RESUMEN
OBJECTIVE: The aim of this study was to evaluate whether there are clinical subgroups that may have different prognoses among FMF patients. METHODS: The cumulative clinical features of a large group of FMF patients [1168 patients, 593 (50.8%) male, mean age 35.3 years (s.d. 12.4)] were studied. To analyse our data and identify groups of FMF patients with similar clinical characteristics, a two-step cluster analysis using log-likelihood distance measures was performed. For clustering the FMF patients, we evaluated the following variables: gender, current age, age at symptom onset, age at diagnosis, presence of major clinical features, variables related with therapy and family history for FMF, renal failure and carriage of M694V. RESULTS: Three distinct groups of FMF patients were identified. Cluster 1 was characterized by a high prevalence of arthritis, pleuritis, erysipelas-like erythema (ELE) and febrile myalgia. The dosage of colchicine and the frequency of amyloidosis were lower in cluster 1. Patients in cluster 2 had an earlier age of disease onset and diagnosis. M694V carriage and amyloidosis prevalence were the highest in cluster 2. This group of patients was using the highest dose of colchicine. Patients in cluster 3 had the lowest prevalence of arthritis, ELE and febrile myalgia. The frequencies of M694V carriage and amyloidosis were lower in cluster 3 than the overall FMF patients. Non-response to colchicine was also slightly lower in cluster 3. CONCLUSION: Patients with FMF can be clustered into distinct patterns of clinical and genetic manifestations and these patterns may have different prognostic significance.
Asunto(s)
Amiloidosis/etiología , Artritis/etiología , Fiebre Mediterránea Familiar/epidemiología , Mialgia/etiología , Sistema de Registros , Adulto , Amiloidosis/epidemiología , Artritis/epidemiología , Análisis por Conglomerados , Fiebre Mediterránea Familiar/complicaciones , Fiebre Mediterránea Familiar/diagnóstico , Femenino , Humanos , Incidencia , Masculino , Mialgia/epidemiología , Prevalencia , Turquía/epidemiologíaRESUMEN
ABSTRACT: Ineffective esophageal motility (IEM), defined as minor esophageal motility disorder, is also the most common esophageal motility disorder. The relationship between gastro-esophageal reflux disease is still controversial. Our aim in this study is to evaluate whether there are differences in terms of demographic, endoscopic, or motility findings between IEM patients with pathological esophageal acid reflux and physiological reflux.Patients diagnosed with IEM according to the Chicago classification v3 with high-resolution manometry (HRM) before acid monitoring constituted the study group of our investigation. The patients were divided into 2 groups as patients with pathological esophageal reflux and patients with physiological reflux according to 24-hour acid monitoring. Demographic data, endoscopic findings, and HRM findings were compared between 2 groups.A total of 62 patients who were diagnosed with IEM according to the Chicago classification v3 were included in the study. Patients in the physiological reflux group were 7âyears younger on average than the pathological reflux group. Esophagitis rates were significantly higher in the pathological reflux group (Pâ=â.033). Lower esophageal sphincter resting pressure, integrated relaxation pressure, and the presence of hernia were found to be similar in the 2 groups (Pâ=â392, Pâ=â182, Pâ=â657, respectively). The rate of severe IEM was also similar between the 2 groups (Pâ=â.143).The fact that the physiological reflux patient group is younger may suggest that the IEM develops in the early period and then reflux accompanies the picture with advancing age.
Asunto(s)
Trastornos de la Motilidad Esofágica/epidemiología , Esofagitis Péptica/epidemiología , Reflujo Gastroesofágico/complicaciones , Adulto , Factores de Edad , Estudios de Casos y Controles , Estudios Transversales , Endoscopía , Trastornos de la Motilidad Esofágica/complicaciones , Trastornos de la Motilidad Esofágica/diagnóstico , Monitorización del pH Esofágico , Esofagitis Péptica/complicaciones , Esofagitis Péptica/diagnóstico , Femenino , Reflujo Gastroesofágico/diagnóstico , Humanos , Masculino , Manometría , Persona de Mediana EdadRESUMEN
BACKGROUND: To examine the activity of the mammalian target of rapamycin (mTOR) pathway and its regulators, transforming growth factor (TGF)-ß1 and phosphatase and tensin homolog (PTEN), in minor salivary gland biopsies of Sjogren's syndrome (SS) and systemic sclerosis (SSc) patients. METHODS: We retrospectively evaluated SS, SSc, and SS-SSc overlap patients admitted to our outpatient rheumatology clinic between January 2007 and December 2015 who underwent a minor salivary gland biopsy. Patient demographics and some clinical features were obtained from hospital records. Immunohistochemistry was used to analyze total mTOR, total PTEN, and TGF-ß1 expression in the biopsied tissues. The biopsy specimens were also examined for the presence and degree of fibrosis. RESULTS: Minor salivary gland biopsies of 58 SS, 14 SSc, and 23 SS-SSc overlap patients were included in the study. There was no significant difference in mTOR expression between these groups (P = 0.622). PTEN protein was expressed in 87.2% of patients with SS, 57.9% with overlap syndrome, and 100% of the SSC patients, and these differences were statistically different (P = 0.023). Although ductal epithelial TGF-ß1 expression was similar between the groups (P = 0.345), acinar cell expression was found to be more frequent in the SSc (72.7%) and overlap patients (85.7%) in comparison with the SS cases (58.2%; P = 0.004). CONCLUSION: mTOR may be one of the common pathways in the pathology of both SS and SSc. Hence, there may be a role for mTOR inhibitors in the treatment of both diseases. Additionally, PTEN and TGF-ß1 expression may be a distinctive feature of SSc.
Asunto(s)
Glándulas Salivales Menores/metabolismo , Esclerodermia Sistémica/metabolismo , Síndrome de Sjögren/metabolismo , Serina-Treonina Quinasas TOR/metabolismo , Adulto , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Glándulas Salivales Menores/patología , Esclerodermia Sistémica/patología , Síndrome de Sjögren/patologíaRESUMEN
It is recommended to investigate the serology of hepatitis B virus (HBV) and vaccinate seronegative patients at the time of diagnosis in inflammatory bowel diseases (IBD). This study aimed to investigate the efficacy of HBV vaccine and factors affecting the response.In this retrospective, observational study, HBV-seronegative IBD patients were administered 3 doses (at months 0, 1, and 6) recombinant 20 âµgâHbsAg. Patients' demographics, IBD attributes, and treatment methods were investigated as the factors with potential impacts on vaccination outcomes.One hundred twenty-five patients with IBD were evaluated. The number of patients with Anti-HBs >10â IU/L was 71 (56.8%), and the number of patients with anti-HBs >100 âIU/L was 50 (40%). Age, disease activity, Crohn disease subtype, and immunosuppressive treatment (IST) were found to have significant effects on immune response (Pâ=â0.011, Pâ<â0.001, Pâ=â0.003, and Pâ<â0.001, respectively). With multivariate analysis, ageâ<â45 years (OR 3.1, 95% CI 1.2-8.3, Pâ=â0.020), vaccination during remission (OR 5.6, 95% CI 2.3-14, Pâ<â0.001), and non-IST (OR 11.1, 95% CI 2.9-43.2, Pâ=â0.001) had favorable effects on the occurrence of adequate vaccine response.The likelihood of achieving adequate immune response with standard HBV vaccination protocol in IBD is low. Selecting vaccination protocols with more potent immunogenicity is a better approach to achieve effective vaccine response in patients with multiple unfavorable factors.