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1.
Clin Exp Rheumatol ; 41(12): 2343-2356, 2023 Dec.
Article in English | MEDLINE | ID: mdl-38149515

ABSTRACT

Primary Sjögren's syndrome (pSS) is a systemic autoimmune disorder characterised by the T-cell-mediated hyperactivation of B-cells and cytokine production. The condition may evolve from an asymptomatic, indolent course, with glandular involvement, to extra-glandular systemic manifestations up to lymphoma development. On tissue level, the typical feature is the lymphocytic infiltration of the salivary gland by B-, T- and antigen presenting cells, as mirrored by the diagnostic cornerstone role of minor salivary gland (MSG) biopsy. Recently, increasing research focused on the investigation of mechanisms underlying the complex pathogenesis of the disease and highlighted the multi-factorial nature of SS consisting of concomitant involvement of environmental, genetic, neuroendocrine and immune factors. In particular, many aspects have been investigated regarding genetic and epigenetics, the role of specific B- and T-cell phenotypes and the investigation of disease-specific biomarkers as predictors of disease development, activity, and lymphomagenesis. Surely, a deeper understanding of these multiple mechanisms may facilitate earlier diagnosis, enable subphenotyping of patients and open novel therapeutic possibilities to address the unmet needs of the disease in the upcoming years.In this review, following the others of this series, we will summarise the most recent literature on pSS pathogenesis and clinical features focusing in particular on new insights into pSS molecular stratification and therapeutic advances in the era of precision medicine.


Subject(s)
Lymphoma , Sjogren's Syndrome , Humans , Sjogren's Syndrome/diagnosis , Sjogren's Syndrome/therapy , Salivary Glands , Salivary Glands, Minor/pathology , B-Lymphocytes
2.
Clin Exp Rheumatol ; 41(12): 2547-2554, 2023 12.
Article in English | MEDLINE | ID: mdl-38149517

ABSTRACT

Sjögren's syndrome (SS) is a complex and heterogeneous disease that typically affects middle-aged women. However, while it is rare, the disease may occur in male patients and in females during their childhood/adolescence or in the elderly. Contrasting data have been reported on these three subgroups clinical features and long-term outcomes. Notably, recent studies have pinpointed the severity of the disease in male patients and in both the early and the late-onset subgroups.The aim of this review is, therefore, to summarise the available evidence from the recent literature on these phenotypes. The focus will be on the clinical and laboratory features, and on the lymphoma risk observed in the three subgroups distinct phenotypes: of male patients as well as young-onset SS and elderly-onset SS. Ultimately, an accurate phenotypic stratification may represent the first step towards individualised medical approaches.


Subject(s)
Lymphoma , Sjogren's Syndrome , Aged , Middle Aged , Adolescent , Humans , Male , Female , Child , Sjogren's Syndrome/diagnosis , Sjogren's Syndrome/epidemiology , Sjogren's Syndrome/therapy , Age of Onset , Phenotype
3.
Rev Esp Enferm Dig ; 115(4): 211-212, 2023 04.
Article in English | MEDLINE | ID: mdl-36779461

ABSTRACT

Aortoesophageal fistula is a very rare cause of upper gastrointestinal bleeding, however its interest lies in the high mortality rate associated with it. Due to this, early diagnosis and treatment of this entity is essential to increase survival. The typical symptoms known as the Chiari´s triad are only present in 45% of reported cases. We present the case of a patient with upper gastrointestinal bleeding due to an aortoesophageal fistula as well as the importance of endoscopic use for its differential diagnosis.


Subject(s)
Aortic Diseases , Esophageal Fistula , Esophageal Perforation , Vascular Fistula , Humans , Esophageal Perforation/diagnostic imaging , Esophageal Perforation/etiology , Vascular Fistula/complications , Vascular Fistula/diagnostic imaging , Esophageal Fistula/etiology , Esophageal Fistula/complications , Gastrointestinal Hemorrhage/complications , Aortic Diseases/etiology , Aortic Diseases/complications
5.
Clin Exp Rheumatol ; 40(12): 2211-2224, 2022 Dec.
Article in English | MEDLINE | ID: mdl-36541236

ABSTRACT

Primary Sjögren's syndrome (pSS) is a complex disabling systemic autoimmune disorder. The hallmark of pSS is the T-cell-mediated hyperactivation of B-cells, evolving from asymptomatic conditions to systemic complications and lymphoma development. On tissue level, the typical feature is the lymphocytic infiltration of the salivary gland by B-, T- and antigen presenting cells, as mirrored by the diagnostic cornerstone role of minor salivary gland (MSG) biopsy. B-cells show multiple possible roles in disease pathogenesis, from autoantibody production, to antigen presentation, and cytokine production. B-cells hyperactivation is supported by genetic risk factors, T-cell dependent and independent mechanisms, and the presence of different pathogenic B-cell subsets must be reminded.Many aspects have been investigated in the last year regarding genetic and epigenetics, B- and T-cell role in pSS pathogenesis, their interaction with salivary gland epithelial cells (SGECs) and in their direct or indirect use as biomarkers and predictors of disease development, activity, and lymphomagenesis.In this review, following the others of this series, we will summarise the most recent literature on pSS pathogenesis and clinical features focusing in particular on new insights into pSS molecular stratification and therapeutic advances in the era of precision medicine.


Subject(s)
Sjogren's Syndrome , Humans , Sjogren's Syndrome/diagnosis , Sjogren's Syndrome/genetics , Sjogren's Syndrome/therapy , Salivary Glands , Salivary Glands, Minor , B-Lymphocytes , Biomarkers
6.
Cir Esp ; 95(3): 135-142, 2017 Mar.
Article in English, Spanish | MEDLINE | ID: mdl-28325497

ABSTRACT

INTRODUCTION: Sleeve gastrectomy (SG) has become a technique in its own right although a selective or global indication remains controversial. The weight loss data at 5 years are heterogeneous. The aim of the study is to identify possible prognostic factors of insufficient weight loss after SG. METHODS: A SG retrospective multicenter study of more than one year follow-up was performed. Failure is considered if EWL>50%. Univariate and multivariate study of Cox regression were performed to identify prognostic factors of failure of weight loss at 1, 2 and 3 years of follow up. RESULTS: A total of 1,565 patients treated in 29 hospitals are included. PSP per year: 70.58±24.7; 3 years 69.39±29.2; 5 years 68.46±23.1. Patients with EWL<50 (considered failure): 17.1% in the first year, 20.1% at 3 years, 20.8% at 5 years. Variables with influence on the weight loss failure in univariate analysis were: BMI>50kg/m2, age>50years, DM2, hypertension, OSA, heart disease, multiple comorbidities, distance to pylorus> 4cm, bougie>40F, treatment with antiplatelet agents. The reinforcement of the suture improved results. In multivariate study DM2 and BMI are independent factors of failure. CONCLUSION: The SG associates a satisfactory weight loss in 79% of patients in the first 5 years; however, some variables such as BMI>50, age>50, the presence of several comorbidities, more than 5cm section of the pylorus or bougie>40F can increase the risk of weight loss failure.


Subject(s)
Gastroplasty , Obesity, Morbid/surgery , Weight Loss , Adolescent , Adult , Aged , Cohort Studies , Female , Humans , Male , Middle Aged , Portugal , Prognosis , Retrospective Studies , Spain , Treatment Outcome , Young Adult
7.
Cir. Esp. (Ed. impr.) ; 95(3): 135-42, mar. 2017. graf, tab
Article in Spanish | IBECS | ID: ibc-162241

ABSTRACT

INTRODUCCIÓN: La gastrectomía vertical (GV) se ha convertido en una técnica con entidad propia cuya indicación selectiva o global sigue siendo objeto de controversia. Los resultados ponderales a 5 años son heterogéneos. El objetivo del estudio es identificar posibles factores pronósticos de pérdida de peso insuficiente tras GV. MÉTODOS: Estudio multicéntrico retrospectivo de GV con seguimiento mayor de un año. Se considera fracaso si el PSP < 50%. Se realiza estudio univariado y multivariado de regresión de Cox para determinar los factores que influyen en el fracaso ponderal a 1, 2 y 3 años de seguimiento. RESULTADOS: Se incluye a 1.565 pacientes intervenidos en 29 hospitales. PSP al año: 70,58 ± 24,8; a los 3 años 69,39 ± 29,2; a los 5 años 68,46 ± 23,1. Pacientes con PSP <50 (considerado fracaso ponderal): 17,1% en el primer año, 20,1% a 3 años, 20,8% a 5 años. Las variables que mostraron relación con el fracaso ponderal en el estudio univariado fueron: IMC > 50 kg/m2, edad > 50 años, DM2, HTA, SAOS, cardiopatía, varias comorbilidades asociadas, distancia a píloro > 5 cm, bujía >40 F, tratamiento con antiagregantes. La sobresutura mejora los resultados. Las variables que mostraron ser factores predictivos de fracaso en el seguimiento fueron la DM2 y el IMC. CONCLUSIÓN: La GV asocia una pérdida de peso satisfactoria en el 79% de los pacientes en los primeros 5 años; sin embargo, algunas variables como el IMC > 50, la DM2, la edad > 50, la presencia de varias comorbilidades, la sección a más de 4cm del píloro o la bujía > 40 F pueden aumentar el riesgo de fracaso ponderal


INTRODUCTION: Sleeve gastrectomy (SG) has become a technique in its own right although a selective or global indication remains controversial. The weight loss data at 5 years are heterogeneous. The aim of the study is to identify possible prognostic factors of insufficient weight loss after SG. METHODS: A SG retrospective multicenter study of more than one year follow-up was performed. Failure is considered if EWL > 50%. Univariate and multivariate study of Cox regression were performed to identify prognostic factors of failure of weight loss at 1, 2 and 3 years of follow up. RESULTS: A total of 1,565 patients treated in 29 hospitals are included. PSP per year: 70.58 ± 24.7; 3 years 69.39 ± 29.2; 5 years 68.46 ± 23.1. Patients with EWL< 50 (considered failure): 17.1% in the first year, 20.1% at 3 years, 20.8% at 5 years. Variables with influence on the weight loss failure in univariate analysis were: BMI > 50 kg/m2, age > 50 years, DM2, hypertension, OSA, heart disease, multiple comorbidities, distance to pylorus> 4cm, bougie > 40F, treatment with antiplatelet agents. The reinforcement of the suture improved results. In multivariate study DM2 and BMI are independent factors of failure. CONCLUSION: The SG associates a satisfactory weight loss in 79% of patients in the first 5 years; however, some variables such as BMI > 50, age > 50, the presence of several comorbidities, more than 5 cm section of the pylorus or bougie > 40F can increase the risk of weight loss failure


Subject(s)
Humans , Obesity/surgery , Gastrectomy/methods , Weight Loss , Time/statistics & numerical data , Prognosis , Retrospective Studies , Body Weights and Measures/statistics & numerical data
9.
Obes Surg ; 26(12): 2829-2836, 2016 12.
Article in English | MEDLINE | ID: mdl-27193106

ABSTRACT

BACKGROUND: Complications in sleeve gastrectomy (SG) can cast a shadow over the technique's good results and compromise its safety. The aim of this study is to identify risk factors for complications, and especially those that can potentially be modified to improve safety. METHODS: A retrospective multicenter cohort study was carried out, involving the participation of 29 hospitals. Data was collected on demographic variables, associated comorbidities, technical modifications, the surgeon's experience, and postoperative morbimortality. A multivariate logistic regression analysis was carried out on risk factors (RFs) for the complications of leak/fistula, hemoperitoneum, pneumonia, pulmonary embolism, and death. RESULTS: The following data were collected for 2882 patients: age, 43.85 ± 11.6. 32.9 % male; BMI 47.22 ± 8.79; 46.2 % hypertensive; 29.2 % diabetes2; 18.2 % smokers; bougie calibre ≥40 F 11.1 %; complications 11.7 % (2.8 % leaks, 2.7 % hemoperitoneum, 1.1 % pneumonia, 0.2 % pulmonary embolism); and death 0.6 %. RFs for complications were as follows: surgeon's experience < 20 patients, OR 1.72 (1.32-2.25); experience > 100 patients, OR 0.78 (0.69-0.87); DM2, OR1.48(1.12-1.95); probe > 40 F, OR 0.613 (0.429-0.876). Leak RFs were the following: smoking, OR1.93 (1.1-3.41); surgeon's experience < 20 patients, OR 2.4 (1.46-4.16); experience of 20-50 patients, OR 2.5 (1.3-4.86); experience >100 patients, OR 0.265 (0.11-0.63); distance to pylorus > 4 cm, OR 0.510 (0.29-0.91). RFs for death were as follows: smoking, OR 8.64 (2.63-28.34); DM2, OR 3.25 (1.1-9.99); distance to pylorus < 5 cm, OR 6.62 (1.63-27.02). CONCLUSIONS: The safety of SG may be compromised by nonmodifiable factors such as age >65, patient comorbidities (DM2, hypertension), and prior treatment with anticoagulants, as well as by modifiable factors such as smoking, bougie size <40 F, distance to the pylorus <4 cm, and the surgeon's experience (<50-100 cases).


Subject(s)
Gastrectomy/mortality , Gastrectomy/statistics & numerical data , Laparoscopy , Learning Curve , Obesity, Morbid/epidemiology , Obesity, Morbid/surgery , Postoperative Complications/epidemiology , Adolescent , Adult , Aged , Female , Gastrectomy/adverse effects , Gastrectomy/education , Humans , Laparoscopy/adverse effects , Laparoscopy/education , Laparoscopy/mortality , Laparoscopy/statistics & numerical data , Male , Middle Aged , Morbidity , Obesity, Morbid/mortality , Portugal/epidemiology , Postoperative Complications/mortality , Prognosis , Retrospective Studies , Risk Factors , Spain/epidemiology , Survival Analysis , Treatment Outcome , Young Adult
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