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IgG4-related disease (IgG4-RD) is a multisystem fibroinflammatory disorder diagnosed and managed across a broad spectrum of specialities. The American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria for IgG4-RD, recently published in this journal,1 provide robust and validated measures for discrimination from disease mimics with excellent test performance. The classification criteria incorporates three domains: entry, exclusion and inclusion criteria, with numerically weighted inclusion criteria reaching a score of 20 points or more to support a diagnosis of IgG4-RD. We sought to evaluate these criteria in a real-world cohort of patients referred for discussion to our supraregional UK IgG4-RD multidisciplinary meeting (MDM) over a 3-year period from 2016 to 2019.2
We reviewed 165 patients discussed at our IgG4 MDM who had been classified as definite IgG4-RD, possible IgG4-RD or not IgG4-RD according to standard diagnostic criteria (HISORt for pancreas/biliary disease,3 Japanese Consensus Diagnostic Criteria for systemic disease,4 Consensus Statement on Pathology5) and MDM consensus opinion with prospective follow-up in a dedicated clinic. We applied the new ACR/EULAR classification criteria retrospectively to these cases to assess …
Contributors RJRP was involved in study design, data collection, analysis and interpretation, in addition to drafting and critical appraisal of the manuscript. AV, EF, HB, MR-J, MC, EB and GW were involved in data collection and analysis, in addition to critical review of the manuscript. ELC was responsible for the original concept and was involved in study design, data collection, analysis and interpretation in addition to drafting and critical appraisal of the manuscript.
Funding This articleand its investigators are supported by (1) the National Institute of Health Research (NIHR) Biomedical Research Centre, based at Oxford University Hospitals Trust, and (2) Oxfordshire Health Service Research Committee as part of Oxford Hospitals Charity, Oxford. The views expressed in this article are those of the authors and not necessarily those of the NHS, the NIHR or the Department of Health.
Competing interests ELC consults for Viela Bio Pharma for IgG4-RD outside the submitted work. There are no commercial or financial conflicts of interest related to this article.
Patient and public involvement Regular patient engagement is conducted through the UK IgG4-related disease patient and public involvement group, and a lay summary of this work will appear on the patient pages of the authors' website (https://igg4-rd.ndm.ox.ac.uk).
Provenance and peer review Not commissioned; internally peer reviewed.