Development and content validity assessment of the Dry Eye Disease Questionnaire in patients with dry eye disease, meibomian gland dysfunction, and Sjögren's syndrome dry eye disease.

BACKGROUND: Dry eye disease (DED), Meibomian gland dysfunction (MGD), and Sjögren's syndrome dry eye disease (SS-DED) are eye dryness conditions that show significant overlap in various symptoms of ocular discomfort. The aim of this study was to qualitatively explore the patient experience and evaluate content validity of the newly developed Dry Eye Disease Questionnaire (DED-Q). METHODS: Semi-structured interviews were conducted with 61 US adults who reported experiencing ocular symptoms due to their physician-confirmed primary diagnosis of DED (n = 21), MGD (n = 20), or SS-DED (n = 20). The open-ended concept-elicitation phase was followed by cognitive debriefing (CD) of the DED-Q to evaluate participants' understanding and relevance of the instructions, items, response options, and recall periods. Interviews were also conducted with eight specialist healthcare professionals to assess clinical relevance of the concepts included. Verbatim interview transcripts were analyzed using thematic analysis in ATLAS.ti v8 software. RESULTS: A total of 29 symptoms and 14 impacts on quality of life were reported across participant interviews. Primary ocular symptoms reported included eye dryness (n = 61/61; 100%), eye irritation (n = 55/61; 90%), eye itch (n = 54/61; 89%), burning sensation (n = 52/61; 85%), and foreign body sensation (n = 51/61; 84%). The most impacted aspects of daily life were using digital screens (n = 46/61; 75%), driving (n = 45/61; 74%), working (n = 39/61; 64%), and reading (n = 37/61; 61%). CD findings showed most participants had good understanding of DED-Q items and confirmed most concepts were relevant to the lived experience of their condition. Aside from few minor changes to the items and examples to facilitate more accurate interpretation, the proposed instruction wording was modified for various symptom and impact modules to encourage participants to focus only on dry eye vision problems. CONCLUSIONS: This research identified multiple prevalent symptoms and impacts of DED, MGD, and SS-DED, most of which were similar across the conditions. The DED-Q was confirmed to be a content-valid PRO measure suitable for use in clinical studies to assess the patient experience of DED, MGD, and SS-DED. Future work will focus on evaluating the psychometric properties of the DED-Q for use as an efficacy endpoint in clinical trials.

Dry Eye Disease (DED), Meibomian Gland Dysfunction (MGD), and Sjögren's Syndrome Dry Eye Disease (SS-DED) are conditions related to dryness of the eye. There is no suitable patient-reported outcome (PRO) measure available for use across the three conditions. Interviews were conducted with 61 participants with any of the three conditions to understand the symptoms they experience and their impact on daily life. The findings were used to refine the content of a new PRO measure, the Dry Eye Disease Questionnaire (DED-Q). To evaluate the suitability of the DED-Q, participants were also asked to complete each item of the DED-Q during the interview and share their understanding of the item wording, response options and the recall period. Interviews were also conducted with eight healthcare professionals (HCPs). The main symptoms reported by the patients across three conditions were eye dryness, eye irritation, eye itch, burning sensation, and foreign body sensation. The symptoms caused difficulties in performing activities such as using digital screens, driving, working, and reading. Almost all participants demonstrated good understanding of the items on the DED-Q and found them to be relevant to their experience of their condition. The HCPs also confirmed that the concepts covered in the DED-Q were relevant to assess the participant experience across these conditions. Next steps will involve a more detailed analysis of the measurement properties of the DED-Q to confirm its usefulness in supporting primary or secondary endpoints in clinical trials.

Assessment of depression, stress, anxiety and oral health-related quality of life in individuals with sjögren's syndrome: case control pilot study / Evaluación de la depresión, el estrés, la ansiedad y la calidad de vida relacionada con la salud bucal en personas con síndrome de Sjögren: estudio piloto de casos y controles

Objective: Sjögren's syndrome (SS) is a chronic auto-immune inflammatory systemic disease, in which the infiltration of mo-nonuclear cells in the exocrine glands leads to physiological and morphological changes. This pilot case-control study aims to describe the profile, evaluate the oral condition, quality of life (QoL) and psychological condition, through complete clinical examination, OHIP-14 and DASS-21 questionnaires. Materials and Methods: The study was conducted with seven individuals with a final diagnosis of SS (case group [CG]), and seven individuals with symptoms of dry mouth (control group [GCO]), consulting at the institution from January to November 2021. participants were selected by free demand and those previously seen at the institution with a diagnosis of SS between 19 and 70 years of age. The questionnaire OHIP-14 was applied to assess the patient's quality of life, where seven dimensions are assessed, subdivided into 14 questions through the Lickert scale (0 to 4) assigned by the individual and which quantifies the impact of oral health on QoL. The questionnaire DASS-21 assessed the psychological condition of the patient, which presents seven questions for each emotional state (depression, anxiety, and stress), totaling 21 questions. The general clinical condition, evolution of SS, oral clinical condition, and the profile of this population were related to QoL factors and psychological conditions, using these assessment instruments. Results: There was no statistically significant difference between the groups regarding stimulated salivary flow. The only symptom with a statistically significant difference in the CG was difficulty in phonation (p< 0.001). The dimensions related to functional limitation and physical pain showed the most expressive results (p=0.004) (p=0.025), showing a strong negative impact on the QoL of the CG individuals, and the dimension related to disability was the least affected (p=0.684). The analysis of depression, anxiety, and stress did not show statistically significant results between the groups; however, in the CG, 5 (71.42%) individuals showed a severe degree of depression, anxiety, and stress. Conclusions: Individuals in the case group showed some changes, with a strong negative impact on QoL compared to the control group.

Objetivo: El síndrome de Sjögren (SS) es una enfermedad inflamatoria sistémica crónica autoinmune, en la que la infiltración de células mononucleares en las glándulas exocrinas provoca cambios fisiológicos y morfológicos. Este estudio piloto de casos y controles tiene como objetivo describir el perfil, evaluar la condición bucal, calidad de vida (CdV) y condición psicológica, mediante examen clínico completo, cuestionarios OHIP-14 y DASS-21. Materiales y Métodos: El estudio se realizó con 7 individuos con diagnóstico final de SS, grupo de casos (CG) y 7 individuos con síntomas de sequedad bucal, grupo control (GCO) atendidos en la institución de enero a noviembre de 2021. Los participantes fueron seleccionados por libre demanda y entre los atendidos previamente en la institución con diagnóstico de SS entre 19 y 70 años de edad. Para evaluar la calidad de vida del paciente se aplicó el cuestionario OHIP-14, donde se evalúan siete dimensiones, sub-divididas en 14 preguntas a través de la escala de Likert (0 a 4) asignada por el individuo y que cuantifica el impacto de la salud bucal en la calidad de vida. El cuestionario DASS-21 evaluó la condición psicológica del paciente, el cual presenta siete preguntas para cada estado emocional (depresión, ansiedad y estrés), totalizando 21 preguntas. El estado clínico general, la evolución del SS, el estado clínico bucal y el perfil de esta población se relacionaron con factores de calidad de vida y condiciones psicológicas, mediante estos instrumentos de evaluación. Resultados: En cuanto al flujo salival estimulado, no hubo diferencias estadísticamente significativas entre los grupos. El único síntoma que mostró diferencia estadísticamente significativa en el CG fue la dificultad en la fonación (p< 0,001). Las dimensiones relacionadas con limitación funcional y dolor físico mostraron los resultados más expresivos (p=0,004) (p=0,025), mostrando un fuerte impacto negativo en la CdV de los individuos del GC, y la dimensión relacionada con discapacidad fue la menos afectada (p=0,684). El análisis de depresión, ansiedad y estrés no mostró resultados estadísticamente significativos entre los grupos; sin embargo, en el GC, 5 (71,42%) individuos presentaron un grado severo de depresión, ansiedad y estrés. Conclusión: Se puede concluir que los individuos del grupo de casos mostraron algunos cambios, con un fuerte impacto negativo en la calidad de vida en comparación con el grupo de control.

Cross-sectional assessment of sleep and fatigue in middle-aged Japanese women with primary Sjogren syndrome or rheumatoid arthritis using self-reports and wrist actigraphy.

ABSTRACT: To investigate fatigue, health-related quality of life (HR-QOL), and sleep quality in women with primary Sjogren syndrome (pSS) or rheumatoid arthritis (RA) as compared with healthy controls using self-reports and wrist actigraphy.In this cross-sectional observational study, we evaluated a total of 25 patients (aged 40-75 years) with pSS, 10 with RA, and 17 healthy control subjects living in Japan. The HR-QOL was assessed using the Short Form-36. Fatigue was evaluated using the Short Form-36 vitality score, visual analog scale (VAS) for fatigue, and 2 questionnaire items using scores based on a 4-point Likert scale. Sleep quality was measured using the Japanese version of the Pittsburgh Sleep Quality Index, VAS for sleep quality, and wrist actigraphy for 14 days.Patients with pSS reported severer fatigue and lower HR-QOL than healthy controls, especially in mental health. Based on the Pittsburgh Sleep Quality Index score, 56% of the patients with pSS were poor sleepers, which was higher than healthy controls (29.4%). Furthermore, the patients with pSS scored significantly lower on the VAS for sleep quality than healthy controls (40.5 vs 63.7, P = .001). Although subjective assessments revealed slight sleep disturbances in patients with pSS, wrist actigraphy revealed no differences when compared with healthy controls for total sleep time (421.8 minutes vs 426.5 minutes), sleep efficiency (95.2% vs 96.4%), number of awakenings (1.4 vs 0.9), and wake after sleep onset (22.4 minutes vs 16.1 minutes). Poor subjective sleep quality was associated with enhanced fatigue. However, sleep efficiency, as determined by actigraphy, was not associated with fatigue. Notably, the patients with RA and healthy controls did not differ significantly in terms of fatigue or sleep quality, although patients with RA experienced more nocturnal awakenings than healthy controls (1.7 vs 0.9, P = .04).Patients with pSS experience severe fatigue, poor HR-QOL, and sleep disturbances, which are associated with fatigue. However, wrist actigraphy did not reveal differences in sleep quality, suggesting that it may not be an appropriate measure of sleep in patients with pSS.

Labial salivary gland assessment in idiopathic pulmonary fibrosis patients with sicca symptoms.

OBJECTIVE: Patients with idiopathic pulmonary fibrosis (IPF) commonly present with sicca symptoms. This study aimed to assess labial minor salivary glands (LMSGs) in those patients to rule out Sjögren's syndrome (SS), in which sicca symptoms are the clinical hallmark. STUDY DESIGN: Cases of patients with IPF with sicca symptoms referred to the oral medicine clinic at the University of Florida within the last 13 years were selected with institutional review board approval. Demographic characteristics, clinical findings, laboratory results, and histomorphologic parameters were retrospectively analyzed. RESULTS: A total of 12 patients (9 men and 3 women, ages 55-76 years) were identified. History of exposure to asbestos or chemicals, smoking, and medication information was obtained. All patients reported sicca symptoms with 57% of those exhibiting objective or borderline dryness. Anti-SSA/Ro and anti-SSB/La were positive in 25% and 8% of the cases, respectively. Microscopically, 1 out of 12 patients was biopsy positive in the absence of anti-SSA/Ro, fulfilling the 2016 SS criteria with positive sialometry. CONCLUSIONS: A LMSG biopsy is critical to identify SS in patients with diagnosed IPF and present sicca symptoms, especially those with negative serology, as revealed in our study.

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.

Burden of illness among subgroups of patients with primary Sjögren's syndrome and systemic involvement.

OBJECTIVES: To describe how patients with primary SS (pSS) and systemic organ involvement are classified and clustered in routine practice. METHODS: This multinational, cross-sectional survey of real-world quantitative data was conducted across Europe and the US. Rheumatologists who treated seven or more adult patients per month with pSS and current/past systemic manifestations undertook a survey before completing a patient record form capturing demographic, clinical and treatment information for their next six eligible patients. Patients with a completed patient record form were invited to complete a patient self-completion questionnaire capturing insights into their disease and treatment. Subgroups were defined by physicians' assessment of disease severity; clusters were derived based on key clinical characteristics using latent class analysis. RESULTS: Rheumatologists completed 316 physician surveys and 1879 patient record forms; 888 patients completed the patient self-completion questionnaire. pSS severity reflected organ involvement and symptomatology. Latent class analysis produced five clusters distinguished by the organ systems involved and the presence of pain and fatigue symptoms at the time of the survey. A minority of patients [n = 67 (4%)] were categorized with multiple organ involvement and the highest frequency of pain and fatigue. A total of 324 patients (17%) were categorized as 'low burden'. The remaining three clusters exhibited high frequencies of articular involvement but were distinguished by the extent of other organ system involvement. CONCLUSION: Cluster analysis using a real-world cohort of patients with pSS and systemic organ involvement highlights the heterogeneous presentation of patients with pSS and confirms the importance of pain and fatigue as well as organ involvement when determining disease burden.

Increased risk of developing dental diseases in patients with primary Sjögren's syndrome-A secondary cohort analysis of population-based claims data.

BACKGROUND: Although it is known that patients with primary Sjögren's syndrome (pSS) have impaired dental conditions, incidence rates and incidence rate ratios of various dental diseases in these patients are not clear. The aim of this study was to investigate the frequency and prevalence of dental diseases in patients with pSS, and to evaluate the risk of common dental diseases in these patients. METHODS: A population-based retrospective cohort study was conducted using the data from the Taiwan's National Health Insurance Research Database. A total of 709 patients with newly diagnosed pSS between 2000 and 2012 were identified to form the pSS cohort. A comparison cohort of patients without pSS was assembled based on frequency matching for sex, 5-year age interval, and index year at a ratio of 10:1. All participants were followed until the end of the follow-up period or when the outcome of interest occurred. The incidence of dental caries, pulpitis, gingivitis, periodontitis, oral ulceration, and stomatitis were calculated using multiple Poisson regression models. RESULTS: A significantly higher prevalence (74.6% vs. 63.0%, P = 0.001) and frequency (median 5.37 vs. 1.45 per year, P < 0.001) dental visits were observed in patients with pSS compared with patients in the comparison cohort. The risk of dental caries (adjusted incidence rate ratio [aIRR] 1.64, P < 0.001), pulpitis (aIRR 1.42, P < 0.001), gingivitis (aIRR 1.43, P < 0.001), periodontitis (aIRR 1.44, P < 0.001), oral ulceration (aIRR 1.98, P < 0.001), and stomatitis (aIRR 2.06, P < 0.001) were significantly higher in patients with pSS. CONCLUSIONS: In this nationwide, population-based cohort study, a higher prevalence and frequency of dental visits were found in patients with pSS. Patients with PSS had increased risk of six most common dental disorders, including dental caries, pulpitis, gingivitis, periodontitis, oral ulceration, and stomatitis. Rheumatologists should remain vigilant for the dental health of patients with pSS.

Entheses ultrasound assessment in primary Sjogren's syndrome.

OBJECTIVE: Musculoskeletal pain is a common complaint among patients with primary Sjogren's syndrome (pSS). Joints clinical examination is oftenly normal. A periarticular origin of this pain may be possible. Since clinical examination lacks sensitivity and precision, the use of musculoskeletal ultrasound (US) is more interesting in the evaluation of the entheses involvement, as it is shown to be a more sensitive tool. Our objective was to assess, by an ultrasonographic study, the entheses involvement in the widespread pain of patients with pSS. METHODS: This is a prospective study including 25 women with pSS and 25 age and sex matched healthy controls. An ultrasound examination, using grey scale and Doppler US, of five enthesitic sites (distal quadricipital, proximal patellar, distal patellar, distal Achillian and distal brachial tricipital) sought bilaterally the following lesions: hypoechogenicity, thickening, loss of fibrillar structure, erosions, enthesophytes, calcifications or Doppler hypervascularisation. A final score was calculated by summing the abnormalities scores of all entheses. RESULTS: The mean age was 53.2±11.3 years in the pSS group and 50.6±9.7 years in the control group. The mean number of pathological entheses on ultrasound was 3.92±1.93 in the pSS group versus 4.52±2.27 in the control group (P>0.05). The total score for enthesitis abnormalities was 4.96±2.59 versus 5.72±2.92 (P>0.05), respectively. There was a positive correlation between total score of ultrasound enthesitic abnormalities and age in both groups. CONCLUSION: In patients with pSS, clinically painful sites were more frequently found than in US. Musculoskeletal pain was not due to enthesitis.

Quantitative assessment of interstitial lung disease in Sjögren's syndrome.

BACKGROUND: Interstitial lung disease (ILD) is a frequent manifestation of Sjögren's syndrome (SS), an autoimmune disease of salivary and lacrimal glands, and affects approximately 20% of patients. No clinical or serological features appear to be useful to predict its presence, severity or progression, and chest high-resolution computed tomography (CT) remains the gold standard for diagnosis. Semiquantitative CT (SQCT) based on visual assessment (Goh and Taouli scoring) can estimate ILD extent, although it is burdened by relevant intra- and interobserver variability. Quantitative chest CT (QCT) is a promising alternative modality to assess ILD severity. AIM: To determine whether QCT assessment can identify extensive or limited lung disease in patients with SS and ILD. METHODS: This multi-center, cross-sectional and retrospective study enrolled patients with SS and a chest CT scan. SQCT assessment was carried out in a blinded and centralized manner to calculate both Goh and Taouli scores. An operator-independent analysis of all CT scans with the open-source software platform Horos was used to evaluate the QCT indices. Patients were classified according to the extent of ILD and differences in QCT index distribution were investigated with non-parametric tests. RESULTS: From a total of 102 consecutive patients with SS, the prevalence of ILD was 35.3% (36/102). There was a statistically significant difference in QCT index distribution between the SS with ILD and SS without ILD groups (p<0.001). Moreover, SS-ILD patients with ILD >20% (by Goh score) had a QCT index statistically different from those with limited ILD extent (p<0.001). Finally, QCT indices showed a moderate-to-good correlation with the Goh and Taouli scores (from 0.44 to 0.65; p<0.001). CONCLUSIONS: QCT indices can identify patients with SS and ILD and discriminate those with lesser or greater lung disease.

Frecuencia del síndrome de Sjögren en pacientes con lupus eritematoso del Hospital Central del Instituto de Previsión Social en el año 2017 que acuden a tratamiento odontológico / Frequency of sjögren's syndrome in patients with lupus erythematosus at the Central Hospital of the Social Welfare Institute in 2017 who seek dental treatment

La xerostomía, síndrome de boca seca o síndrome de Sjögren se define como la disminución del flujo salival en condiciones de reposo. El origen de esta endad clínica es mulcausal, pudiendo ser el resultado de una alteración localizada sobre las glándulas productoras de la saliva, o bien el resultado de un desequilibrio o alteración de índole sistémica. La evaluación del grado de disfunción de las glándulas salivares ha constuido un objevo básico por ello el propósito de este estudio fue determinar frecuencia del síndrome de Sjögren en pacientes con lupus eritematoso del hospital central del Instuto de Previsión Social en el año 2017 que acuden a tratamiento odontológico. Se realizó un descripción observacional retrospecvo para lo cual se recurrió a la historial clínico previo permiso del jefe de reumatología para acceder a los datos . Como conclusión general se obtuvo que existe una predominancia de pacientes de entre 46 a 50 años de edad representada por el 39% y de sexo femenino en el 83% de los casos. Se demuestra la frecuencia mencionando que acudieron 74 pacientes y de los cuales 18 (24%) personas presentan lupus eritematoso y síndrome de Sjögren, y entre estos el 78% acude a tratamiento odontológico.

Xerostomia, dry mouth syndrome or Sjögren's syndrome is defined as decreased salivary flow under resng condions. The origin of this clinical enty is mulcausal, and may be the result of an alteraon located on the glands producing saliva, or the result of an imbalance or alteraon of a systemic nature. The evaluaon of the degree of dysfuncon of the salivary glands has been a basic objecve and therefore the purpose of this study was to determine the frequency of Sjögren syndrome in paents with lupus erythematosus from the central hospital of the Social Welfare Instute in 2017 who seek dental treatment. A retrospecve observaonal descripon was made, for which the clinical history was used with the prior permission of the head of rheumatology to access the data. The general conclusion was that there is a predominance of paents between 46 and 50 years of age represented by 39% and female in 83% of cases. The frequency is demonstrated by menoning that 74 paents aended and of which 18 (24%) people present with lupus erythematosus and Sjögren's syndrome, of which 78% aend dental treatment

Ten-year fracture risk by FRAX and osteoporotic fractures in patients with systemic autoimmune diseases.

The Fracture Risk Assessment Tool (FRAX) has been used universally for the purpose of fracture risk assessment. However, the predictive capacity of FRAX for autoimmune diseases remains inconclusive. This study aimed to compare the applicability of FRAX for autoimmune disease patients. This retrospective study recruited rheumatoid arthritis (RA), systemic lupus erythematosus (SLE) and primary Sjögren syndrome (pSS) patients with bone mineral density (BMD) tests. Patients with any osteoporotic fractures were identified. Taiwan-specific FRAX with and without BMD were then calculated. In total, 802 patients (451 RA, 233 SLE and 118 pSS) were enrolled in this study. The cumulative incidences of osteoporotic fractures in the RA, SLE and pSS patients were 43.0%, 29.2% and 33.1%, respectively. For those with a previous osteoporotic fracture, T-scores were classified as low bone mass. Overall, the patients' 10-year probability of major fracture risk by FRAX without BMD was 15.8%, which then increased to 20.3% after incorporation of BMD measurement. When analyzed by disease group, the fracture risk in RA patients was accurately predicted by FRAX. In contrast, current FRAX, either with or without BMD measurement, underestimated the fracture risk both in SLE and pSS patients, even after stratification by age and glucocorticoid treatment. For pSS patients with major osteoporotic fractures, FRAX risks imputed by RA were comparable to major osteoporotic fracture risks of RA patients. Current FRAX accurately predicted fracture probability in RA patients, but not in SLE and pSS patients. RA-imputed FRAX risk scores could be used as a temporary substitute for SLE and pSS patients.

Tear Meniscus and Corneal Sub-basal Nerve Plexus Assessment in Primary Sjögren Syndrome and Sicca Syndrome Patients.

PURPOSE: To evaluate lower tear meniscus and corneal sub-basal nerve plexus in primary Sjögren's syndrome (pSS) and Sicca syndrome patients. METHODS: Cross-sectional study of 116 patients with Sicca syndrome associated with pSS and not associated with Sjögren's syndrome (non-SS Sicca) and 20 normal control subjects. Tear meniscus height and area were measured using anterior segment optical coherence tomography; corneal sub-basal nerve plexus density, length, and tortuosity were evaluated using in vivo confocal microscopy. Data analysis was performed using IBM-SPSS Statistics 24.0. RESULTS: Corneal sub-basal nerve plexus density and length were significantly lower, and tortuosity was significantly higher in pSS and non-SS Sicca groups than in normal control subjects (P < 0.001; P = 0.018, respectively). Corneal sub-basal nerve plexus presented a strong association with Schirmer test I and tear breakup time. Cutoff values of sub-basal nerve plexus density (36.5 nerve/mm) and length (12.5 mm/mm) presented 80.2% to 81.9% sensitivity and 85% specificity for detecting Sicca syndrome patients. No significant differences were found between the 3 groups regarding tear meniscus height and area. CONCLUSIONS: Corneal sub-basal nerve plexus in vivo confocal microscopy may be a useful tool in the assessment of dry eye disease in Sicca syndrome, complementing the information provided by the conventional modalities used in dry eye disease evaluation.

Risk of Ischemic Heart Disease in Patients With Sjögren's Syndrome.

BACKGROUND: Ischemic heart disease (IHD) has emerged as a major cause of morbidity and mortality in patients with autoimmune conditions such as systemic lupus erythematosus and rheumatoid arthritis, but the risk of IHD in Sjögren's syndrome (SjS) is unknown. To fill this knowledge gap, we estimated the prevalence and risk of IHD with SjS compared to controls from the general population using the Healthcare Cost and Utilization Project National Inpatient Sample 2011 database. MATERIALS AND METHODS: The Healthcare Cost and Utilization Project administrative longitudinal database contains encounter-level information on inpatient stays, emergency department visits and ambulatory surgery in all U.S. hospitals. We conducted a cross-sectional study among the inpatient population diagnosed with SjS and matched 1:4 with controls for age, sex and hospital region. Odds ratio for IHD was calculated as cases compared to controls. The contribution of various risk factors to IHD was also evaluated by logistic regression. RESULTS: Analysis demonstrated that 7,154 of 13,086 cases (54.7%) of SjS had IHD compared to 27,367 of 52,448 controls (52.2%). The adjusted odds ratio for IHD in those with SjS was 0.898 (95% CI: 0.844-0.955). Patients with SjS were significantly more likely to have hypertension, diabetes, apnea and lipid disorders. CONCLUSIONS: To our knowledge, this is the largest population-based study investigating the risk of IHD in patients with SjS. We found a modest, though statistically significant, decrease in the risk of IHD in SjS compared to controls.

Sjogren's syndrome: Clinical aspects.

Sjogren's syndrome (SS) is the 2nd most common chronic autoimmune rheumatic disease and associated with a high burden of illness. Morbidity arises not only from untreated xerostomia and keratoconjunctivitis sicca but also from extra-glandular manifestations including the development of non-Hodgkin's B cell lymphomas. Proper diagnosis of SS requires objective evidence of dry eyes and/or objective evidence of dry mouth as well as proof of autoimmunity. The recent development of new international classification criteria and clinical practice guidelines for SS should not only enhance the existing standards of care but also facilitate further studies to improve future diagnosis and outcomes.

Randomized Controlled Trial of Rituximab and Cost-Effectiveness Analysis in Treating Fatigue and Oral Dryness in Primary Sjögren's Syndrome.

OBJECTIVE: To investigate whether rituximab, an anti-B cell therapy, improves symptoms of fatigue and oral dryness in patients with primary Sjögren's syndrome (SS). METHODS: We conducted a multicenter, randomized, double-blind, placebo-controlled, parallel-group trial that included health economic analysis. Anti-Ro-positive patients with primary SS, symptomatic fatigue, and oral dryness were recruited from 25 UK rheumatology clinics from August 2011 to January 2014. Patients were centrally randomized to receive either intravenous (IV) placebo (250 ml saline) or IV rituximab (1,000 mg in 250 ml saline) in 2 courses at weeks 0, 2, 24, and 26, with pre- and postinfusion medication including corticosteroids. The primary end point was the proportion of patients achieving a 30% reduction in either fatigue or oral dryness at 48 weeks, as measured by visual analog scale. Other outcome measures included salivary and lacrimal flow rates, quality of life, scores on the European League Against Rheumatism (EULAR) Sjögren's Syndrome Patient Reported Index and EULAR Sjögren's Syndrome Disease Activity Index, symptoms of ocular and overall dryness, pain, globally assessed disease activity, and cost-effectiveness. RESULTS: All 133 patients who were randomized to receive placebo (n = 66) or rituximab (n = 67) were included in the primary analysis. Among patients with complete data, 21 of 56 placebo-treated patients and 24 of 61 rituximab-treated patients achieved the primary end point. After multiple imputation of missing outcomes, response rates in the placebo and rituximab groups were 36.8% and 39.8%, respectively (adjusted odds ratio 1.13 [95% confidence interval 0.50, 2.55]). There were no significant improvements in any outcome measure except for unstimulated salivary flow. The mean ± SD costs per patient for rituximab and placebo were £10,752 ± 264.75 and £2,672 ± 241.71, respectively. There were slightly more adverse events (AEs) reported in total for rituximab, but there was no difference in serious AEs (10 in each group). CONCLUSION: The results of this study indicate that rituximab is neither clinically effective nor cost-effective in this patient population.

Vision-related quality of life and psychological status in Chinese women with Sjogren's syndrome dry eye: a case-control study.

BACKGROUND: Sjogren's syndrome dry eye (SSDE) mainly affects middle-aged women and can negatively affect women's psychological and social functioning. However, little is known about the correlation between vision-related quality of life (VR-QoL) and psychological status for women with SSDE. We therefore examined VR-QoL and psychological status in two groups of Chinese women: an SSDE group and a non-SSDE group. We also explored the associations between VR-QoL scores, sociodemographic measures, ophthalmologic parameters, and psychological status in women with SSDE. METHODS: The case-control study recruited 30 female outpatients with SSDE and 30 without SSDE from the Eye and Ear, Nose, and Throat (ENT) Hospital of Fudan University. Demographic and ophthalmologic data were collected from all participants. Ophthalmologic examinations included best-corrected visual acuity (BCVA), corneal fluorescein staining (CFS), tear break-up time (TBUT) and Schirmer test. Data collected using the National Eye Institute's Visual Function Questionnaire (NEI-VFQ) and Ocular Surface Disease Index (OSDI) survey instruments were analyzed to identify potential differences in VR-QoL between the SSDE group and the non-SSDE group. We also used the Zung Self-Rating Anxiety and Self-Rating Depression Scales (SAS and SDS) to determine psychological status in both groups. RESULTS: The SSDE group scored significantly lower than the non-SSDE group on the NEI-VFQ subscales of general health, general vision, and long-distance vision activities (all p < 0.05). The SSDE group achieved a significantly higher ocular symptoms score compared with the control group (p = 0.0256). The SAS and SDS scores of the SSDE group were significantly higher than the non-SSDE group (p = 0.0072 and 0.0162, respectively). The prevalence of anxiety and depression in the SSDE group was significantly higher than the non-SSDE group (p = 0.0240 and 0.0200, respectively). Nine of twelve NEI-VFQ subscales were negatively correlated with SAS/SDS scores (all p values were <0.05). The exceptions were social function, color vision and peripheral vision. The composite OSDI score and its three subscale scores for the women in the SSDE group were all positively correlated with overall SAS/SDS scores (all p values were <0.05). CONCLUSIONS: Both VR-QoL and psychological status were significantly worse in SSDE group than in the non-SSDE group. The VR-QoL of women with SSDE had a negative correlation with their anxiety and depression levels.

The Quality of Life Burden Associated With Voice Disorders in Sjögren's Syndrome.

OBJECTIVES: This study examined quality of life burden of voice disorders in Sjögren's syndrome (SS). METHODS: Patients with SS (n = 101) completed interviews involving patient-reported histories of voice disorders, specific voice symptoms, SS disease severity, the Voice-Related Quality of Life (V-RQOL), and the general health-related quality of life Short Form 36 (SF-36) questionnaires. Relationships among voice symptoms, disease severity, and quality-of-life measures were examined and compared with patient-reported voice disorders. RESULTS: Significant correlations were observed among voice symptoms, disease severity, V-RQOL, SF-36, and patient-reported voice disorders (P < .05). Patients with SS who reported a voice disorder experienced a greater burden on general quality of life as compared with those without voice disorders. Specific voice symptoms significantly correlated with reduced SF-36 scores included frequent throat-clearing, throat soreness, difficulty projecting, and vocal discomfort. Despite the added burden of a voice disorder on quality of life in SS, voice-related treatment seeking was low (15.8%). However, the majority of patients who received voice treatment reported voice improvement. CONCLUSIONS: Individuals with SS frequently experience voice disorders and specific voice-related symptoms that are associated with reduced quality of life. These findings have important implications for voice referral practices and voice disorder symptom management in this population.

Avaliação da fadiga e da secura na síndrome de Sjögren primária: versão brasileira do “Profile of Fatigue and Discomfort – Sicca Symptoms Inventory (short form) (Profad-SSI-SF)” / Assessment of fatigue and dryness in primary Sjögren’s syndrome: Brazilian version of “Profile of Fatigue and Discomfort – Sicca Symptoms Inventory (short form) (PROFAD-SSI-SF)”

Objetivo: Fazer a adaptação transcultural e a validação do Profile of Fatigue and Discomfort – Sicca Symptoms Inventory (short form) (Profad-SSI-SF), questionário que avalia os aspectos subjetivos dos sintomas da síndrome de Sjögren primária (SSp), para a língua portuguesa brasileira. Método: Foi avaliada a equivalência conceitual, de item, semântica e operacional. A versão brasileira do Profad-SSI-SF foi aplicada a 62 mulheres com SSp conforme consenso europeu-americano de 2002 para avaliar a equivalência de mensuração. Foi usado o α-Cronbach para consistência interna; coeficiente de correlação intraclasse (ICC) para reprodutibilidade intraobservador; e coeficiente de correlação de Spearman para validade em comparação com o Patient Global Assessment (PaGA), Eular Sjögren’s Syndrome Patient Reported Index (ESS-PRI), Functional Assessment of Chronic Illness Therapy Fatigue Subscale (Facit-F) e EuroQOL(EQ-5D). Resultados: A consistência interna do Profad, do SSI e da pontuação total foi de 0,80, 0,78 e 0,87, respectivamente. A reprodutibilidade intraobservador do Profad total foi de 0,89; do SSI total de 0,86 e da pontuação total de 0,89. Na validade, o Profad apresentou correlação significativa com o PaGA (r = 0,50), Facit-F (r = 0,59), Esspri (r = 0,58) e todos os domínios do EQ-5D, com exceção da mobilidade. Já o SSI apresentou correlação significativa com o PaGA (r = 0,43), Facit-F (r = 0,57), Esspri (r = 0,55) e a maioria dos domínios do EQ-5D. A pontuação total do Profad-SSI-SF só não obteve correlação estatisticamente significante com o domínio mobilidade e escala 1 a 100 do EQ-D5. .

Objective: To perform a cross-cultural adaptation and validation of the Profile of Fatigue and Discomfort – Sicca Symptoms Inventory (short form) (PROFAD-SSI-SF) questionnaire assessing the subjective aspects of the symptoms of primary Sjögren syndrome (pSS), for the Brazilian Portuguese language. Method: Conceptual, of the item, semantic and operational equivalences were evaluated. The Brazilian version of PROFAD-SSI-SF was administered to 62 women with pSS according to the European-American consensus 2002 to assess measurement equivalence. α-Cronbach was used for internal consistency; intraclass correlation coefficient (ICC) for intraobserver reproducibility; and Spearman correlation coefficient for validity by comparing with Patient Global Assessment (PaGA), EULAR Sjögren’s Syndrome Patient Reported Index (ESSPRI), Functional Assessment of Chronic Illness Therapy Fatigue Subscale (FACIT-F) and EuroQOL(EQ-5D). Results: The internal consistency of PROFAD, SSI and total score was 0.80; 0.78; and 0.87, respectively. The intraobserver reproducibility of total PROFAD was 0.89; of total SSI was 0.86; and total score was 0.89. In terms of validity, PROFAD correlated significantly with PaGA (r = 0.50), FACIT-F (r = 0.59), ESSPRI (r = 0.58) and all domains of EQ-5D, with the exception of Mobility. On the other hand, SSI correlated significantly with PaGA (r = 0.43), FACIT-F (r = 0.57), ESSPRI (r = 0.55) and most domains of EQ-5D. The total score of PROFAD-SSI-SF had a nonstatistically significant correlation only with Mobility domain and with 1–100 range of EQ-5D. .

Assessment of fatigue and dryness in primary Sjögren's syndrome: Brazilian version of "Profile of Fatigue and Discomfort - Sicca Symptoms Inventory (short form) (PROFAD-SSI-SF)".

OBJECTIVE: To perform a cross-cultural adaptation and validation of the Profile of Fatigue and Discomfort - Sicca Symptoms Inventory (short form) (PROFAD-SSI-SF) questionnaire assessing the subjective aspects of the symptoms of primary Sjögren syndrome (pSS), for the Brazilian Portuguese language. METHOD: Conceptual, of the item, semantic and operational equivalences were evaluated. The Brazilian version of PROFAD-SSI-SF was administered to 62 women with pSS according to the European-American consensus 2002 to assess measurement equivalence. α-Cronbach was used for internal consistency; intraclass correlation coefficient (ICC) for intraobserver reproducibility; and Spearman correlation coefficient for validity by comparing with Patient Global Assessment (PaGA), EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI), Functional Assessment of Chronic Illness Therapy Fatigue Subscale (FACIT-F) and EuroQOL (EQ-5D). RESULTS: The internal consistency of PROFAD, SSI and total score was 0.80; 0.78; and 0.87, respectively. The intraobserver reproducibility of total PROFAD was 0.89; of total SSI of 0.86; and total score of 0.89. In terms of validity, PROFAD correlated significantly with PaGA (r = 0.50), FACIT-F (r = 0.59), ESSPRI (r = 0.58) and all domains of EQ-5D, with the exception of Mobility. On the other hand, SSI correlated significantly with PaGA (r = 0.43), FACIT-F (r = 0.57), ESSPRI (r = 0.55) and most areas of EQ-5D. The total score of PROFAD-SSI-SF had a non-statistically significant correlation only with Mobility domain and with 1-100 range of EQ-5D. CONCLUSION: The Portuguese version of PROFAD-SSI-SF proved to be an adaptable, reproducible and valid tool for the Brazilian Portuguese language.