Identification of outcome domains in primary Sjögren's disease: A scoping review by the OMERACT Sjögren disease working group.

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.

Lung involvement in ANCA-associated vasculitis.

Lung involvement is one of the most common clinical features in ANCA-associated vasculitides (AAV), including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). In this review, we detail the five main presentations of pulmonary involvement in AAV: necrotizing granulomatous inflammation, tracheobronchial inflammation, pulmonary capillaritis, interstitial lung disease (ILD) and asthma with their clinical, radiological and therapeutic characteristics. The prevalence of these manifestations is variable according to the subtype of AAV, necrotizing granulomatous inflammation and tracheobronchial inflammation being defining features of GPA whereas ILD is primarily seen in patients with MPA, especially in association with ANCA directed against myeloperoxydase (MPO-ANCA), and asthma is characteristic of EGPA. Despite recent progresses in the diagnosis and management of these conditions, several questions remain and are discussed here, including local treatments for subglottic stenosis, the uncertain efficacy of plasma exchanges for alveolar hemorrhage, the potential role of antifibrotic agents in ILD associated with MPA, and the use of novel anti-IL-5 strategies in EGPA.

Incidence and Mortality of Physician-Diagnosed Primary Sjögren Syndrome: Time Trends Over a 40-Year Period in a Population-Based US Cohort.

OBJECTIVE: To estimate the incidence and mortality rates, and their evolution over time, of physician-diagnosed primary Sjögren syndrome (pSS) in residents of Olmsted County, Minnesota. PATIENTS AND METHODS: Medical records of patients with a diagnosis or suspicion of SS in Olmsted County from January 1, 2006, through December 31, 2015, were reviewed to identify incident cases of pSS (defined by physician diagnosis). These cases were combined with those from a 1976 through 2005 incident cohort (n=111) from the same population. Incidence rates were age and sex adjusted to the 2010 US white population. Survival rates were compared with the expected rates in the population of Minnesota. RESULTS: With 61 incident cases of pSS diagnosed in Olmsted County from 2006 through 2015, the total cohort included 172 patients with incident pSS from 1976 through 2015. Of the 172 patients, 151 (88%) were women and 161 (94%) were white, with a mean ± SD age at diagnosis of 58.3±16.7 years. The average age- and sex-adjusted annual incidence for 2006 through 2015 was 5.9 per 100,000 population (95% CI, 4.4-7.4 per 100,000 population), and the overall incidence for the entire period was 5.8 per 100,000 (95% CI, 4.9-6.6 per 100,000). The incidence increased with calendar time over the 40-year period (P=.005). There was no difference in mortality in the pSS cohort compared with expected (standardized mortality ratio, 1.15; 95% CI, 0.86-1.50). CONCLUSION: The average annual incidence of pSS in this population-based cohort was 5.8 per 100,000, with a progressive increase over the 40 years of the study. Overall survival of patients with pSS was not different from that of the general population.

Prevalence of Primary Sjögren's Syndrome in a US Population-Based Cohort.

OBJECTIVE: To report the point prevalence of primary Sjögren's syndrome (SS) in the first US population-based study. METHODS: Cases of all potential primary SS patients living in Olmsted County, Minnesota, on January 1, 2015, were retrieved using Rochester Epidemiology Project resources, and ascertained by manual medical records review. Primary SS cases were defined according to physician diagnosis. The use of diagnostic tests was assessed and the performance of classification criteria was evaluated. The number of prevalent cases in 2015 was also projected based on 1976-2005 incidence data from the same source population. RESULTS: A total of 106 patients with primary SS were included in the study: 86% were female, with a mean ± SD age of 64.6 ± 15.2 years, and a mean ± SD disease duration of 10.5 ± 8.4 years. A majority were anti-SSA positive (75%) and/or anti-SSB positive (58%), but only 22% met American-European Consensus Group or American College of Rheumatology criteria, because the other tests required for disease classification (ocular dryness objective assessment, salivary gland functional or morphologic tests, or salivary gland biopsy) were rarely performed in clinical practice. According to the physician diagnosis, the age- and sex-adjusted prevalence of primary SS was 10.3 per 10,000 inhabitants, but according to classification criteria, this prevalence was only 2.2 per 10,000. The analysis based on previous incidence data projected a similar 2015 prevalence rate of 11.0 per 10,000. CONCLUSION: The prevalence of primary SS in this geographically well-defined population was estimated to be between 2 and 10 per 10,000 inhabitants. Physicians rarely used tests included in the classification criteria to diagnose the disease in this community setting.