Increased arterial stiffness and accelerated atherosclerosis in Takayasu arteritis

To develop new classification criteria for systemic lupus erythematosus (SLE) jointly supported by the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR). This international initiative had 4 phases: 1) Evaluation of anti-nuclear antibody (ANA) as an entry criterion through systematic review and meta-regression of the literature and criteria generation through an international Delphi exercise, an early patient cohort and a patient survey. 2) Criteria reduction by Delphi and nominal group technique (NGT) exercises. 3) Criteria definition and weighting based on criterion performance and on results of a multi-criteria decision analysis. 4) Refinement of weights and threshold scores in a new derivation cohort of 1001 subjects and validation compared to previous criteria in a new validation cohort of 1270 subjects. The 2019 EULAR/ACR classification criteria for SLE include positive ANA at least once as obligatory entry criterion; followed by additive weighted criteria grouped in 7 clinical (constitutional, hematologic, neuropsychiatric, mucocutaneous, serosal, musculoskeletal, renal) and 3 immunological (antiphospholipid antibodies, complement proteins, SLE-specific antibodies) domains, and weighted from 2 to 10. Patients accumulating ≥10 points are classified. In the validation cohort, the new criteria had a sensitivity of 96.1% and specificity of 93.4%, compared to 82.8% sensitivity and 93.4% specificity of the ACR 1997 and 96.7% sensitivity and 83.7% specificity of the Systemic Lupus International Collaborating Clinics (SLICC) 2012 criteria. These new classification criteria were developed using rigorous methodology with multidisciplinary and international input, and have excellent sensitivity and specificity. Use of ANA entry criterion, hierarchically clustered and weighted criteria reflect current thinking about SLE and provide an improved foundation for SLE research.

Takayasu arteritis, a rare granulomatous vasculitis affecting young people, is associated with considerable morbidity and premature mortality. In most patients the diagnosis is delayed until after the development of substantial arterial injury. Studies of noninvasive imaging techniques suggest that these approaches might facilitate earlier diagnosis and have a role in monitoring disease progress; however, they remain limited in their ability to accurately quantify inflammatory disease activity in the arterial wall. A lack of controlled clinical trial data complicates the choice of therapy for Takayasu arteritis, and clinical indices for monitoring disease activity are currently suboptimal. Increasing knowledge of the pathogenesis of the large vessel vasculitides might eventually lead to novel targeted therapies. Preliminary data from open-label trials of anti-tumor necrosis factor therapy are encouraging, but there is an urgent need for controlled clinical trials to establish optimum therapeutic approaches for this disease. These trials should include a prospective assessment of the use of noninvasive imaging modalities in the diagnosis and subsequent management of Takayasu arteritis.