Abstract

BackgroundTo date, no study has clinically focused on primary Sjögren’s Syndrome (pSS) patients presenting without or with low level of dryness features.ObjectivesTo describe pSS patients presenting no dryness or with low level of dryness and to compare them with pSS patients with dryness features to determine whether a specific clinical presentation can be identified in these patients.MethodsAll patients diagnosed with pSS according to AECG or ACR/EULAR criteria in our tertiary reference center were included. All patients were recruited between 1999 and 2020. Patient were considered without subjective dryness if the VAS for dry eyes or dry mouth was ≤ 30/100 and/or without objective dryness if having both normal Schirmer and salivary flow rate.ResultsOverall, 509 patients were included in the subjective dryness comparison group and 434 in the objective one (Table 1). Compared to patients with subjective dryness (n=456), patients without subjective dryness (n=53) were significantly younger (p=0,0025), were diagnosed earlier (p=0,0056), were more frequently anti-SSA positive (p=0,008), had lower levels of fatigue (p=0,0005). But no difference was observed regarding the level of disease activity or frequency of organ involvement. By contrast, patients reporting subjective dryness had more frequently chronic cough (p=0,022), with no more frequent objective lung involvement. Thus, the higher prevalence of chronic cough might be explained by dryness of the upper airways. Table 1.Comparison of pSS patients with or without drynessNo Subjective drynessSubjective dryness n=456P valuen=53Age, median (IQR)49 (39-62)58 (47-67)0,0025**Diagnostic delay, mean, years4,326,270,0056**ESSDAI, mean (SD)3,55 (3,38)3,22 (3,28)0,52MSGB# Chisolm score of 3 or 4, n (%)36 (68)362 (79)0,08Anti Ro/SSA, n (%)44 (83)292 (64)0,008**Chronic cough, n (%)5 (9)110 (24)0,022*VAS pain, median (IQR)40 (4-70)53 (23-77)0,06VAS fatigue, median (IQR)47 (13-71)65 (44-82)0,0005***No Objective drynessObjective dryness n=326n=108Age, median (IQR)52 (41-60)59 (47-67)0,0026**Diagnostic delay, mean, years5,96,10,75ESSDAI, mean (SD)3,462,960,056MSGB# Chisolm score of 3 or 4, n (%)81 (75)255 (78)0,49Anti Ro/SSA, n (%)84 (78)204 (62)0,0042**Arthralgia, n (%)81 (75)210 (64)0,04*VAS pain, median (IQR)59,5 (32,5-80)50 (20-73)0,03*VAS fatigue, median (IQR)66 (44-83)61 (39-80)0,27VAS eye dryness, median (IQR)49 (17-71)57 (25-80)0,019*VAS mouth dryness, median (IQR)58 (34-75)70 (48-88)0,001**MSGB: minor salivary gland biopsyThe patients without objective dryness (n=108) were also younger (p=0,0026), and had more frequently anti-SSA positive (p=0,0042). They also had a higher disease activity (mean ESSDAI: 3,46 versus 2,96, p=0,056). In this group of patients, the higher frequency of anti-SSA might be explained by the need of other criteria to fulfil diagnostic criteria, in the absence of abnormal Schirmer and salivary flow. Patients with objective dryness had more frequently arthralgia (p=0,04), a higher level of pain VAS (p=0,03) and subjective dryness VAS (p=0,019 for ocular and p=0,01 for oral) than patients without objective dryness.Among the 108 patients with no objective dryness, only 17 had also had no subjective dryness (VAS <30mm).P value corresponds to the comparison of the two strata with Student’s t or Mann Whitney-U testConclusionAmong the patients with pSS, those without subjective or objective dryness features had a younger profile, shorter diagnosis delay which may result from a more acute onset, were more frequently anti-SSA positive. Those having no objective dryness also had more systemic disease, arthralgias and pain than patients with dryness features. Subjective dryness was associated with higher level of fatigue.

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