作者
Martin Aringer,Karen H. Costenbader,David Daikh,Ralph Brinks,Marta Mosca,Rosalind Ramsey‐Goldman,Sofía Ramiro,David Wofsy,Dimitrios T. Boumpas,Diane L. Kamen,David Jayne,Ricard Cervera,N. Costedoat‐Chalumeau,Betty Diamond,Dafna D. Gladman,Bevra H. Hahn,Falk Hiepe,Søren Jacobsen,Dinesh Khanna,Kirsten Lerstrøm,Elena Massarotti,Joseph M. McCune,Guillermo Ruíz-Irastorza,Jorge Sánchez‐Guerrero,Matthias Schneider,Murray B. Urowitz,George Βertsias,Bimba F. Hoyer,Nicolai Leuchten,Chiara Tani,Sara K. Tedeschi,Zahi Touma,Gabriela Schmajuk,Branimir Anić,Florence Assan,Tak Mao Chan,Ann E. Clarke,Mary K. Crow,László Czirják,Andrea Doria,W. Graninger,Bernadett Halda-Kiss,Sarfaraz Hasni,Peter Izmirly,Michelle Jung,Gabór Kumánovics,Xavier Mariette,Ivan Padjen,José María Pego‐Reigosa,Juanita Romero‐Díaz,I Fernández,Raphaèle Séror,Georg Stummvoll,Yoshiya Tanaka,Maria G. Tektonidou,Carlos Vasconcelos,Edward M Vital,Daniel J. Wallace,Şule Yavuz,Pier Luigi Meroni,Marvin J. Fritzler,Ray Naden,Thomas Dörner,Sindhu R. Johnson
摘要
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 four phases. (1) Evaluation of antinuclear 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 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 with 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 seven clinical (constitutional, haematological, neuropsychiatric, mucocutaneous, serosal, musculoskeletal, renal) and three 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 with 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 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.