primary aim was to evaluate both LV and right ventricular free wall GLS (RVFW GLS) in SSc, with and without PAH, using cardiac magnetic resonance with feature tracking. Secondary aim was to relate GLS to invasive mean pulmonary arterial pressure (mPAP) and pulmonary vascular resistance (PVR). METHODS: Thirty-eight patients with SSc, 19 patients with SSc-PAH and 19 healthy controls for comparison, were included. Endocardial and epicardial borders were delineated in cine images (short-axis stack and three long-axis views) for volumetric and strain calculations. RESULTS: Systemic sclerosis-PAH had lower LV and RVFW GLS than SSc (LV: P = 0·01, RV: P<0·001) and controls (LV: P = 0·02; RV: P<0·001), with no difference between SSc and controls. LV strain correlated with mPAP (R = 0·42, P = 0·03) and PVR (R = 0·52, P = 0·006). RVFW GLS correlated with mPAP (R = 0·68, P<0·001) and PVR (R = 0·59, P = 0·001). ROC curves for predicting PAH had AUC 0·73 for LV strain (P = 0·003) and 0·86 for RVFW GLS (P<0·001). CONCLUSIONS: Lower GLS is mainly determined by increased pulmonary pressure and not by SSc per se. Low LV and RVFW GLS are indicative of increased mPAP and PVR, which opens for improved non-invasive methods to select patients eligible for right heart catheterization and to monitor the effects of PAH therapy.


Assuntos
Pressão Arterial , Hipertensão Pulmonar/etiologia , Artéria Pulmonar/fisiopatologia , Escleroderma Sistêmico/complicações , Disfunção Ventricular Esquerda/etiologia , Disfunção Ventricular Direita/etiologia , Função Ventricular Esquerda , Função Ventricular Direita , Adulto , Idoso , Feminino , Humanos , Hipertensão Pulmonar/diagnóstico por imagem , Hipertensão Pulmonar/fisiopatologia , Imagem Cinética por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Fatores de Risco , Escleroderma Sistêmico/diagnóstico por imagem , Escleroderma Sistêmico/fisiopatologia , Resistência Vascular , Disfunção Ventricular Esquerda/diagnóstico por imagem , Disfunção Ventricular Esquerda/fisiopatologia , Disfunção Ventricular Direita/diagnóstico por imagem , Disfunção Ventricular Direita/fisiopatologia
8.
Chron Respir Dis ; 16: 1479972318787906, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30011997

RESUMO

Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) share similar quality of life impairment. The aim of the present study was to investigate health-related quality of life (HRQoL) and its relation to the perception of treatment and psychosocial support among PAH and CTEPH patients. All adult PAH or CTEPH patients in the Swedish Pulmonary Arterial Hypertension Register were invited to participate in a national cohort survey. The survey included the EuroQol 5-dimensions (EQ-5D) instrument that measures an individual's HRQoL; the Beliefs about Medicines Questionnaire-Specific Scale that assesses the perception of PAH-specific treatment; the Mastery scale that evaluates the feeling of control and ability to cope with the disease; and the Social Network and Support Scale that maps the social support network. Of the 440 invited patients, 74% responded. Mean age was 66 ± 14 years, 58% were female and 69% diagnosed with PAH. Patients with PAH were younger, more often female and had a lower EQ-5D index (0.67 ± 0.29 vs. 0.73 ± 0.25, p = 0.050) than patients with CTEPH. Patients with a low EQ-5D index had more concerns about treatment ( p = 0.004), lower coping ability ( p < 0.001), less emotional support ( p = 0.003) and less accessible social network ( p = 0.002). In conclusion, patients with an impaired HRQoL also reported negative effects on their social support network, ability to handle stressors and concerns about treatment.

9.
Clin Exp Rheumatol ; 36 Suppl 113(4): 68-75, 2018 Jul-Aug.
Artigo em Inglês | MEDLINE | ID: mdl-30277860

RESUMO

OBJECTIVES: To describe differences in clinical presentation between men and women in a large group of patients with early (<3 years' duration) systemic sclerosis (SSc) according to disease subsets. METHODS: A cross-sectional analysis of the prospective EULAR Scleroderma Trial and Research database (EUSTAR) was performed. Patients fulfilling preliminary ACR 1980 classification criteria for SSc, with less than 3 years from the first non-Raynaud's symptom at first entry, were selected. A group of patients with less than 3 years from the first SSc symptom, including Raynaud's phenomenon, was also analysed. SSc related variables, including antibodies, SSc subsets, disease activity and organ involvement were included. Descriptive and bivariate analyses were performed. RESULTS: A total of 1,027 patients were included, 90% Caucasian, 80% women, and 40% with diffuse cutaneous disease. In early stages of SSc, men showed more frequently than women active disease, diffuse cutaneous subset, anti-Scl-70 antibodies, elevated acute phase reactants, muscular and pulmonary involvement. Differences between men and women were confirmed in the limited, but not in the diffuse SSc subset. The results were similar when 650 patients with less than three years from the first SSc symptom, including Raynaud's phenomenon, were analysed. CONCLUSIONS: In early stages of SSc, men present signs and symptoms of more severe disease. In the limited disease subset, men might appear with clinical features and organ involvement similar to those of the diffuse subgroup. In clinical practice, the identification of such differences might help to select the appropriate management for each particular patient.

10.
Artigo em Inglês | MEDLINE | ID: mdl-30247696

RESUMO

Objectives: Nailfold capillaroscopy is being increasingly used by rheumatologists in the diagnosis of SSc. However, assessment of all nailfolds can be time-consuming in a busy outpatient clinic. Our aim was to answer the question as to how many (and which) fingers a clinician should routinely assess to capture accurately the true state. Methods: A total of 2994 assessments (by an international panel of expert observers) of 1600 images from 173 participants (101 with SSc, 22 with primary RP and 50 healthy controls) were included in this analysis. Seven single-finger or finger combinations (derived from the middle and ring fingers) were then tested for sensitivity for the presence of two markers of capillary abnormality [presence of giant capillaries and an SSc grade (early, active or late)] compared with assessment of all eight fingers. Results: For the eight-finger gold standard, sensitivity against the diagnostic criteria was 74.6% (53.0% for the presence of giants alone and 73.1% for image grade alone). Examining only one finger gave low sensitivity (ranging from right middle 31.7% to left ring 46.6%). Examining both ring fingers gave a sensitivity of 59.8%, whereas examining the four-finger combination of both ring and both middle fingers gave a sensitivity of 66.7%. Conclusion: During routine capillaroscopic examination, ideally all eight nailbeds (excluding thumbs) should be examined, otherwise some abnormalities will be missed. Examining only four fingers reduces capillaroscopy sensitivity.

11.
ESC Heart Fail ; 5(5): 864-875, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-29916558

RESUMO

AIMS: Pre-capillary pulmonary hypertension (PHpre-cap ) has a poor prognosis, especially when caused by pulmonary arterial hypertension (PAH) associated with systemic sclerosis (SSc-PAH). Whether cardiac magnetic resonance (CMR)-based quantification of atrial volumes in PHpre-cap is beneficial in risk assessment is unknown. The aims were to investigate if (i) atrial volumes using CMR are associated with death or lung transplantation in PHpre-cap , (ii) atrial volumes differ among four unmatched major PHpre-cap subgroups, and (iii) atrial volumes differ between SSc-PAH and idiopathic/familial PAH (IPAH/FPAH) when matched for pulmonary vascular resistance (PVR). METHODS AND RESULTS: Seventy-five PHpre-cap patients (57 ± 19 years, 53 female, 43 de novo) with CMR and right heart catheterization were retrospectively included. Short-axis stacks of cine images were analysed, and right and left atrial maximum (RAVmax and LAVmax ) and minimum volume (RAVmin and LAVmin ) were indexed for body surface area. Increased (mean + 2 SD) and reduced (mean - 2 SD) volumes were predefined from CMR normal values. Transplantation-free survival was lower in patients with increased RAVmax than in those with normal [hazard ratio (HR) = 2.1, 95% confidence interval (CI) 1.1-4.0] but did not differ between those with reduced LAVmax and normal (HR 2.0, 95% CI 0.8-5.1). RAVmax and RAVmin showed no differences among unmatched or matched groups (P = ns). When matched for PVR, LAVmax , LAVmin , and pulmonary artery wedge pressure were reduced in SSc-PAH compared with IPAH/FPAH (95% CI 0.3-21.4, 95% CI 0.8-19.6, and 95% CI 2-7, respectively). CONCLUSIONS: Patients with PHpre-cap and increased right atrial volume measured with CMR had worse clinical outcome. When matched for PVR, left atrial volume was lower in SSc-PAH than in IPAH/FPAH, consistent with left-sided underfilling, indicating a potential differentiator between the groups.

12.
Arthritis Rheumatol ; 70(10): 1644-1653, 2018 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-29687634

RESUMO

OBJECTIVE: Systemic sclerosis (SSc) is a major cause of pulmonary arterial hypertension (PAH). Murine models indicate key roles for chemokines CCL19 and CCL21 and their receptor CCR7 in lung inflammation leading to PAH. The objective of this study was to assess the chemokine CCL19-CCL21 axis in patients with SSc-related PAH. METHODS: Serum samples obtained from 2 independent prospective SSc cohorts (n = 326), patients with idiopathic PAH (n = 12), and healthy control subjects (n = 100) were analyzed for CCL19/CCL21 levels, by enzyme-linked immunosorbent assay. The levels were defined as either high or low, using the mean + 2 SD value in controls as the cutoff value. Risk stratification at the time of PAH diagnosis and PAH-related events were performed. Descriptive and Cox regression analyses were conducted. RESULTS: CCL21 levels were higher in patients with SSc compared with controls and were elevated prior to the diagnosis of PAH. PAH was more frequent in patients with high CCL21 levels (≥0.4 ng/ml) than in those with low CCL21 levels (33.3% versus 5.3% [P < 0.001]). In multivariate analyses, CCL21 was associated with PAH (hazard ratio [HR] 5.1, 95% CI 2.39-10.76 [P < 0.001]) and occurrence of PAH-related events (HR 4.7, 95% CI 2.12-10.46, P < 0.001). Risk stratification at the time of PAH diagnosis alone did not predict PAH-related events. However, when risk at diagnosis was combined with high or low CCL21 level, there was a significant predictive effect (HR 1.3, 95% CI 1.03-1.60 [P = 0.027]). A high CCL21 level was associated with decreased survival (P < 0.001). CONCLUSION: CCL21 appears to be a promising marker for predicting the risk of SSc-related PAH and PAH progression. CCL21 may be part of a dysregulated immune pathway linked to the development of lung vascular damage in SSc.

13.
BMC Cardiovasc Disord ; 18(1): 16, 2018 01 30.
Artigo em Inglês | MEDLINE | ID: mdl-29382301

RESUMO

BACKGROUND: Patients with systemic sclerosis (SSc) have high cardiovascular mortality even though there is no or little increase in prevalence of epicardial coronary stenosis. First-pass perfusion on cardiovascular magnetic resonance (CMR) have detected perfusion defects indicative of microvascular disease, but the quantitative extent of hypoperfusion is not known. Therefore, we aimed to determine if patients with SSc have lower global myocardial perfusion (MP) at rest or during adenosine stress, compared to healthy controls, quantified with CMR. METHODS: Nineteen SSc patients (17 females, 61 ± 10 years) and 22 controls (10 females, 62 ± 11 years) underwent CMR. Twelve patients had limited cutaneous SSc and 7 patients had diffuse cutaneous SSc. One patient had pulmonary arterial hypertension (PAH). MP was quantified using coronary sinus flow (CSF) measurements at rest and during adenosine stress, divided by left ventricular mass (LVM). RESULTS: There was no difference in MP at rest between patients and controls (1.1 ± 0.5 vs. 1.1 ± 0.3 ml/min/g, P = 0.85) whereas SSc patients showed statistically significantly lower MP during adenosine stress (3.1 ± 0.9 vs. 4.2 ± 1.3 ml/min/g, P = 0.008). Three out of the 19 SSc patients showed fibrosis in the right ventricle insertion points despite absence of PAH. None had signs of myocardial infarction. CONCLUSIONS: Patients with SSc have decreased MP during adenosine stress compared to healthy controls. Thus hypoperfusion at stress may be a sensitive marker of cardiac disease in SSc patients possibly signifying microvascular myocardial disease.


Assuntos
Adenosina/administração & dosagem , Circulação Coronária , Cardiopatias/diagnóstico por imagem , Imagem Cinética por Ressonância Magnética , Microcirculação , Imagem de Perfusão do Miocárdio/métodos , Escleroderma Sistêmico/complicações , Vasodilatadores/administração & dosagem , Adulto , Idoso , Idoso de 80 Anos ou mais , Estudos de Casos e Controles , Estudos Transversais , Feminino , Fibrose , Cardiopatias/etiologia , Cardiopatias/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Miocárdio/patologia , Valor Preditivo dos Testes , Escleroderma Sistêmico/diagnóstico , Remodelação Ventricular
14.
Ann Rheum Dis ; 77(7): 1032-1038, 2018 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-29463517

RESUMO

OBJECTIVES: The aim of this study was to adapt the Systemic Sclerosis Quality of Life Questionnaire (SScQoL) into six European cultures and validate it as a common measure of quality of life in systemic sclerosis (SSc). METHODS: This was a seven-country (Germany, France, Italy, Poland, Spain, Sweden and UK) cross-sectional study. A forward-backward translation process was used to adapt the English SScQoL into target languages. SScQoL was completed by patients with SSc, then data were validated against the Rasch model. To correct local response dependency, items were grouped into the following subscales: function, emotion, sleep, social and pain and reanalysed for fit to the model, unidimensionality and cross-cultural equivalence. RESULTS: The adaptation of the SScQoL was seamless in all countries except Germany. Cross-cultural validation included 1080 patients with a mean age 58.0 years (SD 13.9) and 87% were women. Local dependency was evident in individual country data. Grouping items into testlets corrected the local dependency in most country specific data. Fit to the model, reliability and unidimensionality was achieved in six-country data after cross-cultural adjustment for Italy in the social subscale. The SScQoL was then calibrated into an interval level scale. CONCLUSION: The individual SScQoL items have translated well into five languages and overall, the scale maintained its construct validity, working well as a five-subscale questionnaire. Measures of quality of life in SSc can be directly compared across five countries (France, Poland Spain, Sweden and UK). Data from Italy are also comparable with the other five countries although require an adjustment.

16.
SAGE Open Med ; 6: 2050312117749159, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29326818

RESUMO

Objectives: Pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension are severe diseases with complicated treatment that need care at specialist clinics. The aim was to investigate changes in the patients' perceptions on coping, social support and received information when attending a newly started nurse-coordinated pulmonary arterial hypertension-outpatient clinic. Methods: The present study was a descriptive, questionnaire-based cohort study including 42 adult patients. To evaluate coping, the Pearlin Mastery Scale was used. Social support, information and health-related quality of life were measured using Social Network and Support Scale, QLQ-INFO25 and the EQ-5D. Results: Attending the pulmonary arterial hypertension-outpatient clinic increased coping ability (Mastery Scale) significantly (baseline 16.0 ± 3.3 points vs 2-year follow-up 19.6 ± 5.2 points, p < 0.001) while there was no difference in social network and support or in perception of received information after. Patients who improved their coping ability (67%) were younger, had better exercise capacity, experienced better health-related quality of life and were more satisfied with received information about treatment and medical tests than those who reduced the coping ability. There was no difference in gender, diagnosis, time since diagnose, pulmonary arterial hypertension-specific treatment, education level or civil status between the two groups. Conclusion: This study suggests that the pulmonary arterial hypertension-team, in partnership with the patient, can support patients to take control of their disease and increase their health-related quality of life.

17.
Clin Respir J ; 12(6): 2029-2035, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29356440

RESUMO

BACKGROUND: Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are rare diseases with a gradual decline in physical health. Adherence to treatment is crucial in these very symptomatic and life threatening diseases. OBJECTIVE: To describe PAH and CTEPH patients experience of their self-reported medication adherence, beliefs about medicines and information about treatment. METHODS: A quantitative, descriptive, national cohort survey that included adult patients from all PAH-centres in Sweden. All patients received questionnaires by mail: The Morisky Medication Adherence Scale (MMAS-8) assesses treatment-related attitudes and behaviour problems, the Beliefs about Medicines Questionnaire-Specific scale (BMQ-S) assesses the patient's perception of drug intake and the QLQ-INFO25 multi-item scale about medical treatment information. RESULTS: The response rate was 74% (n = 325), mean age 66 ± 14 years, 58% were female and 69% were diagnosed with PAH and 31% with CTEPH. Time from diagnosis was 4.7 ± 4.2 years. More than half of the patients (57%) reported a high level of adherence. There was no difference in the patients' beliefs of the necessity of the medications to control their illness when comparing those with high, medium or low adherence. Despite high satisfaction with the information, concerns about potential adverse effects of taking the medication were significantly related to adherence. CONCLUSIONS: Treatment adherence is relatively high but still needs improvement. The multi-disciplinary PAH team should, together with the patient, seek strategies to improve adherence and prevent concern.


Assuntos
Anti-Hipertensivos/uso terapêutico , Fibrinolíticos/uso terapêutico , Hipertensão Pulmonar/tratamento farmacológico , Adesão à Medicação , Vigilância da População , Embolia Pulmonar/tratamento farmacológico , Autorrelato , Idoso , Atitude Frente a Saúde , Doença Crônica , Estudos Transversais , Feminino , Humanos , Hipertensão Pulmonar/epidemiologia , Hipertensão Pulmonar/etiologia , Masculino , Morbidade/tendências , Prognóstico , Embolia Pulmonar/complicações , Embolia Pulmonar/epidemiologia , Inquéritos e Questionários , Suécia/epidemiologia
19.
Ann Rheum Dis ; 77(4): 563-570, 2018 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-29306872

RESUMO

OBJECTIVES: Our aim was to use the opportunity provided by the European Scleroderma Observational Study to (1) identify and describe those patients with early diffuse cutaneous systemic sclerosis (dcSSc) with progressive skin thickness, and (2) derive prediction models for progression over 12 months, to inform future randomised controlled trials (RCTs). METHODS: The modified Rodnan skin score (mRSS) was recorded every 3 months in 326 patients. 'Progressors' were defined as those experiencing a 5-unit and 25% increase in mRSS score over 12 months (±3 months). Logistic models were fitted to predict progression and, using receiver operating characteristic (ROC) curves, were compared on the basis of the area under curve (AUC), accuracy and positive predictive value (PPV). RESULTS: 66 patients (22.5%) progressed, 227 (77.5%) did not (33 could not have their status assessed due to insufficient data). Progressors had shorter disease duration (median 8.1 vs 12.6 months, P=0.001) and lower mRSS (median 19 vs 21 units, P=0.030) than non-progressors. Skin score was highest, and peaked earliest, in the anti-RNA polymerase III (Pol3+) subgroup (n=50). A first predictive model (including mRSS, duration of skin thickening and their interaction) had an accuracy of 60.9%, AUC of 0.666 and PPV of 33.8%. By adding a variable for Pol3 positivity, the model reached an accuracy of 71%, AUC of 0.711 and PPV of 41%. CONCLUSIONS: Two prediction models for progressive skin thickening were derived, for use both in clinical practice and for cohort enrichment in RCTs. These models will inform recruitment into the many clinical trials of dcSSc projected for the coming years. TRIAL REGISTRATION NUMBER: NCT02339441.

20.
Rheumatology (Oxford) ; 57(4): 625-630, 2018 04 01.
Artigo em Inglês | MEDLINE | ID: mdl-29325173

RESUMO

Objective: To study the impact of disease and treatment with DMARDs on antibody response elicited by either pneumococcal conjugate vaccine (PCV13) or pneumococcal polysaccharide vaccine (PPV23) in patients with SSc. Methods: Forty-four SSc patients and 49 controls received a dose of either PCV13 or PPV23. Twelve patients were treated with DMARDs. Antibody levels to pneumococcal polysaccharides 6B and 23 F were measured before and 4-6 weeks after vaccination using ELISA. Antibody functionality was studied using opsonophagocytic assay performed on serotype 23 F. Results: Number of patients, percentage female and mean age (years) at vaccination were: 32, 94%, 57.5 years in SSc without DMARDs; 12, 100%, 55.5 years in SSc on DMARDs and 49, 63% and 50.6 years in controls. Post-vaccination antibody levels for both serotypes increased significantly in SSc without DMARDs and controls (P < 0.001), but in SSc on DMARDs only for 6B (P = 0.041). Compared with the other groups, patients with SSc receiving DMARDs had lower post-vaccination antibody levels for both serotypes. Opsonophagocytic assay increased significantly in all three groups. No significant difference in immunogenicity between PCV13 and PPV23 was seen. Conclusion: Pneumococcal vaccination using either PCV13 or PPV23 yielded satisfactory antibody response in SSc patients without DMARD treatment, but a lower response in patients treated with synthetic DMARDs. Type of pneumococcal vaccine (conjugate or polysaccharide) did not significantly influence antibody response. Trial registration: ClinicalTrials.gov, http://clinicaltrials.gov, NCT02240888.


Assuntos
Vacinas Pneumocócicas/uso terapêutico , Escleroderma Sistêmico/tratamento farmacológico , Streptococcus pneumoniae/imunologia , Vacinação/métodos , Anticorpos Anti-Idiotípicos/sangue , Anticorpos Anti-Idiotípicos/imunologia , Antirreumáticos/uso terapêutico , Quimioterapia Combinada , Ensaio de Imunoadsorção Enzimática , Feminino , Humanos , Imunoglobulina G/imunologia , Masculino , Pessoa de Meia-Idade , Vacinas Pneumocócicas/imunologia , Escleroderma Sistêmico/sangue , Escleroderma Sistêmico/imunologia , Resultado do Tratamento
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