Search

OBJECTIVE: Classification criteria for calcium pyrophosphate deposition disease (CPPD) will facilitate clinical research on this common crystalline arthritis. We report on the first two phases of a four-phase process for developing CPPD classification criteria. METHODS: CPPD classification criteria development is overseen by a 12-member Steering Committee. Item generation (Phase I) included a scoping literature review of five literature databases and contributions from a 35-member Combined Expert Committee and two Patient Research Partners. Item reduction and refinement (Phase II) involved a Combined Expert Committee meeting, discussions among Clinical, Imaging, and Laboratory Advisory Groups, and an item rating exercise to assess the influence of individual items toward classification. The Steering Committee reviewed the modal rating score for each item (range -3 [strongly pushes away from CPPD] to +3 [strongly pushes toward CPPD]) to determine items to retain for future phases of criteria development. RESULTS: Item generation yielded 420 items (312 from the literature, 108 from experts/patients). The Advisory Groups eliminated items they agreed were unlikely to distinguish between CPPD and other forms of arthritis, yielding 127 items for the item rating exercise. Fifty-six items, most of which had a modal rating of +/- 2 or 3, were retained for future phases. As numerous imaging items were rated +3, the Steering Committee recommended focusing on imaging of the knee, wrist, and one additional affected joint for calcification suggestive of CPP crystal deposition. CONCLUSION: A data- and expert-driven process is underway to develop CPPD classification criteria. Candidate items comprise clinical, imaging, and laboratory features.

European League Against Rheumatism and are jointly supporting multiphase development of systemic lupus erythematosus (SLE) classification criteria based on weighted criteria and a continuous probability scale. Prior steps included item generation, item reduction and hierarchical organisation of candidate criteria using an evidence-based approach. Our objectives were to determine relative weights using multicriteria decision analysis (MCDA) and to set a provisional threshold score for SLE classification. An SLE Expert Panel (8 European, 9 North American) submitted 164 real, unique cases with a wide range of SLE probability in a standardised format. Using the candidate criteria, experts scored and rank-ordered 20 representative cases. At an in-person meeting, experts reviewed inter-rater reliability of scoring, further refined criteria definitions and participated in an MCDA exercise. Based on expert consensus decisions on pairwise comparisons of criteria, 1000minds software calculated criteria weights and rank-ordered the remaining 144 cases based on their additive scores. The score of the lowest-ranked case for which complete expert consensus was achieved defined the provisional threshold classification score. Inter-rater reliability of scoring cases with the candidate criteria was good. MCDA involved 74 pairwise decisions and was repeated for the arthritis and mucocutaneous domains when the initial ranking of some cases did not match expert opinion. After criteria weights and additive scores were recalculated once, experts reached consensus for SLE classification for all cases scoring>83. Using an iterative process, the candidate criteria definitions were refined, preliminary weights were calculated and a provisional threshold score for SLE classification was determined.

Background:Classification criteria for calcium pyrophosphate deposition disease (CPPD) will facilitate clinical research on this common crystalline arthritis. ACR/EULAR are jointly sponsoring development of CPPD classification criteria using a multi-phase process.Objectives:To report preliminary results from the first two phases of a four-phase process for developing CPPD classification criteria.Methods:CPPD classification criteria development is overseen by a 12-member Steering Committee. Item generation (Phase I) included a scoping literature review of five literature databases and contributions from a 35-member Combined Expert Committee and two Patient Research Partners. Item reduction and refinement (Phase II) involved a Combined Expert Committee meeting, discussions among Clinical, Imaging, and Laboratory Advisory Groups, and an item rating exercise to assess the influence of individual items toward classification. The Steering Committee reviewed the modal rating score for each item (range -3 [strongly pushes away from CPPD] to +3 [strongly pushes toward CPPD]) to determine items to retain for future phases of criteria development.Results:Item generation yielded 420 items (312 from the literature, 108 from experts/patients). The Advisory Groups eliminated items they agreed were unlikely to distinguish between CPPD and other forms of arthritis, yielding 127 items for the item rating exercise. Fifty-six items, most of which had a modal rating of +/- 2 or 3, were retained for future phases (see Table 1). As numerous imaging items were rated +3, the Steering Committee recommended focusing on imaging of the knee, wrist, and one additional affected joint for calcification suggestive of CPP crystal deposition.Conclusion:The ACR/EULAR CPPD classification criteria working group has adopted both data- and expert-driven approaches, leading to 56 candidate items broadly categorized as clinical, imaging, and laboratory features. Remaining steps for criteria development include domain establishment, item weighting through a multi-criteria decision analysis exercise, threshold score determination, and criteria validation.Table 1.Categories of items retained for future phases of classification criteria developmentAge in decade at symptom onsetAcute inflammatory arthritis (e.g. knee, wrist, 1st MTP joint*)Recurrence and pattern of joint involvement (e.g. 1 self-limited episode, >1 self-limited episode)Physical findings (e.g. palpable subcutaneous tophus*, psoriasis*)Co-morbidities and family history (e.g. Gitelman disease, hemochromatosis, familial CPPD)Osteoarthritis location and features (e.g. 2nd or 3rd MCP joint, wrist)Synovial fluid findings (e.g. CPP crystals present, CPP crystals absent on 1 occasion* or 2 occasions*, monosodium urate crystals present*)Laboratory findings (e.g. hypomagnesemia, hyperparathyroidism, rheumatoid factor*, anti-CCP*)Plain radiograph: calcification in regions of fibro- or hyaline cartilage+Plain radiograph: calcification of the synovial membrane/capsule/tendon+Conventional CT: calcification in regions of fibro- or hyaline cartilage+Conventional CT: calcification of the synovial membrane/capsule/tendon+Ultrasound: CPP crystal deposition in fibro- or hyaline cartilage+Ultrasound: CPP crystal deposition in synovial membrane/capsule/tendons+Dual-energy CT: CPP crystal deposition in fibro- or hyaline cartilage+Dual-energy CT: CPP crystal deposition in synovial membrane/capsule/tendon+*Potential negative predictor +Assessed in the knee, wrist, and/or 1 additional affected jointDisclosure of Interests:Sara Tedeschi Consultant of: NGM Biopharmaceuticals, Tristan Pascart: None declared, Augustin Latourte Consultant of: Novartis, Cattleya Godsave: None declared, Burak Kundaki: None declared, Raymond Naden: None declared, William Taylor: None declared, Nicola Dalbeth Speakers bureau: Abbvie and Janssen, Consultant of: AstraZeneca, Dyve, Selecta, Horizon, Arthrosi, and Cello Health, Tuhina Neogi: None declared, Fernando Perez-Ruiz: None declared, Ann Rosenthal: None declared, Fabio Becce Consultant of: Horizon Therapeutics, Grant/research support from: Siemens Healthineers, Eliseo Pascual: None declared, Mariano Andrés: None declared, Thomas Bardin: None declared, Michael Doherty: None declared, Hang Korng Ea: None declared, Georgios Filippou: None declared, John FitzGerald: None declared, Marwin Gutierrez: None declared, Annamaria Iagnocco: None declared, Tim Jansen Speakers bureau: Abbvie, Amgen, BMS, Grunenthal, Olatec, Sanofi Genzyme, Consultant of: Abbvie, Amgen, BMS, Grunenthal, Olatec, Sanofi Genzyme, Minna Kohler Speakers bureau: Lilly, Consultant of: Novartis, Frederic Lioté: None declared, Mark Matza: None declared, Geraldine McCarthy Consultant of: PK Med, Roberta Ramonda: None declared, Anthony Reginato: None declared, Pascal Richette: None declared, Jasvinder Singh Speakers bureau: Simply Speaking, Consultant of: Crealta/Horizon, Medisys, Fidia, UBM LLC, Trio health, Medscape, WebMD, Adept Field Solutions, Clinical Care options, Clearview healthcare partners, Putnam associates, Focus forward, Navigant consulting, Spherix, Practice Point communications, Francisca Sivera: None declared, Alexander So: None declared, Lisa Stamp: None declared, Janeth Yinh: None declared, Chio Yokose: None declared, Robert Terkeltaub Consultant of: Sobi, Horizon Therapeutics, Astra-Zeneca, Selecta, Grant/research support from: Astra-Zeneca, Hyon Choi: None declared, Abhishek Abhishek Consultant of: NGM Biopharmaceuticals.

Background Chronic nonbacterial osteomyelitis (CNO) is a severe and occult autoinflammatory bone disease of unknown cause. Early diagnosis is challenging, and CNO may debilitate affected children when left untreated. Currently, evidence-based and validated diagnostic and classification criteria for CRMO/CNO are lacking. The insidious disease course, increasing disease incidence, and significant delay in diagnosis highlight the need for the development of classification criteria that leads to more precise and early selection of patients for clinical trials1,2. Objectives To identify candidate items towards developing classification criteria for CNO using anonymous survey and nominal group technique. Methods An international collaborative effort was formed within the pediatric and adult rheumatology communities to conduct the following phases: 1) to generate candidate criteria items by a Delphi survey among international rheumatologists; 2) to reduce candidate criteria items through consensus processes involving physicians managing CNO and patients or caregivers of children with CNO. This study was approved by Seattle Children’s Hospital Institutional Review Board. Results In Phase 1, 259 pediatric rheumatologists (30%, N=865) participated in an online questionnaire about features most relevant to the classification of CNO. Of those, 77 (30%) practiced in Europe, 132 (51%) in North America, and 50 (19%) in other continents. A total of 138 (53%) responders had >10 years of practicing experience and 108 (42%) had managed >10 CNO patients. There were 33 candidate criteria items initially identified. In Phase 2, candidate items were presented to 39 rheumatologists and 7 parents and items were refined or eliminated through item reduction techniques. Seventy-seven (94%, N=82) workgroup members then participated in a second survey to rank the remaining items by their distinguishing power of CNO from mimicking conditions. Figure 1 shows the mean score for the remaining 31 candidate criteria. Multifocal lesions, ruling out malignancy and infection and typical location on imaging had the greatest means. CRP and/or ESR greater than 3x the normal upper limit had the greatest negative means. Discriminatory Score +3/-3 (increases/decreases the likelihood of CRMO the most) +2/-2 (increases/decreases the likelihood of CRMO moderately) +1/-1 (increases/decreases the likelihood of CRMO slightly) 0 (no difference) Conclusion Through surveys and consensus technique, candidate items towards developing classification criteria for CNO were identified. This list of items will guide the design of a feasible patient data collection form towards weighting of each item in the classification criteria. References [1] Roderick MR, Shah R, Rogers V, Finn A, Ramanan AV. Chronic recurrent multifocal osteomyelitis (CRMO)–advancing the diagnosis. Pediatric Rheumatology. 2016 Dec;14(1):47. [2] Jansson A, Renner ED, Ramser J, Mayer A, Haban M, Meindl A, et al. Classification of non-bacterial osteitis: retrospective study of clinical, immunological and genetic aspects in 89 patients. Rheumatology. 2006 Jun 17;46(1):154-60. Acknowledgement CNO/CRMO Work Group, Childhood Arthritis and Rheumatology Research Alliance Disclosure of Interests Melissa Oliver: None declared, Eveline Wu: None declared, Raymond Naden Speakers bureau: Was a speaker at conferences paid by pharmaceutical companies several times in the past, but not in the last 7 years., Matthew Hollander: None declared, Polly Ferguson: None declared, Fatma Dedeoglu Consultant for: Attended a scientific meeting for Novartis in 2017. Overall monetary amount was less than $5000., Seza Ozen Consultant for: Seza Ozen is receiving consultancy fees from Novartis, Speakers bureau: Roche, Yongdong Zhao Grant/research support from: I have grant support from Bristol-Myer Squibb

Background:An international multi-disciplinary effort is underway to develop rigorous, new, consensus- and evidence-based classification criteria for Antiphospholipid Syndrome (APS). The methodological approach includes four phases; we have previously presented phases I and II (item generation and reduction), which resulted in 27 candidate criteria1organized in laboratory and clinical domains.Objectives:Phase III (item weighting/threshold identification) is currently underway; here we report initial Phase III case collection results.Methods:We used REDCap, a secure data system, for Phase III international case collection. The candidate criteria of 27 items at the end of Phase II was represented in a standardized case collection form. We asked international physicians interested in APS to provide and rate cases using a Likert scale (+3 to -3: highly likely to highly unlikely to be APS). Cases with higher scores (+2 or +3) were categorized as “highly likely” APS, whereas lower scores (+1 to -3) were categorized as “equivocal or unlikely” APS.Results:We collected 314 potential APS cases (mean age 43.8±14.4 years; 79% female; and 77% white) between 6/2019-8/2019 from 17 sites in Europe (47%), North America (41%), and South America (11%). Majority of cases were potential primary APS (64%); 137 were rated as “highly likely” and 177 as “equivocal or unlikely” APS. Lupus anticoagulant, antiβ2glycoprotein-I antibody IgG/M, anticardiolipin antibody IgG, arterial thrombosis, venous thromboembolism, microvascular disease, fetal loss between 16-34 weeks, severe preeclampsia, severe placental insufficiency, cardiac valve disease, and low platelet count occurred with higher frequency in the APS cases categorized as “highly likely” (Table).Conclusion:International collection of cases spanning the spectrum of “highly likely” to “equivocal or unlikely” APS provide “real world” assessment of patients being referred for APS evaluation. In next steps, proposed candidate criteria will be further refined, organized, and weighted, and a preliminary threshold for APS classification will be determined.References:[1]Barbhaiya M et al. Phase II Results [abstract]. Arthritis Rheumatol. 2019;71 (suppl 10).Table.Characteristics of Potential APS Cases Categorized by Physician AssessmentCandidate Relative Criteria, n (%)Total(n=314)Highly Likely APS(n=137)Equivocal or Unlikely APS(n=177)LABORATORYLupus Anticoagulant Test208 (66)117 (85)91 (51)Anticardiolipin Antibody• IgG170 (54)90 (66)80 (45)• IgM138 (44)61 (45)77 (44)Antiβ2glycoprotein-I Antibody• IgG99 (32)66 (48)33 (19)• IgM79 (25)47 (34)32 (18)CLINICALMacrovascular• Superficial Vein Thrombosis15 (5)4 (3)11 (6)• Venous Thromboembolism95 (30)63 (46)32 (18)• Transient Ischemic Attack27 (9)13 (10)14 (8)• Arterial Thrombosis82 (26)58 (42)24 (14)Microvascular*86 (27)62 (45)24 (14)Obstetric**• Embryonic Loss (85 (45)34 (42)51 (48)• Fetal Loss (10w–20 (11)9 (11)11 (10)• Fetal Loss (16w–34w)42 (22)32 (40)19 (18)• Severe Preeclampsia (27 (14)18 (22)9 (8)• Severe Placental Insufficiency (22 (12)13 (16)9 (8)Cardiac Valve Disease***32 (10)21 (15)11 (6)Thrombocytopenia 72 (23)43 (31)29 (16)*Livedo racemosa, livedoid vasculopathy, adrenal hemorrhage, acute ischemic enceophalopathy, cardiac microvascular disease, pulmonary hemorrhage, acute and/or chronic antiphospholipid-related nephropathy; **Total number of patients ever pregnant: 188 (highly likely APS: 81; unlikely APS: 107) ***Valve thickening and/or vegetation.Acknowledgments:The project is supported by ACR/EULARDisclosure of Interests:Medha Barbhaiya: None declared, Doruk Erkan: None declared, Yasaman Ahmadzadeh: None declared, Karen Costenbader Grant/research support from: Merck, GSK, Consultant of: Merck, GSK, Lily, Astra Zeneca, Janssen, Raymond Naden: None declared, Stéphane Zuily: None declared

Background:EULAR/ACR 2019 SLE Classification Criteria were validated in an international cohort.Objectives:To evaluate performance characteristics of SLE classification systems in sex, race/ethnicity, and disease duration subsets.Methods:Sensitivity and specificity of the EULAR/ACR 2019, SLICC 2012 and ACR 1982/1997 criteria were evaluated in the validation cohort.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; and patients with an SLE duration of 1-3 years (n=196), 3-5 years (n=157), and ≥5 years (n=879). Among patients with 1-3 years disease duration, the EULAR/ACR criteria had better sensitivity than the ACR criteria (97% (95%CI 92-99%) vs 81% (95%CI 72-88%). The new criteria performed well in men (sensitivity 93%, specificity 96%) and women (sensitivity 97%, specificity 94%). The new criteria had better sensitivity than the ACR criteria in White (95% vs 83%), Hispanic (100% vs 86%) and Asian patients (97% vs 77%).Conclusion:The EULAR/ACR 2019 criteria perform well in patients with early disease, and across sexes and ethnicities.Disclosure of Interests:Sindhu Johnson Grant/research support from: Boehringer Ingelheim, Corbus Pharmaceuticals, GlaxoSmithKline, Roche, Merck, Bayer, Consultant of: Boehringer Ingelheim, Ikaria, Ralph Brinks: None declared, Karen Costenbader Grant/research support from: Merck, Consultant of: Astra-Zeneca, David Daikh: None declared, Marta Mosca: None declared, Rosalind Ramsey-Goldman: None declared, Josef S. Smolen Grant/research support from: AbbVie, Eli Lilly, Janssen, Merck Sharp & Dohme, Pfizer, Roche – grant/research support, Consultant of: AbbVie, Amgen Inc., AstraZeneca, Astro, Celgene Corporation, Celtrion, Eli Lilly, Glaxo, ILTOO, Janssen, Medimmune, Merck Sharp & Dohme, Novartis, Pfizer, Roche, Samsung, Sanofi, UCB – consultant, Speakers bureau: AbbVie, Amgen Inc., AstraZeneca, Astro, Celgene Corporation, Celtrion, Eli Lilly, Glaxo, ILTOO, Janssen, Medimmune, Merck Sharp & Dohme, Novartis, Pfizer, Roche, Samsung, Sanofi, UCB – speaker, David Wofsy: None declared, Dimitrios Boumpas Grant/research support from: Unrestricted grant support from various pharmaceutical companies, Diane L Kamen Consultant of: Consulted on SLE survey development for Lilly and consulted on SLE trial protocol development for EMD Serono in 2019, David Jayne Grant/research support from: ChemoCentryx, GSK, Roche/Genentech, Sanofi-Genzyme, Consultant of: Astra-Zeneca, ChemoCentryx, GSK, InflaRx, Takeda, Insmed, Chugai, Boehringer-Ingelheim, Ricard Cervera: None declared, Nathalie Costedoat-Chalumeau Grant/research support from: UCB to my institution, Betty Diamond: None declared, Dafna D Gladman Grant/research support from: AbbVie, Amgen Inc., BMS, Celgene Corporation, Janssen, Novartis, Pfizer, UCB – grant/research support, Consultant of: AbbVie, Amgen Inc., BMS, Celgene Corporation, Janssen, Novartis, Pfizer, UCB – consultant, Bevra H. Hahn Grant/research support from: Janssen Research & Development, LLC, Falk Hiepe: None declared, Soren Jacobsen: None declared, Dinesh Khanna Shareholder of: Eicos Sciences, Inc./Civi Biopharma, Inc., Grant/research support from: Dr Khanna was supported by NIH/NIAMS K24AR063120, Consultant of: Acceleron, Actelion, Bayer, Boehringer Ingelheim, Bristol-Myers Squibb, Corbus Pharmaceuticals, Horizon Therapeutic, Galapagos, Roche/Genentech, GlaxoSmithKline, Mitsubishi Tanabe, Sanofi-Aventis/Genzyme, UCB, Kirsten Lerstrom: None declared, Elena Massarotti: None declared, William Joseph McCune: None declared, Guillermo Ruiz-Irastorza: None declared, Jorge Sanchez-Guerrero: None declared, Matthias Schneider: None declared, Murray B Urowitz: None declared, George Bertsias Grant/research support from: GSK, Consultant of: Novartis, Bimba F. Hoyer: None declared, Nicolai Leuchten: None declared, Chiara Tani: None declared, Sara Tedeschi: None declared, Zahi Touma: None declared, Gabriela Schmajuk Grant/research support from: Pfizer, Branimir Anic: None declared, Florence Assan: None declared, Tak Chan: None declared, Ann E Clarke: None declared, Mary K. Crow: None declared, László Czirják Consultant of: Actelion, BI, Roche-Genentech, Lilly, Medac, Novartis, Pfizer, Bayer AG, Andrea Doria Consultant of: GSK, Pfizer, Abbvie, Novartis, Ely Lilly, Speakers bureau: UCB pharma, GSK, Pfizer, Janssen, Abbvie, Novartis, Ely Lilly, BMS, Winfried Graninger: None declared, Bernadett Halda-Kiss: None declared, Sarfaraz Hasni: None declared, Peter Izmirly: None declared, Michelle Jung: None declared, Gabor Kumanovics Consultant of: Boehringer, Teva, Speakers bureau: Roche, Lilly, Novartis, Xavier Mariette: None declared, Ivan Padjen: None declared, Jose M Pego-Reigosa: None declared, Juanita Romero-Diaz Consultant of: Biogen, Iñigo Rua-Figueroa: None declared, Raphaèle Seror Consultant of: BMS, Medimmune, Novartis, Pfizer, GSK, Lilly, Georg Stummvoll: None declared, Yoshiya Tanaka Grant/research support from: Asahi-kasei, Astellas, Mitsubishi-Tanabe, Chugai, Takeda, Sanofi, Bristol-Myers, UCB, Daiichi-Sankyo, Eisai, Pfizer, and Ono, Consultant of: Abbvie, Astellas, Bristol-Myers Squibb, Eli Lilly, Pfizer, Speakers bureau: Daiichi-Sankyo, Astellas, Chugai, Eli Lilly, Pfizer, AbbVie, YL Biologics, Bristol-Myers, Takeda, Mitsubishi-Tanabe, Novartis, Eisai, Janssen, Sanofi, UCB, and Teijin, Maria Tektonidou Grant/research support from: AbbVie, MSD, Novartis and Pfizer, Consultant of: AbbVie, MSD, Novartis and Pfizer, Carlos Vasconcelos: None declared, Edward Vital Grant/research support from: AstraZeneca, Roche/Genentech, and Sandoz, Consultant of: AstraZeneca, GSK, Roche/Genentech, and Sandoz, Speakers bureau: Becton Dickinson and GSK, Daniel J Wallace: None declared, Sule Yavuz: None declared, Pier Luigi Meroni: None declared, Marvin Fritzler: None declared, Raymond Naden: None declared, Thomas Dörner Grant/research support from: Janssen, Novartis, Roche, UCB, Consultant of: Abbvie, Celgene, Eli Lilly, Roche, Janssen, EMD, Speakers bureau: Eli Lilly, Roche, Samsung, Janssen, Martin Aringer Consultant of: Boehringer Ingelheim, Roche, Speakers bureau: Boehringer Ingelheim, Roche

Objective Existing criteria for the classification of gout have suboptimal sensitivity and/or specificity, and were developed at a time when advanced imaging was not available. The current effort was undertaken to develop new classification criteria for gout. Methods An international group of investigators, supported by the American College of Rheumatology and the European League Against Rheumatism, conducted a systematic review of the literature on advanced imaging of gout, a diagnostic study in which the presence of monosodium urate monohydrate (MSU) crystals in synovial fluid or tophus was the gold standard, a ranking exercise of paper patient cases, and a multicriterion decision analysis exercise. These data formed the basis for developing the classification criteria, which were tested in an independent data set. Results The entry criterion for the new classification criteria requires the occurrence of at least 1 episode of peripheral joint or bursal swelling, pain, or tenderness. The presence of MSU crystals in a symptomatic joint/bursa (i.e., synovial fluid) or in a tophus is a sufficient criterion for classification of the subject as having gout, and does not require further scoring. The domains of the new classification criteria include clinical (pattern of joint/bursa involvement, characteristics and time course of symptomatic episodes), laboratory (serum urate, MSU-negative synovial fluid aspirate), and imaging (double-contour sign on ultrasound or urate on dual-energy computed tomography, radiographic gout-related erosion). The sensitivity and specificity of the criteria are high (92% and 89%, respectively). Conclusion The new classification criteria, developed using a data-driven and decision analytic approach, have excellent performance characteristics and incorporate current state-of-the-art evidence regarding gout.

OBJECTIVE: To define candidate criteria within multiphase development of systemic lupus erythematosus (SLE) classification criteria, jointly supported by the American College of Rheumatology and the European League Against Rheumatism. Prior steps included item generation and reduction by Delphi exercise, further narrowed to 21 items in a nominal group technique exercise. Our objectives were to apply an evidence-based approach to the 21 candidate criteria, and to develop hierarchical organization of criteria within domains. METHODS: A literature review identified the sensitivity and specificity of the 21 candidate criteria. Data on the performance of antinuclear antibody (ANA) as an entry criterion and operating characteristics of the candidate criteria in early SLE patients were evaluated. Candidate criteria were hierarchically organized into clinical and immunologic domains, and definitions were refined in an iterative process. RESULTS: Based on the data, consensus was reached to use a positive ANA of ≥1:80 titer (HEp-2 cells immunofluorescence) as an entry criterion and to have 7 clinical and 3 immunologic domains, with hierarchical organization of criteria within domains. Definitions of the candidate criteria were specified. CONCLUSION: Using a data-driven process, consensus was reached on new, refined criteria definitions and organization based on operating characteristics. This work will be followed by a multicriteria decision analysis exercise to weight criteria and to identify a threshold score for classification on a continuous probability scale.

OBJECTIVE: The 1980 American College of Rheumatology (ACR) classification criteria for systemic sclerosis (SSc) lack sensitivity for early SSc and limited cutaneous SSc. The present work, by a joint committee of the ACR and the European League Against Rheumatism (EULAR), was undertaken for the purpose of developing new classification criteria for SSc. METHODS: Using consensus methods, 23 candidate items were arranged in a multicriteria additive point system with a threshold to classify cases as SSc. The classification system was reduced by clustering items and simplifying weights. The system was tested by 1) determining specificity and sensitivity in SSc cases and controls with scleroderma-like disorders, and 2) validating against the combined view of a group of experts on a set of cases with or without SSc. RESULTS: It was determined that skin thickening of the fingers extending proximal to the metacarpophalangeal joints is sufficient for the patient to be classified as having SSc; if that is not present, 7 additive items apply, with varying weights for each: skin thickening of the fingers, fingertip lesions, telangiectasia, abnormal nailfold capillaries, interstitial lung disease or pulmonary arterial hypertension, Raynaud's phenomenon, and SSc-related autoantibodies. Sensitivity and specificity in the validation sample were, respectively, 0.91 and 0.92 for the new classification criteria and 0.75 and 0.72 for the 1980 ACR classification criteria. All selected cases were classified in accordance with consensus-based expert opinion. All cases classified as SSc according to the 1980 ACR criteria were classified as SSc with the new criteria, and several additional cases were now considered to be SSc. CONCLUSION: The ACR/EULAR classification criteria for SSc performed better than the 1980 ACR criteria for SSc and should allow for more patients to be classified correctly as having the disease.

Contains fulltext : 152283.pdf (Publisher’s version ) (Open Access) OBJECTIVE: Existing criteria for the classification of gout have suboptimal sensitivity and/or specificity, and were developed at a time when advanced imaging was not available. The current effort was undertaken to develop new classification criteria for gout. METHODS: An international group of investigators, supported by the American College of Rheumatology and the European League Against Rheumatism, conducted a systematic review of the literature on advanced imaging of gout, a diagnostic study in which the presence of monosodium urate monohydrate (MSU) crystals in synovial fluid or tophus was the gold standard, a ranking exercise of paper patient cases, and a multicriterion decision analysis exercise. These data formed the basis for developing the classification criteria, which were tested in an independent data set. RESULTS: The entry criterion for the new classification criteria requires the occurrence of at least 1 episode of peripheral joint or bursal swelling, pain, or tenderness. The presence of MSU crystals in a symptomatic joint/bursa (i.e., synovial fluid) or in a tophus is a sufficient criterion for classification of the subject as having gout, and does not require further scoring. The domains of the new classification criteria include clinical (pattern of joint/bursa involvement, characteristics and time course of symptomatic episodes), laboratory (serum urate, MSU-negative synovial fluid aspirate), and imaging (double-contour sign on ultrasound or urate on dual-energy computed tomography, radiographic gout-related erosion). The sensitivity and specificity of the criteria are high (92% and 89%, respectively). CONCLUSION: The new classification criteria, developed using a data-driven and decision analytic approach, have excellent performance characteristics and incorporate current state-of-the-art evidence regarding gout. 01 oktober 2015

Objective The 1980 American College of Rheumatology (ACR) classification criteria for systemic sclerosis (SSc) lack sensitivity for early SSc and limited cutaneous SSc. The present work, by a joint committee of the ACR and the European League Against Rheumatism (EULAR), was undertaken for the purpose of developing new classification criteria for SSc. Methods Using consensus methods, 23 candidate items were arranged in a multicriteria additive point system with a threshold to classify cases as SSc. The classification system was reduced by clustering items and simplifying weights. The system was tested by 1) determining specificity and sensitivity in SSc cases and controls with scleroderma-like disorders, and 2) validating against the combined view of a group of experts on a set of cases with or without SSc. Results It was determined that skin thickening of the fingers extending proximal to the metacarpophalangeal joints is sufficient for the patient to be classified as having SSc; if that is not present, 7 additive items apply, with varying weights for each: skin thickening of the fingers, fingertip lesions, telangiectasia, abnormal nailfold capillaries, interstitial lung disease or pulmonary arterial hypertension, Raynaud's phenomenon, and SSc-related autoantibodies. Sensitivity and specificity in the validation sample were, respectively, 0.91 and 0.92 for the new classification criteria and 0.75 and 0.72 for the 1980 ACR classification criteria. All selected cases were classified in accordance with consensus-based expert opinion. All cases classified as SSc according to the 1980 ACR criteria were classified as SSc with the new criteria, and several additional cases were now considered to be SSc. Conclusion The ACR/EULAR classification criteria for SSc performed better than the 1980 ACR criteria for SSc and should allow for more patients to be classified correctly as having the disease. Copyright © 2013 by the American College of Rheumatology.

Background The existing 1980 classification criteria for systemic sclerosis (SSc) are suboptimal for patients with early SSc and some patients with limited cutaneous SSc (lcSSc) where an important subset do not meet those criteria. Objectives To develop and test new classification criteria for SSc by a joint EULAR and ACR committee. Methods Delphi exercises and a nominal group technique were used to create a set of potential items for classification of SSc. Twenty cases were prospectively collected to represent the spectrum of SSc (low probability to high probability) which were ranked by SSc experts and conjoint analysis (1000 Minds®) was used to assign weights to the items and reduce the number of items. Experts agreed that all patients with sclerodactyly and scleroderma skin involvement proximal to the MCPs were considered SSc; patients with skin involvement due another scleroderma-like disorder (e.g. scleromyxedema) or skin thickening sparing the fingers were not regarded to have SSc. The provisional classification system was tested in a random sample of SSc cases and controls of 100 from North America and 100 from Europe (derivation sample). The system was simplified and calibrated to real cases. The classification system was re-analyzed in another sample of SSc cases and controls (validation set; n=405). Results The table provides the final items with the proposed weights. Only the maximum score in each domain is counted. A cut-off of >= 9 (out of 19) had a sensitivity of 91% and specificity of 92% in the validation cohort (n=405). The sensitivity and specificity of the former 1980 ARA criteria in this database were 75% and 72%, respectively. These results are preliminary and not yet approved by the organizational sponsors. Conclusions The preliminary results of the classification criteria for SSc performed better than 1980 Preliminary ARA Criteria for SSc. These criteria can be endorsed for epidemiological studies and clinical trials after approval by ACR and EULAR. Disclosure of Interest None Declared