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OBJECTIVES: The digital ulcers of systemic sclerosis are disabling and frequent· Their pathogenesis involves a capillary microangiopathy and a digital arterial disease that few studies were able to quantify up to now. A multicentre observational study about the predictive value of capillaroscopy in systemic sclerosis offered us the opportunity to evaluate further the complementary information provided by both capillary and arterial evaluations. METHODS: During the SCLEROCAP study, five out of the nine centers performed a systematic evaluation of the finger brachial pressure index (FBPI) in the last four fingers of both hands at baseline, using the same laser-doppler device. In the present work, FBPI measurements were compared between fingers with vs without digital ulcers or scars, before and after adjusting for the capillaroscopic pattern and systemic factors. RESULTS: FBPI measurements were performed in 2537 fingers from 326 patients. Active ulcers or scars were found in 10·8% of those fingers, more often on the right hand, and in the second and third fingers. FBPI was lower than 0·70 in 26% of all fingers and in 57·5% of those with ulcers. A strong association was found between a low FBPI and the presence of digital ulcers, even after adjusting for capillaroscopic pattern, ulcer location and the patient himself. CONCLUSION: These results confirm the importance of digital arterial disease in the pathogenesis of digital ulcers of systemic sclerosis, which is independent from the microangiopathy. FBPI measurements complement the information provided by capillaroscopy and might have an important predictive value for subsequent digital ulcers.
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OBJECTIVE: To evaluate the performance of machine learning and then deep learning to detect a systemic scleroderma (SSc) landscape from the same set of nailfold capillaroscopy (NC) images from the French prospective multicenter observational study SCLEROCAP. METHODS: NC images from the first 100 SCLEROCAP patients were analyzed to assess the performance of machine learning and then deep learning in identifying the SSc landscape, the NC images having previously been independently and consensually labeled by expert clinicians. Images were divided into a training set (70 %) and a validation set (30 %). After features extraction from the NC images, we tested six classifiers (random forests (RF), support vector machine (SVM), logistic regression (LR), light gradient boosting (LGB), extreme gradient boosting (XGB), K-nearest neighbors (KNN)) on the training set with five different combinations of the images. The performance of each classifier was evaluated by the F1 score. In the deep learning section, we tested three pre-trained models from the TIMM library (ResNet-18, DenseNet-121 and VGG-16) on raw NC images after applying image augmentation methods. RESULTS: With machine learning, performance ranged from 0.60 to 0.73 for each variable, with Hu and Haralick moments being the most discriminating. Performance was highest with the RF, LGB and XGB models (F1 scores: 0.75-0.79). The highest score was obtained by combining all variables and using the LGB model (F1 score: 0.79 ± 0.05, p < 0.01). With deep learning, performance reached a minimum accuracy of 0.87. The best results were obtained with the DenseNet-121 model (accuracy 0.94 ± 0.02, F1 score 0.94 ± 0.02, AUC 0.95 ± 0.03) as compared to ResNet-18 (accuracy 0.87 ± 0.04, F1 score 0.85 ± 0.03, AUC 0.87 ± 0.04) and VGG-16 (accuracy 0.90 ± 0.03, F1 score 0.91 ± 0.02, AUC 0.91 ± 0.04). CONCLUSION: By using machine learning and then deep learning on the same set of labeled NC images from the SCLEROCAP study, the highest performances to detect SSc landscape were obtained with deep learning and in particular DenseNet-121. This pre-trained model could therefore be used to automatically interpret NC images in case of suspected SSc. This result nevertheless needs to be confirmed on a larger number of NC images.
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BACKGROUND: A French intersociety consensus on behalf the Société Française de Médecine Vasculaire and the Société de Chirurgie Vasculaire et Endovasculaire was proposed in 2021 for the management of patients with lower extremity peripheral artery disease (LEAD). Recent studies have been published and an update of this consensus about the management of low-density lipoprotein cholesterol (LDLc) and hypertriglyceridemia was required. METHODS: A steering committee of 12 vascular physicians and surgeons defined questions of interest about LDLc and hypertriglyceridemia management. A French expert panel voted the proposals. Consensus was considered to have been achieved if more than 80% of the responses corresponded to either "Agreement" or "Disagreement". RESULTS: Among the 56 experts who were asked to participate, 46 (82%) accepted. After the first round of the Delphi procedure, the 4 proposals reached consensus. The following suggestions and recommendations were approved: 1. For LEAD patients treated by the highest tolerated statin dose ± ezetimibe and who have an LDLc ≥0.70 g/L, we recommend adding a proprotein convertase subtilisin/kexin type 9 inhibitor. 2. For LEAD patients treated by statin and who have elevated triglyceride level between ≥150 mg/dL and ≤500 mg/dL, we suggest adding Icosapent Ethyl. 3. Before adding Icosapent Ethyl in LEAD patients treated with statin, we suggest looking for symptoms that may suggest atrial fibrillation. 4. For LEAD patients treated by Icosapent Ethyl and who have symptoms that suggest atrial fibrillation, we recommend performing an electrocardiogram. CONCLUSIONS: This update will help clinicians to improve LEAD patient management.
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Fibrilación Atrial , Cardiología , Inhibidores de Hidroximetilglutaril-CoA Reductasas , Hipertrigliceridemia , Enfermedad Arterial Periférica , Humanos , Inhibidores de Hidroximetilglutaril-CoA Reductasas/efectos adversos , LDL-Colesterol , Consenso , Resultado del Tratamiento , Hipertrigliceridemia/complicaciones , Hipertrigliceridemia/diagnóstico , Hipertrigliceridemia/tratamiento farmacológico , Enfermedad Arterial Periférica/diagnóstico , Enfermedad Arterial Periférica/cirugíaRESUMEN
Background: In the latest American Heart Association guidelines, influenza vaccination is recommended for patients with peripheral arterial disease (PAD). The vaccination coverage in this specific population is currently unknown. This study aims to determine the adherence to influenza vaccination in a PAD population and identify associated determinants. Patients and methods. Hospitalized patients and outpatients with PAD from two university departments of vascular medicine were prospectively included. A questionnaire was administered to collect sociodemographic data, cardiovascular risk factors, influenza vaccination status, history of cardiovascular disease, and perception and knowledge about vaccination. Logistic regression was conducted to assess vaccination determinants. Results: Over a six-month period, 494 patients were included (median age 69.5, IQR [63-77], 78% male). Overall, 60.1% were either vaccinated or intended to be (Group 1). Vaccination was associated with age (odds-ratio [OR]=1.055, 95% confidence intervals [95%CI]: 1.035-1.075, p<0.0001), abdominal aorta aneurysm (OR=0.390, 95%CI: 0.229-0.664, p=0.001), chronic obstructive pulmonary disease (OR=0.545, 95%CI: 0.367-0.810, p=0.003), chronic renal disease (OR=0.630, 95%CI: 0.400-0.993, p=0.046), and valvulopathy (OR=2.444, 95%CI: 1.122-5.326, p=0.025). Only 25.3% received vaccination information mainly from their general practitioners. Among patients against vaccination, 59.9% considered themselves not concerned about potential influenza consequences on their PAD, and 37.6% did not intend to change their decision. Conclusions: This study highlights the low adherence to influenza vaccination in the PAD population of 2 university hospital centers. Vaccination is often related to age, and there is a need for adapted information regarding influenza consequences on cardiovascular disease overall, particularly on PAD. Addressing common information and advice about vaccination will be a challenge.
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Conocimientos, Actitudes y Práctica en Salud , Vacunas contra la Influenza , Gripe Humana , Enfermedad Arterial Periférica , Cobertura de Vacunación , Humanos , Enfermedad Arterial Periférica/epidemiología , Masculino , Femenino , Anciano , Vacunas contra la Influenza/administración & dosificación , Persona de Mediana Edad , Gripe Humana/prevención & control , Estudios Prospectivos , Cobertura de Vacunación/estadística & datos numéricos , Factores de Riesgo , Factores de Edad , VacunaciónRESUMEN
Systemic sclerosis may be complicated by digital ulcers. Nailfold capillaroscopy on one finger might reflect an increased risk of digital ulcer (DU). In the present study we studied the correlations between a history of ulcer and capillary findings on the finger. METHOD: This study is part of Sclerocap, a multicenter study aiming at validating prospectively the prognostic value of Maricq's and Cutolo's capillaroscopic classifications during a three-year longitudinal follow-up. A history of past or present digital ulcer was recorded at inclusion and nailfold capillaroscopy was performed. Elementary findings as well as Cutolo and Maricq's classifications were assessed. RESULTS: 387 patients were included in Sclerocap (327 females, 60 males) and 3096 fingers were examined by capillaroscopy at inclusion: 316 fingers (10%) belonging to 113 patients had a history of DU. Late Cutolo's stage was statistically correlated with a history of DU, both by univariate: OR 2.08 [1.09-3.96] and multivariate analysis: OR 1.97 [1.06-3.63]. Among the elemental abnormalities, only edema and decreased capillary density were correlated with a history of DU by multivariate analysis: respectively OR 1.92 [1.17-3.16] and 0.65 [0.49-0.85]. CONCLUSION: This cross-sectional study in a large cohort of patients with systemic sclerosis shows a correlation between a history of digital ulcer and edema, a decrease in capillary density and the late stage in Cutolo's classification. The extent of capillary abnormalities on one finger is associated with a history of local digital ulcer. Capillaroscopy might be used to predict the risk of DU but these results need first to be confirmed by prospective studies.
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Esclerodermia Sistémica , Úlcera Cutánea , Capilares/diagnóstico por imagen , Estudios Transversales , Femenino , Dedos/irrigación sanguínea , Humanos , Masculino , Angioscopía Microscópica/métodos , Uñas , Estudios Prospectivos , Esclerodermia Sistémica/complicaciones , Esclerodermia Sistémica/diagnóstico , Úlcera Cutánea/diagnóstico , Úlcera Cutánea/etiología , Úlcera/complicacionesRESUMEN
OBJECTIVE: Innate lymphoid cells-2 (ILC2) were shown to be involved in the development of lung or hepatic fibrosis. We sought to explore the functional and phenotypic heterogeneity of ILC2 in skin fibrosis within systemic sclerosis (SSc). METHODS: Blood samples and skin biopsies from healthy donor or patients with SSc were analysed by immunostaining techniques. The fibrotic role of sorted ILC2 was studied in vitro on dermal fibroblast and further explored by transcriptomic approach. Finally, the efficacy of a new treatment against fibrosis was assessed with a mouse model of SSc. RESULTS: We found that ILC2 numbers were increased in the skin of patients with SSc and correlated with the extent of skin fibrosis. In SSc skin, KLRG1- ILC2 (natural ILC2) were dominating over KLRG1+ ILC2 (inflammatory ILC2). The cytokine transforming growth factor-ß (TGFß), whose activity is increased in SSc, favoured the expansion of KLRG1- ILC2 simultaneously decreasing their production of interleukin 10 (IL10), which regulates negatively collagen production by dermal fibroblasts. TGFß-stimulated ILC2 also increased myofibroblast differentiation. Thus, human KLRG1- ILC2 had an enhanced profibrotic activity. In a mouse model of SSc, therapeutic intervention-combining pirfenidone with the administration of IL10 was required to reduce the numbers of skin infiltrating ILC2, enhancing their expression of KLRG1 and strongly alleviating skin fibrosis. CONCLUSION: Our results demonstrate a novel role for natural ILC2 and highlight their inter-relationships with TGFß and IL10 in the development of skin fibrosis, thereby opening up new therapeutic approaches in SSc.
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Fibroblastos/metabolismo , Linfocitos/inmunología , Esclerodermia Sistémica/inmunología , Piel/patología , Factor de Crecimiento Transformador beta/inmunología , Adulto , Animales , Antiinflamatorios no Esteroideos/farmacología , Biopsia , Diferenciación Celular , Colágeno/metabolismo , Modelos Animales de Enfermedad , Femenino , Fibrosis , Perfilación de la Expresión Génica , Humanos , Interleucina-10/inmunología , Interleucina-10/farmacología , Lectinas Tipo C/metabolismo , Linfocitos/efectos de los fármacos , Linfocitos/metabolismo , Masculino , Ratones , Persona de Mediana Edad , Miofibroblastos/citología , Piridonas/farmacología , Receptores Inmunológicos/metabolismo , Esclerodermia Sistémica/metabolismo , Esclerodermia Sistémica/patología , Piel/citología , Piel/efectos de los fármacosRESUMEN
OBJECTIVE: The aim of this study was to examine the external applicability of the COMPASS and the VOYAGER-PAD trials in patients with lower extremity artery disease (LEAD) in the real world. METHODS: This was a multicentre retrospective analysis of prospectively collected COPART data, a French multicentre registry of patients hospitalised for symptomatic LEAD. The proportion of patients eligible for the combination of rivaroxaban 2.5 mg twice daily plus aspirin based on either COMPASS or VOYAGER-PAD criteria is reported. The one year cumulative incidence of outcomes between eligible and non-eligible patients, as well as eligible patients vs. control arms of the COMPASS (LEAD patient subgroup) and the VOYAGER-PAD trials were compared. Analyses were performed using Cox models. RESULTS: Of 2 259 evaluable patients, only 679 (30.1%) were eligible for a low dose rivaroxaban plus aspirin regimen. Others were not eligible because of the need for anticoagulant (48.5% and 38.9% of patients meeting COMPASS and VOYAGER-PAD exclusion criteria, respectively) or dual antiplatelet therapy use (15.7% and 16.5%, respectively), high bleeding risk (14.4% and 11.6%, respectively), malignancy (26.1% and 21.0%, respectively), history of ischaemic/haemorrhagic stroke (21.1% and 19.8%, respectively), and severe renal failure (13.2% and 10.5%, respectively). COMPASS and VOYAGER-PAD eligible and ineligible patients were at higher risk of ischaemic events than participants in these trials. The one year cumulative incidences were 6.0% (95% CI 4.3 - 8.1) in the COMPASS eligible subset vs. 3.5% (95% CI 2.9 - 4.3) in the COMPASS control arm for major adverse cardiovascular events, and 27.9% (95% CI 19.9 - 38.3) in the VOYAGER-PAD eligible subset vs. 6.0% (95% CI 5.3 - 6.9) in the VOYAGER-PAD control arm for major adverse limb events. CONCLUSION: Many patients hospitalised for symptomatic LEAD in France are not eligible for the low dose rivaroxaban plus aspirin combination. In turn, those eligible may potentially have greater absolute benefit because of higher risk than those enrolled in the trials.
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Aspirina/uso terapéutico , Inhibidores del Factor Xa/uso terapéutico , Isquemia/prevención & control , Extremidad Inferior/irrigación sanguínea , Enfermedad Arterial Periférica/tratamiento farmacológico , Inhibidores de Agregación Plaquetaria/uso terapéutico , Rivaroxabán/uso terapéutico , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Esquema de Medicación , Quimioterapia Combinada , Femenino , Francia , Hospitalización , Humanos , Incidencia , Isquemia/epidemiología , Isquemia/etiología , Extremidad Inferior/patología , Masculino , Persona de Mediana Edad , Enfermedad Arterial Periférica/fisiopatología , Guías de Práctica Clínica como Asunto , Ensayos Clínicos Controlados Aleatorios como Asunto , Sistema de Registros , Estudios Retrospectivos , Resultado del Tratamiento , Adulto JovenRESUMEN
Several international guidelines concerning lower extremity arterial disease (LEAD) have been published recently, in particular, by the American Heart Association the European Society of Cardiology/European Society for Vascular Surgery, the European Society for Vascular Medicine and the Society for Vascular Surgery. These guidelines differ in some respects and certain issues are not addressed. The objective of this consensus driven by the French Societies of vascular Medicine and surgery was to analyze the disparities between the different guidelines, as well as certain issues not covered, and develop proposals with regard to these points. The following fields of LEAD have been explored: 1) classifications, 2) clinical evaluation, 3) diagnostic criteria, 4) quantification of arterial stenosis using duplex ultrasound, 5) detection of asymptomatic multisite lesions, 6) screening for LEAD in the context of cardiac disease, 7) medical treatment, 8) supervised exercise therapy, 9) revascularization and revascularization of the internal artery stenosis, 10) management of chronic limb ischemia, 11) longitudinal follow-up, and 12) diet.
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Fármacos Cardiovasculares/uso terapéutico , Procedimientos Endovasculares/normas , Terapia por Ejercicio/normas , Disparidades en Atención de Salud , Extremidad Inferior/irrigación sanguínea , Enfermedad Arterial Periférica/terapia , Procedimientos Quirúrgicos Vasculares/normas , Fármacos Cardiovasculares/efectos adversos , Consenso , Técnica Delphi , Dieta Saludable , Procedimientos Endovasculares/efectos adversos , Medicina Basada en la Evidencia/normas , Terapia por Ejercicio/efectos adversos , Humanos , Enfermedad Arterial Periférica/diagnóstico por imagen , Conducta de Reducción del Riesgo , Sociedades Médicas , Resultado del Tratamiento , Procedimientos Quirúrgicos Vasculares/efectos adversosRESUMEN
OBJECTIVE: Finger systolic blood pressure measurement (FSBP) has been shown helpful in the detection of distal arterial insufficiency in upper limbs. This work assesses the possibility to measure FSBP on the 2nd phalanx instead of the first one in order to improve its sensitivity and to verify this would not alter the repeatability of the measurement. METHODS: In this multicenter study, FSBP was measured twice in all fingers but the thumbs in consecutive systemic sclerosis patients on the first phalanx and the second phalanx in alternate order using laser-Doppler flowmetry. RESULTS: Thirty-seven patients were enrolled. The repeatability of FSBP was excellent and similar on the first and 2nd phalanxes with coefficients of variation respectively of 7.1% and 7.6%. While the correlation between the FSBP at the two sites was fair (Pearson coefficient 0.69; p < 0.001). The agreement was poor, with a mean difference of 14 mm Hg between the two sites. Significantly higher differences were found in fingers with digital ulcers. The ROC curves showed a better prediction of the 2nd phalanx measurements. CONCLUSION: FSBP has an excellent repeatability whatever the site of phalanx. However, measurements performed on the 2nd phalanx have a better sensitivity for the prediction of digital ulcers.
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Determinación de la Presión Sanguínea/métodos , Presión Sanguínea , Dedos/irrigación sanguínea , Flujometría por Láser-Doppler , Esclerodermia Sistémica/diagnóstico , Úlcera Cutánea/diagnóstico , Velocidad del Flujo Sanguíneo , Humanos , Estudios Longitudinales , Paris , Valor Predictivo de las Pruebas , Estudios Prospectivos , Reproducibilidad de los Resultados , Esclerodermia Sistémica/fisiopatología , Úlcera Cutánea/fisiopatología , Factores de TiempoRESUMEN
Autosomal dominant inherited Protein S deficiency (PSD) (MIM 612336) is a rare disorder caused by rare mutations, mainly located in the coding sequence of the structural PROS1 gene, and associated with an increased risk of venous thromboembolism. To identify the molecular defect underlying PSD observed in an extended French pedigree with seven PSD affected members in whom no candidate deleterious PROS1 mutation was detected by Sanger sequencing of PROS1 exons and their flanking intronic regions or via an multiplex ligation-dependent probe amplification (MLPA) approach, a whole genome sequencing strategy was adopted. This led to the identification of a never reported C to T substitution at c.-39 from the natural ATG codon of the PROS1 gene that completely segregates with PSD in the whole family. This substitution ACGâATG creates a new start codon upstream of the main ATG. We experimentally demonstrated in HeLa cells that the variant generates a novel overlapping upstream open reading frame (uORF) and inhibits the translation of the wild-type PS. This work describes the first example of 5'UTR PROS1 mutation causing PSD through the creation of an uORF, a mutation that is not predicted to be deleterious by standard annotation softwares, and emphasizes the need for better exploration of such type of non-coding variations in clinical genomics.
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Regiones no Traducidas 5'/genética , Codón Iniciador/genética , Mutación/genética , Biosíntesis de Proteínas , Deficiencia de Proteína S/genética , Proteína S/genética , Secuencia de Bases , Femenino , Células HeLa , Humanos , Masculino , Linaje , Adulto JovenRESUMEN
OBJECTIVES: We assessed the correlation between severity of systemic sclerosis (SSc) and current staging systems based on nailfold capillaroscopy. METHODS: SCLEROCAP is a multicenter prospective study including consecutive scleroderma patients who have a yearly routine follow-up with capillaroscopy and digital blood pressure measurement. Capillaroscopy images were read by two observers blinded from each other, then by a third one in the case of discordance. A follow-up of 3 years is planned. The present study assessed the correlation between severity of systemic sclerosis (SSc) and current staging systems based on nail fold capillaroscopy at enrollment in the SCLEROCAP study. Univariate and multivariate logistic regression analysis was performed for both the Maricq and Cutolo classifications. RESULTS: SCLEROCAP included 387 patients in one year. Maricq's active and Cutolo's late classifications were very similar. In multivariate analysis, the number of digital ulcers (OR for 2 ulcers or more, respectively 2.023 [1.074-3.81] and 2.596 [1.434-4.699]) and Rodnan's skin score >15 (OR respectively 32.007 [6.457-158.658] and 18.390 [5.380-62.865]) correlated with Maricq's active and Cutolo's late stages. Haemoglobin rate correlated with Cutolo's late stage (hemoglobin<100 vs. >120 g/dl: OR 0.223 [0.051-0.980]), and total lung capacity with Maricq's active one: increase in 10%: OR0.833 [0.717-0.969]. CONCLUSIONS: The correlations found between capillaroscopy and severity of SSc are promising before the ongoing prospective study definitively assesses whether capillaroscopy staging predicts complications of SSc. Only two capillaroscopic patterns seem useful: one involving many giant capillaries and haemorrhages and the other with severe capillary loss.
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Angioscopía Microscópica/métodos , Esclerodermia Sistémica , Úlcera Cutánea , Capilares , Femenino , Humanos , Masculino , Uñas , Estudios Prospectivos , Esclerodermia Sistémica/clasificación , Esclerodermia Sistémica/patología , Índice de Severidad de la Enfermedad , Úlcera Cutánea/clasificación , Úlcera Cutánea/patologíaAsunto(s)
Procedimientos Endovasculares , Enfermedad Arterial Periférica , Humanos , Isquemia Crónica que Amenaza las Extremidades , Iloprost/efectos adversos , Estudios de Cohortes , Enfermedad Arterial Periférica/diagnóstico , Enfermedad Arterial Periférica/tratamiento farmacológico , Enfermedad Arterial Periférica/etiología , Isquemia/diagnóstico , Isquemia/tratamiento farmacológico , Isquemia/etiología , Recuperación del Miembro , Resultado del Tratamiento , Estudios Retrospectivos , Enfermedad Crónica , Procedimientos Endovasculares/efectos adversosRESUMEN
BACKGROUND: Skin pigmentation disorders in systemic sclerosis (SSc) have been sparsely described in the literature. Nevertheless, they could be a diagnostic and/or severity marker. OBJECTIVES: To assess the association between pigmentation disorders and systemic involvement in patients with SSc. METHODS: A total of 5 patterns of skin pigmentation disorders were defined: diffuse hyperpigmentation; hyperpigmentation of sun-exposed areas; hypopigmentation of the head, neck, and/or upper part of the chest; acral hypopigmentation; and diffuse hypopigmentation. RESULTS: A total of 239 patients were included; 88 patients (36.8%) had skin pigmentation disorders as follows: diffuse hyperpigmentation and hyperpigmentation of sun-exposed areas in 38.6% (n = 34) and 27.3% (n = 24) of patients, respectively; hypopigmentation of the face, neck, and/or chest in 10.2% of patients (n = 9); diffuse hypopigmentation in 12.5% (n = 11); and acral hypopigmentation in 17% (n = 15). Diffuse hyperpigmentation was associated with diffuse SSc (P = .001), increased modified Rodnan skin score (P = .001), and shorter duration of Raynaud phenomenon (P = .002) in univariate analysis but not in multivariate analysis. Moreover, diffuse hyperpigmentation was associated with digital ulcers (P = .005), as confirmed by multivariate analysis (odds ratio, 2.96; 95% confidence interval, 1.28-6.89). LIMITATIONS: This was a single-center retrospective study of a cohort of patients with SSc. CONCLUSION: Screening for skin pigmentation disorders could be useful in the management of patients with SSc to identify those with a high risk of development of digital ulcers, which is a symptom of vascular involvement in SSc.
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Dedos/patología , Hiperpigmentación/epidemiología , Esclerodermia Sistémica/epidemiología , Úlcera Cutánea/epidemiología , Adulto , Distribución por Edad , Anciano , Estudios de Cohortes , Comorbilidad , Femenino , Francia , Humanos , Hiperpigmentación/diagnóstico , Hiperpigmentación/terapia , Modelos Logísticos , Masculino , Persona de Mediana Edad , Análisis Multivariante , Prevalencia , Enfermedad de Raynaud/fisiopatología , Estudios Retrospectivos , Esclerodermia Sistémica/diagnóstico , Esclerodermia Sistémica/terapia , Índice de Severidad de la Enfermedad , Distribución por Sexo , Úlcera Cutánea/diagnóstico , Úlcera Cutánea/terapia , Estadísticas no ParamétricasRESUMEN
Chronic critical lower limb ischemia (CLI) has been defined as ischemia that endangers the leg. An attempt was made to give a precise definition of CLI, based on clinical and hemodynamic data (Second European Consensus). CLI may be easily defined from a clinical point of view as rest pain of the distal foot or gangrene or ulceration. It is probably useful to add leg ulcers of other origin which do not heal because of severe ischemia, and to consider the impact of frailty on adverse outcome. From a hemodynamic viewpoint there is no consensus and most of the existing classifications are not based upon evidence. We should thus propose a definition and then validate it in a prospective cohort in order to define the patients at major risk of amputation, and also to define the categories of patients whose prognosis is improved by revascularisation. From today's available data, it seems clear that the patients with a systolic toe pressure (STP) below 30 mmHg must be revascularised whenever possible. However other patients with clinically suspected CLI and STP above 30 mmHg must be evaluated and treated in specialised vascular units and revascularisation has to be discussed on a case by case basis, taking into account other data such as the WiFi classification for ulcers.In conclusion, many useful but at times contradictory definitions of CLI have been suggested. Only a few have taken into account evidence, and none have been validated prospectively. This paper aims to address this and to give notice that a CLI registry within Europe will be set up to prospectively validate, or not, the previous and suggested definitions of CLI.
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Isquemia , Enfermedades Vasculares Periféricas , Amputación Quirúrgica , Europa (Continente) , Extremidades , Humanos , Estudios Prospectivos , Resultado del TratamientoRESUMEN
OBJECTIVES: To assess the efficacy of tocilizumab in patients with Takayasu arteritis (TA). METHODS: We conducted a retrospective multicenter study in 46â¯TA patients treated with tocilizumab. We analyzed factors associated with response to tocilizumab (assessed using NIH score). RESULTS: Forty-six patients with TA were included, with a median age of 43 years [29-54], and 35 (76%) females. We observed a decrease in the median NIH scale (from 3 [2-3] at baseline to 0 [0-1] and 0â¯at 3 and 6 months, respectively; pâ¯<â¯0.0001). The daily prednisone dose also decreased from 15â¯mg [8-19] at baseline to 4â¯mg [5-21] and 5â¯mg [4.5-9] at 3 and 6 months, respectively (pâ¯<â¯0.0001) under tocilizumab. The overall tocilizumab failure free survival was 81% [CI 95%; 0.7-0.95], 72% [CI 95%; 0.55-0.95] and 48% [CI 95%; 0.2-0.1] at 12, 24 and 48 months, respectively. The presence of constitutional symptoms (HR 5.6 [CI 95%; 1.08-29], pâ¯=â¯0.041), and C-reactive protein level (HR 1.16 [CI 95%; 1.01-1.31], Pâ¯=â¯0.003) at the time of tocilizumab initiation were significantly associated with tocilizumab event-free survival. The event-free survival was significantly better under tocilizumab therapy in comparison to DMARDs (pâ¯=â¯0.02). CONCLUSION: This large multicenter study shows that tocilizumab is efficient and may reduce the incidence of relapses in TA.
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Anticuerpos Monoclonales Humanizados/uso terapéutico , Antirreumáticos/uso terapéutico , Inmunosupresores/uso terapéutico , Arteritis de Takayasu/tratamiento farmacológico , Adulto , Proteína C-Reactiva/metabolismo , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Prednisona/uso terapéutico , Estudios Retrospectivos , Análisis de Supervivencia , Arteritis de Takayasu/mortalidad , Resultado del TratamientoRESUMEN
We report the case of a young woman who experienced ischemia of upper limb after osteopathic manipulation. Duplex and computed tomography scan showed wall hematoma of the ostium of subclavian artery. The patient spontaneously recovered so that no surgery was necessary. Dissection of vertebral and carotid arteries has been reported after osteopathic manipulations. We report ischemia of upper limb secondary to dissection of subclavian artery. Arterial dissections associated with manipulation should be recorded in a register in order to assess more carefully the vascular risk that this method carries.
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Hematoma/etiología , Isquemia/etiología , Osteopatía/efectos adversos , Arteria Subclavia/lesiones , Extremidad Superior/irrigación sanguínea , Lesiones del Sistema Vascular/etiología , Adulto , Anticoagulantes/uso terapéutico , Angiografía por Tomografía Computarizada , Femenino , Hematoma/diagnóstico por imagen , Hematoma/tratamiento farmacológico , Hematoma/fisiopatología , Heparina de Bajo-Peso-Molecular/uso terapéutico , Humanos , Iloprost/uso terapéutico , Isquemia/diagnóstico por imagen , Isquemia/tratamiento farmacológico , Isquemia/fisiopatología , Arteria Subclavia/diagnóstico por imagen , Arteria Subclavia/efectos de los fármacos , Arteria Subclavia/fisiopatología , Tinzaparina , Resultado del Tratamiento , Ultrasonografía Doppler en Color , Lesiones del Sistema Vascular/diagnóstico por imagen , Lesiones del Sistema Vascular/tratamiento farmacológico , Lesiones del Sistema Vascular/fisiopatología , Vasodilatadores/uso terapéuticoRESUMEN
Objectives: The aim of this work was to study inter- and intra-observer agreement for the diagnosis of scleroderma pattern by wide-field capillaroscopy. Methods: Images were taken from 50 patients known to have SSc and 50 controls consulting for RP who did not have SSc. These images were rated simultaneously by 11 experienced vascular medicine physicians as scleroderma pattern or not. Two weeks later, 7 of the 11 observers again rated the same images. Results: Inter-observer agreement was almost perfect between the 11 observers (κ 0.86 ± 0.01), and the proportion of concordant observations was 79% (70-87). When each observer was compared with the reference, agreement was also almost perfect: κ coefficient 0.92 ± 0.03 and proportion of concordant observations 79% (70-87). Intra-observer agreement was also almost perfect: median κ coefficient 0.94 (0.78-0.96) and median proportion of concordant observations 97% (89-98). Conclusion: Excellent inter- and intra-observer agreement was obtained in experienced vascular physicians for the diagnosis of capillaroscopic landscape by wide-field nailfold capillary microscopy.
Asunto(s)
Angioscopía Microscópica/estadística & datos numéricos , Enfermedad de Raynaud/fisiopatología , Esclerodermia Sistémica/diagnóstico , Adulto , Estudios de Casos y Controles , Femenino , Humanos , Masculino , Angioscopía Microscópica/métodos , Uñas/irrigación sanguínea , Uñas/diagnóstico por imagen , Variaciones Dependientes del Observador , Enfermedad de Raynaud/etiología , Reproducibilidad de los Resultados , Esclerodermia Sistémica/complicacionesRESUMEN
Objectives: Subgroups of capillaroscopic scleroderma landscape have been correlated with stages of SSc: two groups for Maricq's classification (slow and active), and three for Cutolo's classification (early, active and late). We report inter- and intra-observer agreement for these classifications as a preliminary step in the multicentre prospective SCLEROCAP study, which aims to assess the classification and single capillaroscopic items as prognostic tools for SSc. Methods: SCLEROCAP included 385 patients. Agreement was studied in the first 100 patients, who were independently rated twice by two observers, blind to patients' characteristics; 30 of the patients were rated once by six observers. After consensus meetings, these ratings were held again. Kappa and intraclass correlation coefficients were used to assess agreement. Results: Interobserver agreement on 100 patients was moderate for Maricq and Cutolo classifications [κ 0.47 (0.28, 0.66) and 0.49 (0.33, 0.65), respectively], and became substantial after consensus meetings [0.64 (0.50, 0.77) and 0.69 (0.56, 0.81)]. Intra-observer agreement between two observers was moderate to substantial: κ 0.54 (0.33, 0.75) and 0.70 (0.57, 0.83) for Maricq's classification; 0.57 (0.38, 0.77) and 0.76 (0.65, 0.87) for Cutolo's. Thirty patients were rated once by each of six observers, and agreement was moderate to substantial: κ 0.57 ± 0.10 (Maricq) and 0.61 ± 0.12 (Cutolo). Agreement was substantial for bushy, giant capillaries and microhaemorrhages, moderate for capillary density and low for oedema, disorganization and avascular areas. Conclusion: The moderate reproducibility of Maricq and Cutolo classifications might hamper their prognostic value in SSc patients. Consensus meetings improve reliability, a prerequisite for better prognostic performances. A focus on giant capillaries, haemorrhages and capillary density might be more reliable.
Asunto(s)
Angioscopía Microscópica/estadística & datos numéricos , Esclerodermia Sistémica/clasificación , Anciano , Femenino , Humanos , Masculino , Angioscopía Microscópica/métodos , Persona de Mediana Edad , Variaciones Dependientes del Observador , Valor Predictivo de las Pruebas , Pronóstico , Estudios Prospectivos , Reproducibilidad de los ResultadosRESUMEN
18Fluoro-desoxy-glucose positron-emission tomography (F-FDG-PET) may be useful to diagnose complications of endocarditis in difficult cases. We report the case of a young patient who had endocarditis and a painful leg. FDG PET/computed tomography showed hypermetabolism in the leg, and duplex ultrasound suggested an inflammatory aneurysm at the site of the hypermetabolism. The combination of FDG-PET/computed tomography and duplex ultrasound is useful to diagnose mycotic aneurysm in the setting of bacterial endocarditis.