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Primary Sjögren's syndrome (SS) probably has the dubious distinction of having more proposed criteria than any other rheumatic condition—certainly this was the case in the 1980s and 1990s (eg, Copenhagen,1 Californian,2 Greek3 and Japanese4 criteria). In 2002, Vitali et al5 published the revised American European Consensus Group (AECG) criteria, which have now served as the gold standard for over 10 years. In 2012, Shiboski et al6 published an alternative set of criteria, provisionally adopted by the American College of Rheumatology (ACR). These latter criteria (Sjögren's International Collaborative Clinical Alliance (SICCA-ACR)) are based on objective criteria and do not necessarily require patients to have symptoms of dry eyes or dry mouth. The study by Rasmussen et al7 examines patients with primary SS in a large cohort who fulfil either the existing AECG criteria and/or the newly proposed SICCA–ACR criteria, and discusses the similarities and differences between them with some important lessons for the field.
The AECG criteria were developed to screen patients with a specific set of questions regarding dryness and then validate the diagnosis with a series of objective tests that indicated immune factors in the pathogenesis. The questionnaires were developed by an expert consensus panel for sensitivity and specificity as an initial screening tool. The AECG criteria require the presence of four out of six components (one of which is symptomatic dry eyes and a second is symptomatic dry mouth), or three out of the four other objective components, although in either case one of the components has to be positive antibodies and/or a positive biopsy. The four objective components are: (1) positive anti-Ro and/or anti-La antibodies; (2) a positive labial gland biopsy defined as at least one periductal focus of 50 or more lymphocytes per 4 mm2 high powered field; (3) reduced …
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