Performance of the 2019 EULAR/ACR classification criteria for systemic lupus erythematosus in early disease, across sexes and ethnicities.

Funder: American College of Rheumatology Research and Education Foundation; FundRef: http://dx.doi.org/10.13039/100000960Funder: National Institute of Arthritis and Musculoskeletal and Skin Diseases; FundRef: http://dx.doi.org/10.13039/100000069Funder: European League Against Rheumatism; FundRef: http://dx.doi.org/10.13039/501100008741OBJECTIVES: The European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) 2019 Classification Criteria for systemic lupus erythematosus (SLE) have been validated with high sensitivity and specificity. We evaluated the performance of the new criteria with regard to disease duration, sex and race/ethnicity, and compared its performance against the Systemic Lupus International Collaborating Clinics (SLICC) 2012 and ACR 1982/1997 criteria. METHODS: Twenty-one SLE centres from 16 countries submitted SLE cases and mimicking controls to form the validation cohort. The sensitivity and specificity of the EULAR/ACR 2019, SLICC 2012 and ACR 1982/1997 criteria were evaluated. RESULTS: The cohort consisted of female (n=1098), male (n=172), Asian (n=118), black (n=68), Hispanic (n=124) and white (n=941) patients; with an SLE duration of 1 to <3 years (n=196) and ≥5 years (n=879). Among patients with 1 to <3 years disease duration, the EULAR/ACR criteria had better sensitivity than the ACR criteria (97% vs 81%). The EULAR/ACR criteria performed well in men (sensitivity 93%, specificity 96%) and women (sensitivity 97%, specificity 94%). Among women, the EULAR/ACR criteria had better sensitivity than the ACR criteria (97% vs 83%) and better specificity than the SLICC criteria (94% vs 82%). Among white patients, the EULAR/ACR criteria had better sensitivity than the ACR criteria (95% vs 83%) and better specificity than the SLICC criteria (94% vs 83%). The EULAR/ACR criteria performed well among black patients (sensitivity of 98%, specificity 100%), and had better sensitivity than the ACR criteria among Hispanic patients (100% vs 86%) and Asian patients (97% vs 77%). CONCLUSIONS: The EULAR/ACR 2019 criteria perform well among patients with early disease, men, women, white, black, Hispanic and Asian patients. These criteria have superior sensitivity than the ACR criteria and/or superior specificity than the SLICC criteria across many subgroups

European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) SLE classification criteria item performance.

BACKGROUND/OBJECTIVES: The European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) 2019 classification criteria for systemic lupus erythematosus system showed high specificity, while attaining also high sensitivity. We hereby analysed the performance of the individual criteria items and their contribution to the overall performance of the criteria. METHODS: We combined the EULAR/ACR derivation and validation cohorts for a total of 1197 systemic lupus erythematosus (SLE) and n=1074 non-SLE patients with a variety of conditions mimicking SLE, such as other autoimmune diseases, and calculated the sensitivity and specificity for antinuclear antibodies (ANA) and the 23 specific criteria items. We also tested performance omitting the EULAR/ACR criteria attribution rule, which defines that items are only counted if not more likely explained by a cause other than SLE. RESULTS: Positive ANA, the new entry criterion, was 99.5% sensitive, but only 19.4% specific, against a non-SLE population that included other inflammatory rheumatic, infectious, malignant and metabolic diseases. The specific criteria items were highly variable in sensitivity (from 0.42% for delirium and 1.84% for psychosis to 75.6% for antibodies to double-stranded DNA), but their specificity was uniformly high, with low C3 or C4 (83.0%) and leucopenia 80% for all items, explaining the higher overall specificity of the criteria set

Who's next? A new process for creating points systems for prioritising patients for elective health services

We describe a new process for creating points systems for prioritising patients for elective health services. Beginning in 2004, the authors were closely involved in a project to develop the process, initially for coronary artery bypass graft surgery and then successively for other elective services. The project was led by New Zealand’s Ministry of Health in collaboration with the relevant clinical professional organisations. The objective was to overcome the limitations of earlier methodologies and to create points systems that are valid and reproducible and based on a consensus of clinical judgements. As the project progressed and the process was refined, other points systems were successively created (and clinically endorsed) for hip and knee replacements, varicose veins surgery, cataract surgery, gynaecology, plastic surgery, otorhinolaryngology, and heart valve surgery. Other points systems are planned for the future. Since 2008 the process has also been used in the public health systems of Canada’s western provinces. The process is explained in a step-by-step manner so that others are able to follow it to create their own points systems if desired

Who's next? A new process for creating points systems for prioritising patients for elective health services

We describe a new process for creating points systems for prioritising patients for elective health services. Beginning in 2004, the authors were closely involved in a project to develop the process, initially for coronary artery bypass graft surgery and then successively for other elective services. The project was led by New Zealand’s Ministry of Health in collaboration with the relevant clinical professional organisations. The objective was to overcome the limitations of earlier methodologies and to create points systems that are valid and reproducible and based on a consensus of clinical judgements. As the project progressed and the process was refined, other points systems were successively created (and clinically endorsed) for hip and knee replacements, varicose veins surgery, cataract surgery, gynaecology, plastic surgery, otorhinolaryngology, and heart valve surgery. Other points systems are planned for the future. Since 2008 the process has also been used in the public health systems of Canada’s western provinces. The process is explained in a step-by-step manner so that others are able to follow it to create their own points systems if desired

Clinical phenotypes of IgG4-related disease: an analysis of two international cross-sectional cohorts.

Objective IgG4-related disease (IgG4-RD) is a heterogeneous, multiorgan condition of unclear aetiology that can cause organ failure. Difficulty recognising IgG4-RD contributes to diagnostic delays. We sought to identify key IgG4-RD phenotypes. Methods We used two cross-sectional studies assembled by an international, multispecialty network of IgG4-RD specialists who submitted 765 cases to derive and replicate phenotypic groups. Phenotype groups of disease manifestations and key covariate distributions across the identified groups were measured using latent class analysis. Results In the derivation cohort (n=493), we identified four groups with distinct manifestations: Group 1 (31%), Pancreato-Hepato-Biliary disease; Group 2 (24%), Retroperitoneal Fibrosis and/or Aortitis; Group 3 (24%), Head and Neck-Limited disease and Group 4 (22%), classic Mikulicz syndrome with systemic involvement. We replicated the identification of four phenotype groups in the replication cohort. Compared with cases in Groups 1, 2 and 4, respectively, cases in Group 3 were more likely to be female (OR 11.60 (95% CI 5.39 to 24.98), 10.35 (95% CI 4.63 to 23.15) and 9.24 (95% CI 3.53 to 24.20)) and Asian (OR 6.68 (95% CI 2.82 to 15.79), 7.43 (95% CI 2.97 to 18.56) and 6.27 (95% CI 2.27 to 17.29)). Cases in Group 4 had a higher median serum IgG4 concentration (1170 mg/dL) compared with groups 1–3 (316, 178 and 445 mg/dL, respectively, p<0.001). Conclusion We identified four distinctive IgG4-RD phenotypes according to organ involvement. Being Asian or female may predispose individuals to head and neck-limited disease. These phenotypes serve as a framework for identifying IgG4-RD and studying its aetiology and optimal treatment.status: publishe

Independent Determinants of Maternal and Fetal Outcomes in a Sample of Pregnant Outpatients With Normal Blood Pressure, Chronic Hypertension, Gestational Hypertension, and Preeclampsia

The aim of this retrospective study was to evaluate the main independent prognostic factors of negative maternal and fetal outcomes in a relatively large sample of pregnant outpatients (N=906) who were normotensive or affected by chronic hypertension, gestational hypertension, or preeclampsia. Among the studied parameters, the ones significantly associated with negative maternal outcomes were a diagnosis of preeclampsia (vs other forms of hypertension or normotension) and higher serum uric acid level, while antihypertensive treatment, number of previous deliveries, and blood pressure (BP) control at deliveries seemed to be protective. Regarding negative fetal outcomes, the parameters significantly associated with a negative maternal outcome were a diagnosis of preeclampsia (vs other forms of hypertension or normotension) and mother pre-pregnancy body mass index, while antihypertensive treatment and BP control at delivery seemed to be protective. Specific patient characteristics should help to predict the risk of negative maternal and fetal outcomes

Development of new international antiphospholipid syndrome classification criteria phase I/II report : generation and reduction of candidate criteria

OBJECTIVES: An international multi-disciplinary initiative, jointly supported by American College of Rheumatology (ACR) and European League Against Rheumatism (EULAR), is underway to develop new rigorous classification criteria to identify patients with high likelihood of Antiphospholipid Syndrome (APS) for research purposes. We applied an evidence- and consensus-based approach to identify candidate criteria and develop a hierarchical organization of criteria within domains. METHODS: During Phase I, the APS classification criteria Steering Committee used systematic literature reviews and surveys of international APS physician scientists to generate a comprehensive list of items related to APS. In Phase II, we reviewed the literature, administered surveys, formed domain subcommittees, and used Delphi exercises and nominal group technique to reduce potential APS candidate criteria. Candidate criteria were hierarchically organized into clinical and laboratory domains. RESULTS: Phase I generated 152 candidate criteria, expanded to 261 items with the addition of subgroups and candidate criteria with potential negative weights. Using iterative item reduction techniques in Phase II, we initially reduced these items to 64 potential candidate criteria organized into ten clinical and laboratory domains. Subsequent item reduction methods resulted in 27 candidate criteria, hierarchically organized into six additive domains (laboratory, macrovascular, microvascular, obstetric, cardiac, and hematologic) for APS classification. CONCLUSION: Using data- and consensus-driven methodology, we identified twenty-seven APS candidate criteria in six clinical or laboratory domains. In the next phase, the proposed candidate criteria will be used for real-world case collection and further refined, organized, and weighted to determine an aggregate score and threshold for APS classification