Factors influencing the EULAR Sjögren's Syndrome Patient-Reported Index in primary Sjögren's syndrome

Abstract

Objective. The EULAR Sjögren's Syndrome Patient-Reported Index (ESS-PRI) is a validated tool for measuring pain, fatigue and dryness in primary Sjögren's syndrome (pSS). We evaluated its association with disease and non-disease related variables, and its variation though the follow-up. Methods. We included 130 pSS patients who were interviewed to register demographics, schooling, smoking, menopause, body mass index, disease duration, use of hormonal replacement, associated sicca drugs, prednisone, immunosuppressors/antimalarials, comorbidities such as diabetes mellitus, hypothyroidism, depression, fibromyalgia and scored the Charlson comorbidity index. We assessed the non-stimulated whole salivary flow (NSWSF), Schirmer-I test, ESSDAI and ESSPRI scores. In a subset of patients, we scored a second ESSPRI.Results. Most patients were women, mean age 57 years and median disease duration 9.3 years. The median ESSPRI score was 6 (fatigue 6, pain 4, dryness 8). Eighty patients (61.5%) had an ES-SPRI ≥5 points and were characterised by a higher prevalence of depression (OR 3.7,) and lower NSWSF (OR 0.59,. Among 62 patients with a second ESS-PRI (median time 25 months), 44 (70%) experienced a decrement/increment ≥1 in the ESSPRI (16 were decrement). We did not find any of the studied variables associated with this variation, also including change in prednisone or immunosuppressors.Conclusion. An ESSPRI ≥5 (unsatisfactory symptom state) was associated with low NSWSF and depression. Most of the patients experienced a clinically significant ESSPRI variation (increment or decrement), nevertheless, we were not able to identify any variable associated with this change. Further studies would be helpful to understand the underlying causes.

Patient Acceptable Symptom State (PASS) in patients with primary Sjögren's syndrome in daily clinical practice

“…In the study by Seror et al (12), 38% of patients were in an acceptable symptom state according to ESSPRI in the ASSESS cohort, and this was 32% in the EULAR cohort, which was more comparable to the findings in our study. Another observational cohort study, showed that 38% had an ESSPRI score <5, which was also comparable to our cohort (19). We found that PASS was independently associated with ESSPRI and disease duration, although the explained variance of these parameters to predict PASS was low (Nagelkerke R 2 0.31).…”

“…Since previous research showed that subjective and objective dryness measurements also show poor correlations, this is not an unexpected finding (24). However, an observational cohort study in 130 pSS patients found that patients with an ESSPRI≥5 had lower UWSF scores than patients with ESS-PRI<5 (19). Since the ESSPRI includes a question specifically about dryness and the PASS is a global question, this might explain this difference.…”

Saliva Free Light Chains in Patients with Neuro-Sjögren

“…In the present study, salivary protein concentrations significantly correlated with the ESSPRI pain subscore. A correlation between increasing ESSPRI scores and a decrease in non-stimulated total salivary flow has been reported in the literature [ 34 ]. Interestingly, ESSPRI dryness did not correlated with tear or salivary flow, but ESSPRI pain did [ 35 ].…”

“…Similarly, Sandhya et al reported a positive correlation of salivary KFLC with features of dry mouth, but beyond that, there were no other statistically significant correlations of salivary FLC with sicca symptoms [ 17 ]. In addition, several studies found a weak correlation between objective and subjective indices of ocular dryness [ 33 , 34 ]. On the one hand, it could be hypothesized that the lower salivary fluid production in patients with SS leads to more concentrated saliva, and thus to increased FLC concentrations.…”

“…Therefore, the authors noted that the ESSPRI dryness score may not reflect the true assessment of dryness [ 35 ]. In contrast, a more complex relationship and a more general concept of dryness under the influence of pain and fatigue should be assumed [ 34 , 35 ]. Other studies investigating FLC in patients with SS reported significant correlations with the ESSDAI total score, as well as specific domains of the score with serum IgG, KFLC, and LFLC, but none of the salivary biomarkers [ 12 , 13 , 14 , 15 , 16 , 17 ].…”