The aim of this study was to evaluate health-related quality of life (HR-QOL) in patients with primary Sjögren's syndrome (pSS) using both Short-Form 36 (SF-36) and World Health Organization Quality of Life Assessment-BREF (WHOQOL-BREF) questionnaires and to determine the effects of anxiety and depression on HR-QOL using the Hospital Anxiety-Depression Scale (HADS). In this cross-sectional study, 107 female patients with pSS (mean age 54.10 ± 10.2 years), fulfilling US-European Consensus Criteria and 109 female controls (mean age 53.4 ± 10.9 years) were included. Student's t test, Mann-Whitney U test, and analysis of variance (ANOVA) were used for statistical analysis. P values > 0.05 were accepted as significant. All domains of the SF-36, with the exception of "Vitality", and all domains of the WHOQOL-BREF with the exception of "Environment", were significantly lower in pSS patients compared with healthy controls. In pSS patients having anxiety according to HADS, the scores of all domains of WHOQOL-BREF were significantly lower, and in patients having depression according to HADS, three of four domains of WHOQOL-BREF were significantly lower compared with the rest of the group. However, the scores of two domains of the SF-36, namely "Role-Physical" and "Role-Emotional" domains, were significantly higher in pSS patients having depression according to HADS. We confirmed the presence of impaired HR-QOL in pSS. Whereas the presence of anxiety and/or depression generally showed a negative affect on HR-QOL, interestingly, depression seemed to improve the scores of "Role-Physical" and "Role-Emotional" domains of the SF-36. This surprising finding might be related to adaptation to changing health. Social support based upon cultural traditions might also have contributed.
Smoking is well known to contribute to the pathogenesis and severity of some systemic autoimmune rheumatic diseases and especially to the production of certain autoantibodies. Primary Sjögren's syndrome (pSS) is an autoimmune disease, affecting primarily the exocrine glands. It may also cause extraglandular involvement in some cases. In this study, we aimed to determine the frequency of smoking habits in our cohort of pSS patients and to investigate whether the frequencies of autoantibody positivity and extraglandular involvement were significantly different between patients with and without smoking. In this cross-sectional study, 207 patients with pSS (F/M 203/4), fulfilling the United States-European Consensus Criteria, and 602 healthy controls (F/M 534/68) were included. Patients and controls were classified into five groups: never smokers, current smokers, former smokers; ever smokers, and passive smokers. The χ(2) and Kruskal-Wallis tests were used for statistical analysis; a p value of less than 0.05 was accepted as statistically significant. While the frequency of current smokers was significantly lower in the pSS group compared with the healthy controls (11.6 vs 22.3%), the frequencies of former smokers (30.4 vs 11.8%), ever smokers (42.0 vs 34.1%), and passive smokers (47.3 vs 37.5%) were significantly higher in the pSS group compared with the healthy controls. In pSS patients, only antinuclear antibody (ANA) positivity was significantly associated with smoking habits, while there was no significant association with other autoantibodies or with the presence of extraglandular involvement. We found that in pSS patients smoking was significantly associated only with ANA positivity. Unlike the deleterious effects of smoking upon disease severity and anti-cyclic citrullinated protein (CCP) antibody production in rheumatoid arthritis, we could not find any association of smoking with extraglandular involvement and/or anti-Ro/anti-La antibody positivity in pSS. These results are indeed in line with the limited number of previous studies reported in the literature. Further studies with higher numbers of pSS patients are required to confirm the seemingly negative association of smoking with pSS.
Patients with Sjögren's syndrome (SS) manifest symptoms such as dry eyes, dry mouth, and dysphagia. This study aims to evaluate the swallowing functions of the patients with SS. 69 patients with SS (65 females, 4 males) and 40 healthy individuals (33 females, 7 males) were included as study and control groups, respectively. Mean ages were 52.86 and 48.25 years for study and control groups, respectively. Swallowing functions were evaluated by fiberoptic endoscopic evaluation of swallowing (FEES). All the patients underwent FEES and were given 3, 5, and 10 ml water, yogurt, and fish-shaped crackers twice, respectively. The presence of bolus control, residue, penetration, and aspiration were evaluated. Additionally, certain types of foods triggering the dysphagia, any difficulties in bolus control, need to clean the throat, sensation of having a lump in the throat, sensation of choking, and xerostomia were included in the questionnaire. The MD Anderson Dysphagia Inventory and the Beck Depression Inventory were administered to patients. Considering the presence of residue with yogurt and fish cracker, there was a significant difference between groups (P < 0.05). Penetration was present in two patients in the study group; however, the difference was not significant (P > 0.05). Regarding the MD Anderson Dysphagia Inventory, the average scores were 48.18 ± 13.21 and 87.6 ± 10.67 for study and control groups, respectively, and a statistically significant difference was detected (P < 0.05). Regarding the Beck Depression Inventory, the average scores were 11.83 ± 9.37 and 8.03 ± 6.84 for study and control groups, respectively (P < 0.05). SS affected the swallowing functions significantly. The presence of residue with yogurt and cracker was the most obvious finding, whereas penetration/aspiration was not clinically significant. Swallowing dysfunction reduced the quality of life in patients with SS.
Segmental arterial mediolysis (SAM) is a rare, nonarteriosclerotic, noninflammatory vascular disease and mostly affects medium-to-large sized abdominal arteries as well as presents with hemorrhages in the abdominal cavity. We report the case of a patient with SAM of the celiac, right renal, jejunal branch of the superior mesenteric, left gastric, and splenic arteries who was diagnosed by excluding other causes and in whom transcatheter embolization was performed in two different sessions, but he died because of an undefined reason. SAM mimics systemic vasculitis and causes abdominal pain; it should be considered because abdominal hemorrhage or arterial infarction can result in death.
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