Salivary Glands

In February 2004, the panel of experts gathered for a face-to-face meeting moderated by a statistician (SCS) and an epidemiologist (CHS). The goal of this meeting was to obtain consensus (at least 80%) on the target population to whom the classification criteria would apply, and the initial list of variables or criteria items that would be collected as part of SICCA. The meeting began with presentation of a comprehensive literature review by one of the senior investigators (TED) of the 11 previous classification and diagnostic criteria for SS that had been published in the past 40 years, none of which had been endorsed by the ACR or EULAR.
There was consensus among the panel that the criteria should apply to the population of patients who may be referred to a specialist because of signs and/or symptoms possibly suggesting SS. Recruitment strategies and eligibility criteria are described below. The rationale for selecting this target population is that a given patient would not be evaluated for SS unless she/he had signs or symptoms suggesting this diagnosis. There was also consensus that if asked to select cases and controls for validation of new classification criteria, panel members would use objective tests (e.g., specific serum measures of autoimmunity, ocular staining reflecting lacrimal hypofunction, and LSG biopsy reflecting FLS) that would likely be part of the new classification criteria, leading to circularity. Therefore, it was agreed that no diagnostic labels would be used for enrollment, and that all participants would undergo the same set of standardized objective tests, and questionnaires capturing various signs and symptoms.
The panel agreed upon examinations and tests used to assess ocular and oral signs and symptoms, tear and salivary function, LSG biopsy results and various serum measures of autoimmunity. The list created was based both on published results and on the clinical experience of panel members. There was discussion among the rheumatologists regarding which extra-glandular manifestations possibly associated with SS should be captured, and a consensus was achieved regarding a list of signs/symptoms that would be measured through a targeted rheumatologic examination, review of systems, careful medical history and serologic laboratory measures. Similarly, the oral medicine specialists agreed on a list of tests measuring salivary function (both stimulated parotid and UWS flow rates), and salivary gland expression of autoimmunity through biopsy of LSG, examining them for the presence of FLS, and measuring FS accordingly as described in detail elsewhere (15 (link)). The ophthalmologists agreed on tests evaluating participants for the presence of keratoconjunctivitis sicca (KCS). There was consensus that, while rose Bengal had been widely used for grading conjunctival and corneal damage in patients with KCS, it is inherently toxic to epithelial cells and very painful for patients. Therefore, fluorescein was selected to grade the cornea and lissamine green the bulbar conjunctiva. Effectiveness for grading KCS is established for both (16 (link)). They agreed on a standardized quantitative grading system that would be easily reproducible and could be used in clinical practice in the future (17 (link)). Ocular staining score (OSS) is the sum of a 0–6 score for fluorescein staining of the cornea and a 0–3 score for lissamine green staining of both nasal and temporal bulbar conjunctivae, yielding a total score ranging from 0 to 12. Alternative established tests for dryness used in prior criteria, such as tear break-up time (TBUT) and unanesthetized Schirmer test, were also included.
The final list of criteria items that was agreed upon by the end of the first meeting included nearly all those previously reported in the relevant literature. It has been described previously (12 (link)) and is available at http://sicca.ucsf.edu.