30.3Evaluation of Primary SS Patients’ Symptoms

Since correlation between objective measures of dryness and symptoms is poor [18, 23], the evaluation of symptoms, along the measure of objective tests, such as SchirmerÕs test and salivary ßow, is necessary. For these reasons, three disease-speciÞc scores are now available for evaluation of patientsÕ symptoms (Table 30.3). The Þrst two scores were the SSI for evaluation of dryness features [18] and the PROFAD for evaluation of fatigue and discomfort [17]. More recently, we developed a simple global score the ESSPRI that encompasses these both components [24].

30.3.1The SSI: Sicca Symptoms Inventory

The SSI was the Þrst disease-speciÞc score developed in a multicenter cohort of 112 UK primary SS patients in 2002 [18]. The deÞnition of each item was based on phrases elicited by patients. Among a large number of initial phrases, items were selected based on the symptom frequencies. Severity of each item is assessed by a 0Ð7 numerical scale. A principal component analysis was then used to gather the ten items into four domains: ocular, oral, vaginal, and cutaneous dryness. Oral and ocular domains include, respectively, Þve and three items, but have the same weight on the Þnal score than cutaneous and vaginal dryness. The Þnal score is the sum of the four domains and varied from 0 to 28.

30.3.2The PROFAD: Profile of Fatigue and Discomfort

The PROFAD was developed in 2004 using the same methodology as SSI. It includes nine items gathered into four domains: somatic fatigue, mental fatigue, arthralgias and RaynaudÕs phenomenon. Even if somatic fatigue is the predominant domain and includes four items, like for the

SSI, in the PROFAD all domains have the same weight on the Þnal score. The Þnal score is the sum of the four domains and varied from 0 to 28.

30.3.3The ESSPRI: EULAR Sjögren’s Syndrome Patients Reported Index

The ESSPRI has been recently developed in a multicenter international cohort of 230 patients (Table 30.4). The domains were selected based on previous data from development of SSI and PROFAD. Selection of domains and determination of their weights were determined from the patientsÕ perspective, using multiple linear regression with patient global assessment as gold standard. The ESSPRI uses 0Ð10 numerical scales, one for assessment of each of the three domains: dryness, fatigue, and pain (articular and/or muscular). The weights of the domains were identical, and the Þnal score is the mean of the score of each domain.

30.3.4Comparisons of Scores for Evaluation of Patients’ Symptoms

Table 30.3 compared the methodology used to develop the three scores. Until development of ESSPRI, the SSI assessed dryness features and the PROFAD fatigue and discomfort; these both scores are not supposed to be combined, and no global score was available. Therefore, having a global score, such as the ESSPRI, able to capture all important symptoms of the disease, may be a consistent help to design and conduct clinical trials in primary SS. Compared to ESSPRI, which evaluated all dryness features and also all fatigue component with only one scale for each, the SSI evaluates separately each dryness feature and separate fatigue in two components (somatic and mental). Even if the number of items is higher for the most relevant components (oral and ocular dryness in SSI, and somatic fatigue in PROFAD), these domains have the same weight

Table 30.3 Comparisons of the three patientsÕ indexes for primary SjogrenÕs syndrome

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