Symptom-Based Cluster Analysis Categorizes Sjögren's Disease Subtypes: An International Cohort Study Highlighting Disease Severity and Treatment Discordance.

OBJECTIVE: Although symptom relief is a critical aspect for successful drug development in Sjögren's disease, patient experiences with Sjögren's-related symptoms are understudied. Our objective was to determine how pain, dryness, and fatigue, the cardinal symptoms of Sjögren's disease, drive cluster phenotypes. METHODS: We used data from the Sjögren's International Collaborative Clinical Alliance (SICCA) Registry and a Sjögren's Foundation survey. We performed hierarchical clustering of symptoms by levels of dryness, fatigue, and pain. Using international and US cohorts, we performed multiple logistic regression analysis to compare the clusters, which included comparisons of differences in symptoms, quality of life (QoL), medication use, and systemic manifestations. RESULTS: Four similar clusters were identified among 1,454 SICCA registrants and 2,920 Sjögren's Foundation survey participants: 1) low symptom burden in all categories (LSB); 2) dry with low pain and low fatigue (DLP); 3) dry with high pain and low to moderate fatigue (DHP); and 4) high symptom burden in all categories (HSB). Distribution of SICCA registrants matching the symptom profile for each cluster was 10% in the LSB cluster, 30% in the DLP cluster, 23% in the DHP cluster, and 37% in the HSB cluster. Distribution of survey participants matching the symptom profile for each cluster was 23% in the LSB cluster, 14% in the DLP cluster, 21% in the DHP cluster, and 42% in the HSB cluster. Individuals in the HSB cluster had more total symptoms and lower QoL but lower disease severity than those in the other clusters. Despite having milder disease as measured by laboratory tests and organ involvement, individuals in the HSB cluster received immunomodulatory treatment most often. CONCLUSION: We identified 4 symptom-based Sjögren's clusters and showed that symptom burden and immunomodulatory medication use do not correlate with Sjögren's end-organ or laboratory abnormalities. Findings highlight a discordance between objective measures and treatments and offer updates to proposed symptom-based clustering approaches.

A comprehensive overview of living with Sjögren's: results of a National Sjögren's Foundation survey.

To gain insight into the Sjögren's disease (SjD) patient experience using a survey generated by patients and providers. We evaluated the results of the 2016 Sjögren's Foundation survey, with 25 questions designed in a collaborative effort between the Foundation, patients with SjD, SjD provider experts, and a marketing research company. We used descriptive statistics to provide a thorough understanding of SjD demographics, symptoms, quality of life (QoL), cost, and treatments. Analyses revealed high symptoms, QoL, and financial burdens in SjD. Dry mouth and eye were the most commonly reported symptoms (94 and 93%, respectively). The most frequent extra-glandular symptoms included fatigue, dry or itchy skin, and morning stiffness. The top three aspects of QoL most impaired included (i) sex life (53%), (ii) participating in hobbies/social activities/extracurricular activities (52%), and (iii) job/career or ability to work (49%). SjD respondents commonly reported taking health food supplements/remedies, vitamin D, and exercising, in addition to taking treatments for symptomatic dryness. SjD costs were high, including a total yearly cost, on average, of $2026 for dental care. SjD respondents reported that dryness and risk factors for lymphoma and fatigue are essential to address with new therapies. In this comprehensive overview of the SjD experience, we demonstrated a high burden of disease to SjD respondents, including symptoms, QoL, and financial burden. We also identify the top goals of therapy for new systemic SjD therapies. Key Points • The top three symptoms or signs that patients with Sjögren's hope new treatments will address are dryness, fatigue, and reduction in lymphoma or blood cancer risk • The top aspects of quality of life reported to be impaired by Sjögren's are sex life, hobbies, social activities and extracurricular activities, job/career or ability to work, and finding the correct word during conversations • Patients with Sjögren's have a yearly mean dental cost of $2026 but also have high costs associated with prescription medications, healthcare appointments, over-the-counter medications, alternative therapies, and medical equipment.

National Sjögren's Foundation Survey: Burden of Oral and Systemic Involvement on Quality of Life.

OBJECTIVE: To define the association between oral and systemic manifestations of Sjögren syndrome (SS) and quality of life (QOL). METHODS: We analyzed a cross-sectional survey conducted by the Sjögren's Foundation in 2016, with 2961 eligible responses. We defined oral symptom and sign exposures as parotid gland swelling, dry mouth, mouth ulcers/sores, oral candidiasis, trouble speaking, choking or dysphagia, sialolithiasis or gland infection, and dental caries. Systemic exposures included interstitial lung disease, purpura/petechiae/cryoglobulinemia, vasculitis, neuropathy, leukopenia, interstitial nephritis, renal tubular acidosis, autoimmune hepatitis, primary biliary cholangitis, or lymphoma. Outcomes included SS-specific QOL questions generated by SS experts and patients. RESULTS: Using multivariable regression models adjusted for age, sex, race, and employment, we observed that mouth ulcers or sores, trouble speaking, and dysphagia were associated with poor quality of life. The following oral aspects had the greatest effect on the following QOL areas: (1) mouth ulcers/sores on the challenge and burden of living with SS (OR 4.26, 95% CI 2.89-6.28); (2) trouble speaking on memory and concentration (OR 4.24, 95% CI 3.28-5.48); and (3) dysphagia on functional interference (OR 4.25, 95% CI 3.13-5.79). In contrast, systemic manifestations were associated with QOL to a lesser extent or not at all. CONCLUSION: Oral manifestations of SS, particularly mouth ulcers or sores, trouble speaking, and dysphagia, were strongly associated with worse QOL. Further study and targeted treatment of these oral manifestations provides the opportunity to improve quality of life in patients with SS.

Diabetes, obesity, and recommended fruit and vegetable consumption in relation to food environment sub-types: a cross-sectional analysis of Behavioral Risk Factor Surveillance System, United States Census, and food establishment data.

BACKGROUND: Social and spatial factors are an important part of individual and community health. The objectives were to identify food establishment sub-types and evaluate prevalence of diabetes, obesity, and recommended fruit and vegetable consumption in relation to these sub-types in the Washington DC metropolitan area. METHODS: A cross-sectional study design was used. A measure of retail food environment was calculated as the ratio of number of sources of unhealthier food options (fast food, convenience stores, and pharmacies) to healthier food options (grocery stores and specialty food stores). Two categories were created: ≤ 1.0 (healthier options) and > 1.0 (unhealthier options). k-means clustering was used to identify clusters based on proportions of grocery stores, restaurants, specialty food, fast food, convenience stores, and pharmacies. Prevalence data for county-level diabetes, obesity, and consumption of five or more fruits or vegetables per day (FV5) was obtained from the Behavioral Risk Factor Surveillance System. Multiple imputation was used to predict block-group level health outcomes with US Census demographic and economic variables as the inputs. RESULTS: The healthier options category clustered into three sub-types: 1) specialty food, 2) grocery stores, and 3) restaurants. The unhealthier options category clustered into two sub-types: 1) convenience stores, and 2) restaurants and fast food. Within the healthier options category, diabetes prevalence in the sub-types with high restaurants (5.9 %, p = 0.002) and high specialty food (6.1 %, p = 0.002) was lower than the grocery stores sub-type (7.1 %). The high restaurants sub-type compared to the high grocery stores sub-type had significantly lower obesity prevalence (28.6 % vs. 31.2 %, p < 0.001) and higher FV5 prevalence (25.2 % vs. 23.1 %, p < 0.001). Within the larger unhealthier options category, there were no significant differences in diabetes, obesity, or higher FV5 prevalence across the two sub-types. However, restaurants (including fast food) sub-type was significantly associated with lower diabetes and obesity, and higher FV prevalence compared to grocery store sub-type. CONCLUSIONS: These results suggest that there are sub-types within larger categories of food environments that are differentially associated with adverse health outcomes. These observations support the specific food establishment composition of an area may be an important component of the food establishment-health relationship.