Six medico-psycho-social dimensions of a pedagogical model used to define clusters of patients with Sjögren's syndrome and intentionality to participate in a patient education programme.

OBJECTIVES: Sjögren's syndrome (SS) is an autoimmune disease with an impact on quality of life (QoL). The aim of patient education (PE) is to improve patients' QoL. The main objective of this study was to describe the medico-psycho-social characteristics defining the six spheres of an allosteric educational model in order to characterise clusters of patients with SS and intentionality for patients to participate in a programme of patient education. METHODS: A self-administered questionnaire was proposed to 408 patients with SS followed in the Department of Internal Medicine of the University Hospital of Lille, France with the aim of assessing the six spheres of the allosteric model: intentional, perceptual, affective, cognitive, infra-cognitive and meta-cognitive. Sub objectives were to determine factors that can influence intentionality to participate in a PE programme and to determine, using cluster analysis, similar characteristics of patients with SS. RESULTS: 127 patients (31%) agreed to participate and were included in the study; 96% were women and the median age was 51 years (±14.5). They mostly reported dry syndrome and fatigue, had a good knowledge of SS, and presented anxiety symptoms. They mainly had problem-centred coping strategies, internal locus of control and low self-esteem. SS had an impact on their social interactions. Considering intentionality to participate in a PE programme, the patients were significantly younger, had a shorter duration of the disease, more frequently had disabled status, reported more fatigue, more self-reported symptoms and a poorer QoL. Two clusters of patients could be individualised, with one group including 75 (59%) patients presenting a higher global impact of the disease, including a more severe impairment for the scores of the perceptual, emotional and infra-cognitive spheres, worse physical QoL, and a higher intentionality to participate in a PE programme. CONCLUSIONS: Our study described an SS population in terms of the different spheres of an allosteric model applicable to the practice of PE. A cluster of patients appeared to present more impact of the disease and more intentionality to participate in a programme of PE. There was no difference between the two groups in terms of the cognitive sphere (i.e. knowledge of the disease), thus indicating that motivation to participate in a PE programme is influenced by non-cognitive factors. Considering intentionality to participate in a PE programme, duration disease, age of the patient and QoL should be more considered to propose to patients to participate in a PE programme. Use of the allosteric model appears promising for future research in PE.

Coping strategies, illness perceptions, and relationship dynamics contribute to female sexual function and sexual distress in Sjögren's syndrome.

BACKGROUND: Sexual dysfunction and sexual distress are common complaints for women with the autoimmune rheumatic disease Sjögren's syndrome (SS); however, the role of psychosocial and interpersonal factors has not previously been explored in SS. AIM: This study investigated whether psychosocial variables, such as coping strategies, illness perceptions, and relationship dynamics, contributed to sexual function and sexual distress for women with SS. METHODS: Participants with SS completed an online cross-sectional survey that included prevalidated questionnaires assessing sexual function, sexual distress, disease-related symptom experiences, cognitive coping strategies, illness perceptions, relationship satisfaction, and partners' behavioral responses. Multiple linear regression was used to identify factors significantly associated with sexual function (total Female Sexual Function Index [FSFI] score) and sexual distress (total Female Sexual Distress Scale score) for women with SS. OUTCOMES: Study outcome measures were the FSFI, Female Sexual Distress Scale, EULAR SjÓ§gren's Syndrome Patient Reported Index, numeric rating scale for vaginal dryness (0-10), Profile of Fatigue and Discomfort, Cognitive Emotion Regulation Questionnaire (CERQ), Brief Illness Perceptions Questionnaire (B-IPQ), West Haven-Yale Multidimensional Pain Inventory (WHYMPI), and Maudsley Marital Questionnaire. RESULTS: A total of 98 cisgender women with SS participated in the study (mean age = 48.13 years, SD = 13.26). Vaginal dryness was reported by 92.9% of participants, and clinical levels of sexual dysfunction (total FSFI score <26.55) were observed in 85.2% (n = 69/81) of cases. More vaginal dryness, lower CERQ positive reappraisal, and higher CERQ catastrophizing were significantly associated with poorer self-rated sexual function (R2 = 0.420, F3,72 = 17.394, P < .001). Higher CERQ rumination, lower CERQ perspective, lower WHYMPI distracting responses, and higher B-IPQ identity were significantly associated with higher sexual distress (R2 = 0.631, F5,83 = 28.376, P < .001). CLINICAL IMPLICATIONS: This study suggests that interpersonal and psychosocial factors are important contributors to sexual function and distress in women with SS and that the development of psychosocial interventions for this population is warranted. STRENGTHS AND LIMITATIONS: This study is one of the first to explore the impacts of coping strategies, illness perceptions, and relationship dynamics on sexual function and sexual distress for women with SS. Limitations of our study include its cross-sectional nature and narrow sample demographic, which limit the generalizability of our results to other population groups. CONCLUSION: Women with SS who utilized adaptive coping strategies had better sexual function and lower levels of sexual distress than women who utilized maladaptive coping strategies.

The impact of the COVID-19 pandemic on patients with primary Sjögren syndrome.

The aim of this study was to investigate the perspective of Romanian patients with Sjögren syndrome (SS) on various aspects of the disease during the SARS-CoV-2 outbreak, including both the impact of COVID-19 on the disease itself as well as the effects of vaccination against SARS-CoV-2 in this group of patients. The study is an online questionnaire-based survey. We received responses from 137 SS patients. Regarding the general emotional status, 33 patients (24.0%) and 47 patients (34.3%) declared to have been sadder/depressive and more agitated/anxious during the SARS-CoV2 outbreak, respectively. During the lockdown, 49 (33.7%) patients strictly and 77 patients (56.2%) did their best to respect the home isolation measures. The income was unchanged for most of the patients (94 patients, 68.6%). Regarding access to healthcare providers, 27 patients (18.7%) postponed the consultation for fear of getting SARS-CoV2. In our study group, 31 patients (22.6%) responded that they have had COVID-19. Only one patient was completely asymptomatic, while the most frequently declared symptom was weakness (84.0%). In 17 patients among the respondents (68%) the symptoms lasted for at least 2 weeks; the most frequent long-lasting symptoms were fatigue (40.0%) and weakness (36.0%). Out of all the respondents, 53 patients (41.4%) were vaccinated against SARS-CoV2 with at least one dose. After the first dose, the most prevalent side effect was pain at the site of injection (89.2%), followed by weakness (25.0%) and myalgias (21.4%). This information will be useful for developing special programs dedicated to SARS-CoV2 infection and vaccination in patients with SS and other autoimmune diseases.

Instruments for Outcome Evaluation of Specific Domains in Primary Sjögren's Syndrome.

Primary Sjögren's syndrome (pSS) is a systemic autoimmune disorder characterized by very heterogeneous features. The spectrum of this disorder may vary from benign but disabling symptoms such as dryness, due to lachrymal and salivary involvement, pain and fatigue, to systemic, potentially severe, manifestations that may involve any organ. In recent decades, the arrival of biotechnological therapy has offered new opportunities for the treatment of this-until now-orphan disease. Currently, the possible use of these new drugs in therapeutic trials has made it necessary to have reliable outcome measures to evaluate their efficacy in this disease. A great effort has been made in multicenter, often multinational, studies to develop and validate instruments capable of assessing the different disease-related features. The adoption in therapeutic trials of the newly developed outcome measures aimed at assessing systemic features and patient reported symptoms has often yielded disappointing results. These negative data have been ascribed, on the one hand, to the trial design not being completely appropriate, and, on the other hand, to the fact that a single instrument may be not sufficient to cover the great clinical heterogeneity of the disease features. There is now growing belief that composite end points that include instruments that are able to assess the various aspects of the disease may be more properly and successfully used in future therapeutic trials.

Resilience in women with primary Sjögren's syndrome.

To assess the relationship between resilience and several diseases and individual features in primary SjÓ§gren's Syndrome (SS) patients. Resilience was assessed using the Resilience Scale (RS-14). Disease activity, damage, and reported symptoms were assessed by means of ESSDAI (EULAR Sjögren's syndrome disease activity index), SSDDI (SjÓ§gren's Syndrome Disease Damage Index) and ESSPRI (EULAR SjÓ§gren's Syndrome Patient Reported Index). EuroQol, HADS (Hospital Anxiety and Depression Scale), SF-12 (Short-form 12 health survey), FAS (Fatigue Assessment Scale), FACIT-F (Functional Assessment of Chronic Illness Therapy - Fatigue), and IPAQ (International Physical Activity Questionnaire) questionnaires were submitted to evaluate physical and mental well-being of the recruited patients. Data about the autoimmune profile, systemic manifestations, and current therapy were collected. Educational qualifications and work activities were also considered. Descriptive, correlational, and linear regression analysis were performed. 74 consecutive women with primary SS and 74 sex and age-matched healthy subjects as a control group were recruited. SS patients displayed a moderate value of resilience (median 78.5) with no significant difference compared to controls (p = 0.38). An inverse relationship was found between resilience and mood disorders such as anxiety (p = 0.038) and depression (p < 0.001). Greater resilience was associated with a better perception of the quality of life (p = 0.02) and general health (p < 0.001), as well as with less fatigue (p = 0.008) and a more physically active lifestyle (p = 0.001). No significant relationship was found neither between resilience and age, socio-demographic and disease characteristics, nor with ESSDAI (p = 0.26), ESSPRI (p = 0.83) and SSDDI (p = 0.67). This is the first study assessing resilience in a large group of unselected primary SS patients. Most resilient primary SS patients are less depressed and show a better perception of their health. Greater resilience tends to correlate with less anxiety, physical and mental fatigue, and a more active lifestyle, while no association with disease activity and duration, damage, and socio-demographic features was detected. Considering the well-known role of resilience in helping to better cope with chronic illnesses, its assessment in SS patients should not be overlooked and the possible strategies for its improvement should be better explored and further implemented.

Illness Experiences of Chilean Women With Sjögren's Syndrome: The Patient Perspective.

OBJECTIVE: Sjögren's syndrome (SS) challenges everyday functioning and well-being. The aim of this study was to structure and summarize the life experiences of Chilean women with SS in an integrated model. METHODS: Interviews from a previous study yielded 75 experiences of living with SS. A sample of 30 women with SS sorted these experiences by content and rated their level of agreement with each experience. A hierarchical cluster analysis was used to structure the experiences of the participants with SS in a comprehensive overview. A team-based consensus analysis was used to define the number of clusters. The level of agreement was examined with Wilcoxon's signed rank test. RESULTS: Ten clusters were identified and grouped into 6 main categories: symptoms (clusters: mucosal dryness and related symptoms), social environment, emotion management (clusters: fears and sadness), information (clusters: uncertainty and lack of knowledge), coping strategy (clusters: resilience and self-care), and health staff relationship. The clusters that describe the more common experiences among patients were resilience, self-care, uncertainty, lack of knowledge, health staff relationship, and mucosal dryness. CONCLUSION: This study provided an integrated and structured overview of disease experiences comprising both biomedical and psychosocial aspects as being of vital importance for the health of patients with SS. The overview can be used to get a quick impression of disease experiences that are important for an individual patient, in a therapeutic goal setting, and in the construction and evaluation of medical and nonmedical interventions or education.

Investigation of the relationship between the pelvic floor and sexual dysfunction in women with Sjögren's syndrome.

BACKGROUND AND AIM: Women with Sjögren's syndrome (SS) may have sexual dysfunctions due to vaginal dryness and may also have pelvic floor problems. The aim of this study was to examine the pelvic floor distress of women with SS with a self-reported questionnaire, to compare this group with healthy individuals, and to examine the relationship between pelvic floor problems and sexual dysfunction. METHODS: The study included 94 women with SS, aged 47.26 ± 7.56 years, and 94 age-matched healthy women, aged 48.15 ± 8.73 years. The Pelvic Floor Disease Inventory (PFDI-20), Pelvic Floor Impact Questionnaire (PFIQ-7), and Female Sexual Function Scale (FSFI) were used for assessment. RESULTS: The PFDI-20, PFIQ-7, and FSFI scores of the healthy control group were found to be statistically significantly better than those of the primary SS group (Z = -2.69 to -8.03, P = .00). A moderate-high correlation was found between the total and sub-parameters of PFDI-20 and disease duration, the total and sub-parameters of the PFIQ-7 and the pain sub-parameter and total score of the FSFI (r = -0.66 to 0.78, P = .00-.04). CONCLUSION: According to the study results, as the disease duration increases, the distress increases and this distress has a negative effect on the health-related quality of life of these women. Pelvic floor distress was associated with poor sexual function and pain. From these results, it can be recommended that the methods of pharmacology, education, exercise, relaxation, lifestyle change, and so on for pelvic floor dysfunction and pain in women with SS should be applied together with a multidisciplinary approach.

Chemosensory dysfunction, Oral disorders and Oral health-related quality of life in patients with primary Sjögren's syndrome: comparative cross-sectional study.

BACKGROUND: The aim of this study was to evaluate chemosensory function and oral disorders in patients with primary Sjögren's syndrome (pSS) and to compare these findings with those of age- and gender-matched healthy controls. METHODS: This comparative cross-sectional study included 58 patients with primary Sjögren's syndrome (pSS) and 55 age- and gender-matched healthy controls. Olfactory and gustatory function, burning sensations in the tongue (BST) and halitosis were assessed. Oral health-related quality of life (OHRQoL) was evaluated using the short-form Oral Health Impact Profile (OHIP-14). RESULTS: Patients with pSS had significantly lower self-reported visual analogue scale (VAS) smell score (8.6 ± 2.2 vs. 9.6 ± 0.7, p = 0.016) and VAS taste score (8.5 ± 2.1 vs. 9.5 ± 0.7, p = 0.014) than healthy controls. A greater proportion of patients with pSS had anosmia (3.8% vs. 0.0%) or hyposmia (36.5% vs. 13.2%) and ageusia for basic tastes: sweetness (34.0% vs. 7.5%), sourness (10.6% vs. 0.0), saltiness (10.0% vs. 5.7%) or bitterness (19.1% vs. 1.9%) as evaluated using Sniffin Sticks test and taste stripts, respectively. A higher proportion of pSS patients complained of dysgeusia (52.6% vs. 9.4%, p < 0.0001) and BST (45.6% vs. 0.0%, p < 0.0001), while similar number of patients with pSS and controls reported halitosis (31.6% vs. 28.3%, p = 0.434). The mean OHIP-14 score was significantly higher in patients with pSS (6.8 ± 7.0 vs. 2.3 ± 8.5, p < 0.001) indicating patients' poorer OHRQoL compared with controls. CONCLUSIONS: The majority of patients with pSS had impaired chemosensory function and indicators of oral health in comparison with the age- and gender-matched healthy controls. Further studies of oral hygiene habits and dietary intake of these patients are needed to ensure better management of oral health problems in patients with pSS.

Ocular Manifestations and Burden Related to Sjögren Syndrome: Results of a Patient Survey.

PURPOSE: To compare the burden related to dry eye with systemic symptoms of Sjögren syndrome; to estimate the burden related to ocular treatments; and to compare the impact of dry eye and extraocular manifestations of Sjögren syndrome on various aspects of patient life. DESIGN: Cross-sectional study. METHODS: We conducted a postal survey of adult patients with a history of physician-diagnosed Sjögren syndrome. RESULTS: The survey was completed by 2,961 patients (mean age 65.1 years, standard deviation 11.7 years), most of whom were women (96%) and white (94%). Forty-one patients younger than 18 years of age were excluded. More than half (53%) experienced severe dry eye (ie, dry eye daily/almost daily with major impact on their life). Corresponding proportions for dry mouth and fatigue were 48% and 45%, respectively. Almost all patients (97%) had used nonprescription eye drops/artificial tears/ointments. Compared with patients who did not experience dry eye, those who experienced significant dry eye (ie, daily/almost daily dry eye) more often agreed that living with Sjögren syndrome made every day a challenge (adjusted odds ratio [OR] 3.81, 95% confidence interval [CI] 2.49 to 5.86) and added a significant emotional burden (adjusted OR 2.22, 95% CI 1.49 to 3.31). Adjusted ORs for the impact of dry eye were generally lower than those for fatigue, but were similar to dry mouth and considerably higher than use of systemic treatments for serious manifestations of the disease and diagnosis of lymphoma. CONCLUSIONS: Sjögren-related dry eye is more burdensome than systemic manifestations of the disease. While fatigue has the greatest impact on patient life, the impact of dry eye is comparable to that of other systemic manifestations.

Peripheral neuropathy and health-related quality of life in patients with primary Sjögren's syndrome: a preliminary report.

Sjögren's syndrome (SS) is a chronic autoimmune disease with a wide spectrum of possible organ involvement. Peripheral (PNS) and central nervous system (CNS)-related symptoms may occur in the course of the disease. The aim of this study was to compare the health-related quality of life (HR-QOL) in SS patients with and without peripheral neuropathy. The study involved 50 patients with primary Sjögren's syndrome (pSS). All patients underwent neurological clinical examination followed by nerve conduction studies (NCS) and rheumatological examination. Thirty-six-item Short Form Health Survey (SF-36) was used for evaluating HR-QOL. To assess pSS activity, the EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI) and EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI) were used. For the assessment of clinical disability due to peripheral neuropathy, the Overall Disability Sum Score scale (ODSS) was used. Additional evaluation of pain was performed with the use of the Visual Analogue Scale (VAS) and a semistructured interview. Twenty-three (46%) patients were diagnosed with peripheral neuropathy. The most common PNS manifestation was sensorimotor neuropathy (47%). Neurological symptoms preceded the diagnosis of pSS in eight patients. The following domains of the SF-36 form were significantly lower scored by patients with peripheral nervous system involvement: role-physical [0 (0-100) vs. 75 (0-100)], role-emotional [67 (0-100) vs. 100 (0-100)], vitality [40 (10-70) vs. 50 (20-75)], bodily pain [45 (10-75) vs. 55 (0-100)], and general health [20 (5-50) vs. 30 (0-50)] (p ≤ 0.05). Our study showed that peripheral neuropathy was a common organ-specific complication in SS patients. In pSS patients, coexisting neurological involvement with symptoms such as pain and physical disability may be responsible for diminished HR-QOL.

Adolescent Sjogren's syndrome presenting as psychosis: a case series.

BACKGROUND: Neurological involvement has been reported in up to 80% of adults with Primary Sjogren's syndrome (pSS) with psychiatric abnormalities including anxiety, depression, and cognitive dysfunction being common. Psychosis due to pSS has been reported in adult patients but has never been previously reported in the adolescent/pediatric literature. Here we describe for the first time four cases of adolescent Sjogren's syndrome that presented with psychotic symptoms. Rituximab treatment was followed by improvement of psychiatric symptoms in all patients. CASE PRESENTATION: 1: 16 year old female without significant past medical history presented to the emergency department with 4 days of abnormal behavior, tremors, insomnia, polyphagia, polyuria, and suicidal ideation. 2: 16 year old female with a 4 year history of severe anxiety, OCD, and tic disorder treated with fluoxetine with partial benefit presented with an abrupt and severe worsening of anxiety, OCD and new auditory hallucinations. 3: 19 year old female without significant past medical history presented with a 3 day history of progressively altered behavior, incoherent speech, insomnia, headache, and tangential thoughts. 4: 17 year old female without significant past medical history presented with new onset suicidal ideation, paranoia, confusion, and emotional lability. CONCLUSION: Psychosis is more common in autoimmune disease than previously known. To our knowledge, the four teenage women described above are the first reported patients with adolescent pSS manifesting as psychosis. pSS should be considered in the differential diagnosis of young patients with new psychiatric disorders, even in the absence of sicca symptoms. Psychiatric symptoms improved with rituximab infusions in all 4 of our patients, which suggests rituximab may be an effective treatment option that should be considered early after the diagnosis of pSS-associated psychiatric disturbance.

Associated factors with interstitial lung disease and health-related quality of life in Chinese patients with primary Sjögren's syndrome.

OBJECTIVE: The main purpose of this study was to investigate the current situation of primary Sjögren's syndrome (pSS) patients with interstitial lung disease (ILD) in China. The relationships between social demography, disease activity, psychological status, clinical variables, and ILD were analyzed. Besides that, the quality of life (QoL) in pSS with ILD was also analyzed. METHODS: In the cross-sectional study, 101 pSS patients participated in this study. Under the guidance of the researchers, the European League Against Rheumatism Sjögren's Syndrome Disease Activity Index (ESSDAI), EULAR Sjögren's Syndrome Patient-Reported Index (ESSPRI), the Short-Form 36 health survey (SF-36), Hospital Anxiety and Depression Scale (HADS), Pittsburgh sleep quality index (PSQI), Summated Xerostomia Inventory (SXI), and other related questionnaires were completed. Independent sample t tests, Mann-Whitney U test, chi-square test, and correlational analysis were used. RESULTS: The data showed that 28 (30.1%) pSS people with ILD. The occurrence and development of ILD were associated with disease duration, fatigue, alanine aminotransferase (ALT), neutrophils, albumin, and use of hormone. The survey also found that the QoL of pSS-ILD was significantly lower than non-ILD, especially the score in the dimension of role physical function. CONCLUSIONS: ILD was very common in pSS. ILD had a significant negative impact on the QoL of patients. Therefore, it is of great significance to strengthen the early intervention and drug treatment of pSS patients to prevent ILD and improve their QoL.Key Points• This cross-sectional study finds that the incidence of ILD in patients with pSS is 30.1%.• The occurrence and development of ILD is associated with disease duration, fatigue, alanine aminotransferase (ALT), neutrophils, albumin, and use of hormone.•The study also finds that the QoL of pSS-ILD is significantly lower than in patients with non-ILD, especially the score in the dimension of role physical function.

Psychological status and disease activity were major predictors of body image disturbances in patients with Sjögren's syndrome.

Sjögren's syndrome (SS) often leads to disease-related body defects and functional impairments, which may result in the body image disturbances (BID) of patients. The aim of this study was to investigate the severity and predictors of BID among SS patients. Two hundred and thirty-one SS patients [mean (IQR) age: 51 (42-58); females: 94.4%] and 224 age and sex-matched healthy controls were included in this cross-sectional study. Questionnaires were applied: body image disturbance questionnaire (BIDQ), the hospital anxiety and depression scale (HADS), fatigue severity scale (FSS), the 10-cm pain Visual Analog Scale (VAS), the oral health impact profile-14 (OHIP-14), the ocular surface disease index (OSDI), the social support rate scale (SSRS). Independent sample t-test, Mann-Whitney U-test, Chi-square test, spearman rank correlation, and stepwise linear regression were performed by SPSS version 20.0 to analyze these data. In 231 SS patients, the mean of the overall BIDQ score was 1.80 ± 1.21, and SS patients had significantly higher scores in each domain of BIDQ compared with healthy controls (p < 0.05). The stepwise multiple linear regression analysis revealed that high BIDQ score was predicted by severe anxiety (ß = 0.081; p < 0.001), high disease activity (ß = 0.038; p < 0.001) and poor oral health (ß = 0.017; p = 0.007) in SS patients. Patients with SS suffer from severe BID and it is necessary for rheumatologists to pay more attention to SS patients' body image disturbance, especially those with high disease activity, severe anxiety, and poor oral health to improve patients' quality of life.

Prevalence, correlates, and impact of sleep disturbance in Chinese patients with primary Sjögren's syndrome.

AIM: Sleep disturbances are common in primary Sjögren's syndrome (pSS) patients and may lead to disease aggravation and decreased health-related quality of life (HRQoL). There are currently no known reported studies related to the prevalence, correlates, and impact of sleep disturbance in pSS patients from China. Therefore, this study aims to assess the sleep quality in Chinese pSS patients and evaluate its relationship with the disease activity, quality of life and mood disorders. METHODS: A self-report survey was administered to 221 pSS patients and 198 healthy individuals using the Pittsburgh Sleep Quality Index (PSQI) for sleep quality. Disease activity and damage were evaluated with the European League Against Rheumatism Sjögren's syndrome disease activity index (ESSDAI). Independent samples t tests, Chi-square analysis, logistic regression were used to analyze these data. RESULTS: Our results found that the prevalence of poor sleep (PSQI ≥ 6) was 57.5% and the mean global score of PSQI was 6.57 (SD 3.19) in patients, which were significantly higher than the controls (32.3% and 4.93 [SD 2.86], respectively). When trying to fall asleep, patients with pSS had some sleep disturbances, reduced sleep efficiency, increased number of awakenings than controls. There were significant correlations among dryness, ocular surface disease, HRQoL, pain, disease activity, anxiety/depression and sleep quality in pSS patients. Meanwhile, logistic regression models identified depression and Short Form-36 mental composite score as predictors of poor sleep quality. CONCLUSIONS: Sleep disturbances are commonly reported in pSS patients and sleep quality is lower in pSS patients than in healthy controls. The data suggested the need for holistic assessment and management of pSS patients.

Increased risks of psychiatric disorders in patients with primary Sjögren's syndrome-a secondary cohort analysis of nationwide, population-based health claim data.

INTRODUCTION/OBJECTIVES: Primary Sjögren's syndrome (pSS) is a chronic systemic autoimmune disease. The aim of this study was to investigate the risk of five common psychiatrist-diagnosed disorders in patients with pSS. METHOD: Using Taiwan's National Health Insurance Research Database, 688 patients with newly diagnosed pSS between 2000 and 2012 were identified. Two comparison cohorts were assembled, namely, 3440 patients without pSS and 1302 newly diagnosed patients with rheumatoid arthritis. The incidences of depressive disorder, anxiety disorder, bipolar disorder, sleep disorder, and schizophrenia between the pSS cohort and the comparison cohorts were compared using Poisson regression models. RESULTS: Patients with pSS exhibited a significantly higher risk of developing depressive disorder (adjusted incidence rate ratio [aIRR] = 2.11, p < 0.001), anxiety disorder (aIRR = 2.20, p < 0.001), and sleep disorder (aIRR = 1.76, p = 0.012) when compared with the non-pSS cohort. The risks of developing depressive, anxiety, and sleep disorders were also significantly increased when compared to the rheumatoid arthritis comparison cohort. When the analyses were stratified by sex, depressive disorder (aIRR = 2.10, p < 0.001), anxiety disorder (aIRR = 2.02, p = 0.001), and sleep disorder (aIRR = 1.74, p = 0.022) were found to be significantly increased in female patients with pSS. However, only anxiety disorder (aIRR = 4.88, p = 0.044) was also significantly increased in male patients with pSS. The peak age group of developing depressive disorder was 65-80 years old (aIRR = 3.46, p < 0.001). CONCLUSIONS: In this retrospective cohort study based on population-based claim data, significantly increased incidences of depressive disorder, anxiety disorder, and sleep disorder were observed in patients with pSS. Key Points • Patients, particularly women, with primary Sjögren's syndrome exhibited a significantly higher risk of developing depressive disorder, anxiety disorder, and sleep disorder. • The peak age group of developing depressive disorder was 65-80 years old.

Pain and depression are associated with both physical and mental fatigue independently of comorbidities and medications in primary Sjögren's syndrome.

Objectives: To report on fatigue in patients from the United Kingdom primary Sjögren's syndrome (pSS) registry identifying factors associated with fatigue and robust to assignable causes such as comorbidities and medications associated with drowsiness. Methods: From our cohort (n = 608), we identified those with comorbidities associated with fatigue, and those taking medications associated with drowsiness. We constructed dummy variables, permitting the contribution of these potentially assignable causes of fatigue to be assessed. Using multiple regression analysis, we modelled the relationship between Profile of Fatigue and Discomfort physical and mental fatigue scores and potentially related variables. Results: Pain, depression and daytime sleepiness scores were closely associated with both physical and mental fatigue (all p ≤ 0.0001). In addition, dryness was strongly associated with physical fatigue (p ≤ 0.0001). These effects were observed even after adjustment for comorbidities associated with fatigue or medications associated with drowsiness. Conclusions: These findings support further research and clinical interventions targeting pain, dryness, depression and sleep to improve fatigue in patients with pSS.This finding is robust to both the effect of other comorbidities associated with fatigue and medications associated with drowsiness.

P300 values in patients diagnosed with primary Sjogren syndrome.

PURPOSE: Primary Sjogren syndrome (PSS) is an autoimmune disease characterized by symptoms of a dry mouth and eyes, in which other organs and systems are widely involved. Central nervous system (CNS) involvement in PSS is reported in a wide range between 2.5-60%. The reason is that the clinical picture can remain asymptomatic despite the presence of CNS involvement in the disease process. In this study, our aim was to evaluate subclinical cognitive impairment in patients with PSS by investigating P300 potential parameters. METHOD: Forty-three female patients with PSS (mean age: 52.6 ± 11.4 years) and 35 healthy female controls (mean age: 54.5 ± 8.09 years) were included in the study. Mini-Mental State evaluations (MMSE) and brain MRI were performed in the patient and control groups. An event-related evoked potentials test (P300) was applied to those with normal MMSE. RESULTS: The P300 latencies of patients with PSS were significantly longer compared with the control group (p = .019). In patients with PSS, there was no difference in P300 parameters between ANA, Anti-SSA, Anti-SSB-positive and negative patients, and patients with or without sedimentation and CRP elevation. In addition, brain MRI revealed no statistically significant difference between patients with PSS with and without ischemic gliotic lesions (p=.48). CONCLUSION: In our study, P300 latency was also found to be significantly longer in patients who had no white matter change. We believe that prolonged P300 potential latencies without associated white matter lesions in brain imaging may be associated with subclinical CNS involvement.

Health-related quality of life in systemic sclerosis compared with other rheumatic diseases: a cross-sectional study.

BACKGROUND: Systemic sclerosis (SSc) is a rare autoimmune disease characterized by fibrosis of the skin and the involvement of multiple internal organs. Previous studies reported poorer health-related quality of life (HRQoL) in patients with SSc compared with the general population. However, very little is known about how HRQoL in SSc patients compares with that in patients with other systemic autoimmune diseases, such as rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), and Sjogren's syndrome (SjS). Thus, the main aim of this study was to compare HRQoL in SSc patients, patients with other rheumatic diseases, and the general population. METHODS: In this cross-sectional study, patients from the rheumatology clinics of Seoul National University Hospital with SSc, RA, SLE, and SjS were enrolled via a random sampling technique. HRQoL was captured by the Short Form (36) health survey (SF-36), the Short Form Six-Dimensional health index (SF-6D), and the EuroQol Five-Dimensional descriptive system (EQ-5D). Demographic characteristics and standardized disease activity for each disease were also obtained. Previously reported data from 600 healthy Koreans were used for the healthy controls. An ANCOVA test was used to compare the SF-36, SF-6D, and EQ-5D values between study subjects with adjustments for age, sex, disease duration, comorbidities, and disease activity status. RESULTS: One hundred twenty patients were included in each of the SSc, RA, SLE, and SjS cohorts. Patients with rheumatic diseases had significantly lower SF-36, SF-6D, and EQ-5D scores than healthy controls (all P < 0.001). After statistical adjustments, SSc patients reported significantly lower mental component summary (MCS) scores than patients with RA (P < 0.001) or SLE (P = 0.001). Specifically, the mental health and general health domains were significantly lower in SSc patients than reported in RA or SLE patients (P < 0.001 and P = 0.001, respectively, in both domains). In SSc patients, higher modified Rodnan skin scores (mRSS) correlated with lower MCS scores. CONCLUSIONS: SSc patients report poorer HRQoL than patients with RA or SLE. The extent of skin involvement is associated with poorer HRQoL in SSc patients. TRIAL REGISTRATION: NCT03257878 . Registered 22 August 2017.