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Objectives: Sjögren's disease (SjD) is a heterogenous disease with a wide range of manifestations, ranging from symptoms of dryness, fatigue, and pain, to systemic involvement. Considerable advances have been made to evaluate systemic activity or patient-reported outcomes, but most of the instruments were not able to assess all domains of this multifaceted disease. The aim of this scoping review was to generate domains that have been assessed in randomized controlled trials, as the first phase of the Outcome Measures in Rheumatology (OMERACT) process of core domain set development., Methods: We systematically searched Medline (Pubmed) and EMBASE between 2002 and March 2023 to identify all randomized controlled trials assessing relevant domains, using both a manual approach and an artificial intelligence software (BIBOT) that applies natural language processing to automatically identify relevant abstracts. Domains were mapped to core areas, as suggested by the OMERACT 2.1 Filter., Results: Among the 5,420 references, we included 60 randomized controlled trials, focusing either on overall disease manifestations (53%) or on a single organ/symptom: dry eyes (17%), xerostomia (15%), fatigue (12%), or pulmonary function (3%). The most frequently assessed domains were perceived dryness (52% for overall dryness), fatigue (57%), pain (52%), systemic disease activity (45%), lacrimal gland function (47%) and salivary function (55%), B-cell activation (60%), and health-related quality of life (40%)., Conclusion: Our scoping review highlighted the heterogeneity of SjD, in the study designs and domains. This will inform the OMERACT SjD working group to select the most appropriate core domains to be used in SjD clinical trials and to guide the future agenda for outcome measure research in SjD., Competing Interests: Declaration of competing interest Yann Nguyen, Maxime Beydon, Nathan Foulquier, Rachael Gordon, Coralie Bouillot, and Katherine M. Hammit declared no competing interest. Simon Bowman reports receiving funds for consulting from Bristol-Myers Squibb, Iqvia, Janssen, Kiniksa, Novartis, Otsuka-Visterra. His-salary is part funded by the NIHR Birmingham Biomedical Research centre, Birmingham, UK. Xavier Mariette received consulting fees from Astra-Zeneca, Bristol Myer Squib, Galapagos, GSK, Novartis and Pfizer; travel fees from Novartis. Sara McCoy received consulting fees from BMS, Novartis, Otuska/Visterra, Horizon, Target RWE, Horizon, and Kiniksa. Her time is supported by the Clinical and Translational Science Award (CTSA) program, through the NIH National Center for Advancing Translational Sciences (NCATS), grant 1KL2TR002374 and NIH/NIDCR R03DE031340. Divi Cornec declares no personal financial competing interests and received research funding from Novartis and GSK. Raphaele Seror reports receiving funds for consulting to Bristol-Myers Squibb, Novartis, GSK, Janssen, Amgen, and Boehringher; honoria from Bristol-Myers Squibb, Boehringher, GSK, and travel fees from Amgen and GSK., (Copyright © 2024. Published by Elsevier Inc.)

Sjögren's disease (SjD) is a systemic autoimmune exocrinopathy with key features of dryness, pain, and fatigue. SjD can affect any organ system with a variety of presentations across individuals. This heterogeneity is one of the major barriers for developing effective disease modifying treatments. Defining core disease domains comprising both specific clinical features and incorporating the patient experience is a critical first step to define this complex disease. The OMERACT SjD Working Group held its first international collaborative hybrid meeting in 2023, applying the OMERACT 2.2 filter toward identification of core domains. We accomplished our first goal, a scoping literature review that was presented at the Special Interest Group held in May 2023. Building on the domains identified in the scoping review, we uniquely deployed multidisciplinary experts as part of our collaborative team to generate a provisional domain list that captures SjD heterogeneity., Competing Interests: Declaration of competing interest Valerie Devauchelle reports receinving funds for consulting to Novartis, Abbvie, Fresenius Kabi. Divi Cornec declares no personal financial competing interests and received research funding from Novartis and GSK. Benjamin A. Fisher has undertaken consultancy for Novartis, BMS, Servier, Galapagos, Roche, UCB, Sanofi and Janssen, and received grant/research support from Janssen, Celgene, Galapagos, Servier. Alberta Hoi reports receiving research funding from AstraZeneca, Bristol-Myers Squibb, Novartis, Janssen. Chiara Baldini reports receiving funds for consulting to GSK, Novartis and Horizon, honoraria for educational events from GSK and Sanofi, support for attending meetings from Abbvie and Bristol-Myers Squibb. WF Ng has consulted for Novartis, GlaxoSmithKline, Abbvie, BMS, Sanofi, MedImmune, Resolves Therapeutics, Janssen and UCB. Simon Bowman receiving funds for consulting from Bristol-Myers Squibb, Iqvia, Janssen, Kiniksa, Novartis, Otsuka-Visterra. His-salary is part funded by the Birmingham Biomedical Research Centre, Birmingham, UK. Karina Torralba reports receiving funds for consulting to Horizon, AstraZeneca, Janssen; for contracted research work with Bioclinica; for clinical trial funding from Novartis, AstraZeneca, GlaxoSmithKline, Amgen. Athena Papas declares grant funding from Novartis and Horizon; advisory board for Novartis. Ionut Pintilie reports receiving funds for consulting to Abbvie, Novartis, Pfizer, Sandoz, Ewopharma, KRKA, Stada, Boehringer Ingelheim, MagnaPharm, MSD. Xavier Mariette declares consulting fee from Astra Zeneca, BMS, Galapagos, GSK, Novartis, and Pfizer. Maria Antonietta D'Agostino, MD, PhD Speakers, or consultant fees from Amgen, Abbvie, BMS, Novartis, Galapagos, UCB, Pfizer, Lily, Janssen. Alan Baer reports receiving funds for consulting to Bristol-Myers Squibb and iCell Gene Therapeutics. Blake M. Warner declares research funding and material transfer agreements with Pfizer, Inc., and Mitobridge, Inc. Soumya D. Chakravarty is an employee of Janssen Scientific Affairs, LLC, and owns stock or stock options in Johnson & Johnson, of which Janssen Scientific Affairs, LLC is a wholly owned subsidiary. Nevsun Inanc reports claims to have received speakers fee from Novartis, Abbvie, Pfizer, UCB, Eli-Lilly and consultancy fee from Abbvie, UCB, Eli-Lilly. Vibeke Strand reports being a founding member of the executive committee of Outcome Measures in Rheumatology (OMERACT) [1992 – present], an international consensus organization that develops and validates outcome measures in rheumatology randomized controlled trials and longitudinal observational studies and has received arms-length funding from as many as 36 sponsors. Md Yuzaiful Md Yusof has received speaker fees from Roche and Novartis and consultancy fees from Aurinia Pharmaceuticals and UCB. Suzanne Arends declares consultancy fees from Argenx and Novartis. Anas Alexis Benyoussef is a consultant for Horus Pharma and Quantel Medical. Sharmila Masli is a consultant for Stellular Bio Inc. and Proteris Biotech. Maureen Rischmueller has undertaken consultancy/speaker engagements for AbbVie, Boehringer Ingelheim, Janssen Global Services, Novartis, Pfizer and Sandoz, and received grant/research support from AbbVie, Amgen, AstraZeneca, BMS, GSK, Janssen, Lilly, Novartis, Pfizer, Servier and UCB. Sara McCoy reports receiving funds for consulting to Bristol-Myers Squibb, Horizon, Novartis, Kiniksa, Targe RWE, Otsuka, Visterra, and iCell. Her time is supported by the Clinical and Translational Science Award (CTSA) program, through the NIH National Center for Advancing Translational Sciences (NCATS), grant 1KL2TR002374 and NIH/NIDCR R03DE031340. Raphaele Seror reports receiving funds for consulting to Bristol-Myers Squibb, Novartis, GSK, Janssen, Amgen. Hendrika Bootsma declares consultancy fees from Argenx, Novartis, BMS, AztraZeneca, Galapagos.Independent grants from AstraZeneca, Novartis, BMS., (Copyright © 2024. Published by Elsevier Inc.)

Objectives: To determine the types of products used to treat dry mouth and their perceived effectiveness, the relationship between salivary function and xerostomia symptoms, and whether salivary function predicts response to management strategies., Study Design: Cross-sectional study of 87 patients with dry mouth and documented unstimulated whole salivary (UWS) and stimulated whole salivary (SWS) flow rates. Participants completed a questionnaire assessing dry mouth complaints and symptoms and effectiveness of specific dry mouth products., Results: Mean (SD) age was 61.7 (12.9) years including 78 (90%) females. 47 (54%) participants had Sjögren's disease. Oral dryness symptoms (0-10 scale) rated highest with a mean (SD) of 7.2 (2.17); other symptoms scored from 3.4 to 5.1. Lower levels of UWS and SWS were associated with worse dry mouth and difficulty speaking, while lower levels of SWS flow alone were associated with greater difficulty swallowing and a decline in taste. More than half of the participants (55%, n = 48) reported using ≥4 dry mouth products. Participants with normal SWS flow had significantly better responses to lozenges and prescription products., Conclusions: Patients with dry mouth and normal stimulated flow rates (i.e., residual salivary capacity) respond better to stimulatory products (parasympathomimetic and lozenges). Salivary flow rate assessments may help with recommendations of dry mouth products., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Purpose: To understand patients' perspectives on living with dry eye disease (DED), and on the unmet needs in DED and chronic ocular surface pain (COSP) management., Methods: A moderated, structured discussion with patients with ocular surface diseases and healthcare professionals (HCPs) was conducted using a virtual platform to capture patients' journey with DED, their opinion on unmet needs, and design and conduct of clinical trials in DED and COSP., Results: Nine participants, including four patient representatives from patient organisations, one ophthalmologist and one optometrist participated in the discussion. Patients had DED of varying severity and aetiology; three patients had Sjögren's. Over 4 weeks, 785 posts were entered on the platform. Prior to diagnosis, patients rarely associated their symptoms with DED. Convenience and symptomatic relief scored higher than treating the disease. Patients expressed the need for plain language information and dialogue with knowledgeable and sensitive HCPs. Online forums and social media were suggested as key recruitment resources, whereas convenience and safety concerns were highlighted as main barriers to enrolment. The need for the inclusion of outcome measures that have a real impact on patients' experience of their condition was highlighted. Both target product profiles were received positively by participants, highlighting the twice-daily dosing regimen and convenience of the products. Participants acknowledged the value of digital tools and suggested the need to feel valued post-trial., Conclusions: This moderated dialogue provided actionable insights on the unmet needs in DED and useful inputs for consideration when designing future clinical trials for DED and COSP.

Background: An understanding of the experience of patients with progressive fibrosing interstitial lung disease (PF-ILD) is needed to select appropriate patient-reported outcome measures (PROMs) to evaluate treatment effect in clinical trials., Methods: A systematic literature review was conducted to develop a preliminary conceptual model of the symptoms experienced by patients with PF-ILD and the impacts the disease has on them. An online survey and consensus meetings were then conducted with 12-14 stakeholders (patients, clinicians, regulatory and payer advisors) to refine the conceptual model and critically appraise how key concepts should be measured by PROMs. PROMs assessed included Living with Idiopathic Pulmonary Fibrosis, Living with Pulmonary Fibrosis, the King's Brief Interstitial Lung Disease questionnaire, Cough and Sputum Assessment Questionnaire, Evaluating Respiratory Symptoms, Leicester Cough Questionnaire, Functional Assessment of Chronic Illness Therapy (Dyspnoea/Fatigue) and St George's Respiratory Questionnaire for Idiopathic Pulmonary Fibrosis., Results: The literature review identified 36 signs/symptoms and 43 impacts directly or indirectly related to pulmonary aspects of PF-ILD. The most relevant symptoms identified by participants included shortness of breath on exertion, fatigue and cough; relevant impacts included effects on physical functioning, activities of daily living and emotional wellbeing. These are presented in a conceptual model. Consensus opinion was that existing PROMs need further modification and validation before use in clinical trials., Conclusions: The conceptual model improves understanding of the symptoms and impacts that living with PF-ILD has on patients' wellbeing. It can help to inform the choice of PROMs in clinical trials and highlight aspects to assess in the clinical care of patients with PF-ILD., Competing Interests: Conflict of interest: M. Wijsenbeek reports receiving support for the present manuscript from Boehringer Ingelheim; grants or contracts received from Hoffmann-La Roche and Boehringer Ingelheim, outside the submitted work; consulting fees received from Roche, Boehringer Ingelheim, Galapagos, Bristol Myers Squibb, Galecto and Respivant, outside the submitted work; payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events from Boehringer Ingelheim, F. Hoffmann-La Roche and Novartis, outside the submitted work; support for attending meetings and/or travel received from Boehringer Ingelheim and F. Hoffmann-La Roche, outside the submitted work; participation on a data safety monitoring or advisory boards for Savara and Galapagos; and is chair of the Idiopathic Interstitial Pneumonia group of the European Respiratory Society, a member of the board of the Netherlands Respiratory Society, a member of the scientific advisory board of the European Idiopathic Pulmonary Fibrosis and Related Disorders Federation, chair of the educational committee of the European Reference Network for Rare Lung Diseases, and on the advisory board of the Dutch Lung Fibrosis and Sarcoidosis patient associations. Conflict of interest: M. Molina-Molina reports receiving support for the present manuscript from Boehringer Ingelheim; grants received from Roche and Boehringer Ingelheim, outside the submitted work; consulting fees received from Roche, Boehringer Ingelheim, Esteve-Teijin, Pfizer and Chiesi, outside the submitted work. Conflict of interest: O. Chassany reports receiving support for the present manuscript from Boehringer Ingelheim; and consulting fees received from IQVIA, Sanofi, Boiron and ViiV, outside the submitted work. Conflict of interest: J. Fox reports receiving support for the present manuscript from Boehringer Ingelheim. Conflict of interest: L. Galvin reports receiving support for the present manuscript from Boehringer Ingelheim, and is an unpaid director and committee member of the Irish Lung Fibrosis Association, outside the submitted work. Conflict of interest: K. Geissler reports receiving support for the present manuscript from Boehringer Ingelheim. Conflict of interest: K.M. Hammitt reports receiving support for the present manuscript from Boehringer Ingelheim. Novartis paid the Sjogren's Foundation for her to serve on its advisory board although her position does not involve anything pulmonary related. Conflict of interest: M. Kreuter reports receiving support for the present manuscript from Boehringer Ingelheim, and receiving grants or contracts from Boehringer Ingelheim and Roche, outside the submitted work; consulting fees received from Boehringer Ingelheim, Galapagos and Roche, outside the submitted work; payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events received from Boehringer, Galapagos and Roche, outside the submitted work; and participation on data safety monitoring or advisory boards for Boehringer Ingelheim, Galapagos and Roche, outside the submitted work. Conflict of interest: T. Moua reports receiving support for the present manuscript from Boehringer Ingelheim; and grants or contracts received from Boehringer Ingelheim, outside the submitted work. He is an associate editor of this journal. Conflict of interest: E.C. O'Brien reports receiving support for the present manuscript from Boehringer Ingelheim, and consulting fees received from Boehringer Ingelheim outside the submitted work. Conflict of interest: A.F. Slagle reports receiving support for the present manuscript from Boehringer Ingelheim, and receiving consulting fees from Boehringer Ingelheim and IQVIA outside the submitted work. Conflict of interest: A. Krasnow reports receiving support for the present manuscript from Boehringer Ingelheim. The author is a current employee of IQVIA. Conflict of interest: M. Reaney reports receiving support for the present manuscript from Boehringer Ingelheim. The author is a current employee of IQVIA. Conflict of interest: M. Baldwin reports receiving support for the present manuscript from Boehringer Ingelheim. The author is a current employee of Boehringer Ingelheim. Conflict of interest: N. Male reports receiving support for the present manuscript from Boehringer Ingelheim. The author is a current employee of Boehringer Ingelheim. Conflict of interest: K.B. Rohr reports receiving support for the present manuscript from Boehringer Ingelheim. Conflict of interest: J. Swigris reports receiving support for the present manuscript from Boehringer Ingelheim. The author is a current employee of Boehringer Ingelheim. He has received consulting fees from Boehringer Ingelheim, Bristol Myers Squibb, Genentech and Live Fully, outside the submitted work. Conflict of interest: K. Antoniou received support for the present manuscript from Boehringer Ingelheim; payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events from Boehringer Ingelheim and Roche, outside the submitted work; support for attending meetings and/or travel received from Boehringer Ingelheim and Roche, outside the submitted work; and is the ERS Assembly 12 Head (unpaid)., (Copyright ©The authors 2022.)

Background: Pulmonary disease is a potentially serious yet underdiagnosed complication of Sjögren's syndrome, the second most common autoimmune rheumatic disease. Approximately 16% of patients with Sjögren's demonstrate pulmonary involvement with higher mortality and lower quality of life., Research Question: Clinical practice guidelines for pulmonary manifestations of Sjögren's were developed by the Sjögren's Foundation after identifying a critical need for early diagnosis and improved quality and consistency of care., Study Design and Methods: A rigorous and transparent methodology was followed according to American College of Rheumatology guidelines. The Pulmonary Topic Review Group (TRG) developed clinical questions in the PICO (Patient, Intervention, Comparison, Outcome) format and selected literature search parameters. Each article was reviewed by a minimum of two TRG members for eligibility and assessment of quality of evidence and strength of recommendation. Guidelines were then drafted based on available evidence, expert opinion, and clinical importance. Draft recommendations with a clinical rationale and data extraction tables were submitted to a Consensus Expert Panel for consideration and approval, with at least 75% agreement required for individual recommendations to be included in the final version., Results: The literature search revealed 1,192 articles, of which 150 qualified for consideration in guideline development. Of the original 85 PICO questions posed by the TRG, 52 recommendations were generated. These were then reviewed by the Consensus Expert Panel and 52 recommendations were finalized, with a mean agreement of 97.71% (range, 79%-100%). The recommendations span topics of evaluating Sjögren's patients for pulmonary manifestations and assessing, managing, and treating upper and lower airway disease, interstitial lung disease, and lymphoproliferative disease., Interpretation: Clinical practice guidelines for pulmonary manifestations in Sjögren's will improve early identification, evaluation, and uniformity of care by primary care physicians, rheumatologists, and pulmonologists. Additionally, opportunities for future research are identified., (Copyright © 2020 The Authors. Published by Elsevier Inc. All rights reserved.)

This article presents an Executive Summary of the conclusions and recommendations of the 10-chapter TFOS DEWS II report. The entire TFOS DEWS II report was published in the July 2017 issue of The Ocular Surface. A downloadable version of the document and additional material, including videos of diagnostic and management techniques, are available on the TFOS website: www.TearFilm.org., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Context: The severity of Sjögren's syndrome has been evaluated using a wide variety of clinical measures and patient-reported outcomes (PROs). This may contribute to the lack of clarity concerning the burden of Sjögren's from the patient perspective., Objective: To perform a comprehensive peer-reviewed literature analysis of the patient aspects of Sjögren's, focusing on PROs, to investigate the complexity underlying the evaluation of the syndrome and to elucidate the discordance between the different measures., Methods: We searched Embase for articles published between January 2005 and September 2015. Research articles, clinical and diagnostic reviews, and validation studies with a focus on patient aspects of Sjögren's were selected as the primary information source., Results: 157 articles met the eligibility criteria. A wide variety of assessment measures used to evaluate glandular, extraglandular and functional domains were observed. Many different, non-validated Visual Analogue Scales, with a wide range of anchor words, were used in the quantification of Sjögren's disease burden, impeding comparisons between studies. Relatively few clinical trials of drug therapies used validated scales: European League Against Rheumatism Sjögren's Syndrome Patient Reported Index was used most often for symptom assessment and 36 Item Short Form Survey for quality of life (QoL)., Conclusion: A wide range and diversity of measures are used to evaluate the patient burden of Sjögren's; most are not validated for use in this disease. PRO endpoints, validated specifically in Sjögren's, that demonstrate improvement are needed. These measures should focus on QoL aspects important to patients and will most likely involve gauging change in function rather than patient-reported symptoms., Competing Interests: Competing interests: KMH is Vice President of Medical and Scientific Affairs of the Sjögren’s Syndrome Foundation. ANN and JAB are employees and stockholders of Eli Lilly and Company. RWMB is employed by Excerpta Medica B.V.

Objective: The Sjögren's Syndrome Foundation clinical practice guidelines (CPGs) are designed to improve quality and consistency of care in Sjögren's syndrome by offering recommendations for management., Methods: Management questions for the systemic manifestations of Sjögren's syndrome were posed by the CPG committee with input from patients and rheumatologists. Clinical questions were assigned to a topic review group that performed systematic reviews and data extraction and drafted guidelines. Quality of evidence and strength of recommendation were rated using the American Society of Clinical Oncology's modification of the Grading of Recommendations Assessment, Development, and Evaluation. Guideline recommendations were reviewed by a consensus expert panel (CEP) composed of 30-40 clinicians from academia and community practices, as well as registered nurses and patients, using a modified Delphi process. A CEP agreement level of 75% was set as a minimum for adoption of a guideline recommendation., Results: Consensus was achieved for 19 recommendations; for 11 additional modules, available data were insufficient to allow a recommendation to be formulated. Of the 19 recommendations, 15 required 1 Delphi round, 2 required 2 rounds, and 2 required 3 rounds., Conclusion: Key recommendations include a decision tree for the use of oral disease-modifying antirheumatic drugs for inflammatory musculoskeletal pain, use of self-care measures and advice regarding exercise to reduce fatigue, and the use of rituximab in selected clinical settings for oral and ocular dryness and for certain extraglandular manifestations, including vasculitis, severe parotid swelling, inflammatory arthritis, pulmonary disease, and mononeuritis multiplex. The CPG committee strongly discouraged the use of tumor necrosis factor inhibitors for sicca symptoms and for the majority of clinical contexts in primary Sjögren's syndrome., (© 2016, American College of Rheumatology.)

Sjögren's disease is associated with a high burden of illness, diminished quality of life, and increased health care costs. The Sjögren's Syndrome Foundation developed the first US clinical practice guidelines for management of the oral, ocular, and rheumatologic or systemic manifestations. Guideline recommendations were reviewed by a consensus expert panel using a modified Delphi process. This initiative should improve the quality and consistency of care for Sjögren's disease in the United States, guide insurance reimbursement, and define areas for future study. Guidelines will be periodically reviewed and revised as new information becomes available., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: Salivary dysfunction in Sjögren disease can lead to serious and costly oral health complications. Clinical practice guidelines for caries prevention in Sjögren disease were developed to improve quality and consistency of care., Methods: A national panel of experts devised clinical questions in a Population, Intervention, Comparison, Outcomes format and included use of fluoride, salivary stimulants, antimicrobial agents, and nonfluoride remineralizing agents. The panel conducted a systematic search of the literature according to pre-established parameters. At least 2 members extracted the data, and the panel rated the strength of the recommendations by using a variation of grading of recommendations, assessment, development, and evaluation. After a Delphi consensus panel was conducted, the experts finalized the recommendations, with a minimum of 75% agreement required., Results: Final recommendations for patients with Sjögren disease with dry mouth were as follows: topical fluoride should be used in all patients (strong); although no study results link improved salivary flow to caries prevention, the oral health community generally accepts that increasing saliva may contribute to decreased caries incidence, so increasing saliva through gustatory, masticatory, or pharmaceutical stimulation may be considered (weak); chlorhexidine administered as varnish, gel, or rinse may be considered (weak); and nonfluoride remineralizing agents may be considered as an adjunct therapy (moderate)., Conclusions and Practical Implications: The incidence of caries in patients with Sjögren disease can be reduced with the use of topical fluoride and other preventive strategies., (Copyright © 2016 American Dental Association. Published by Elsevier Inc. All rights reserved.)