Multimodal Assessment and Characterization of Sicca Syndrome

Background: Sicca syndrome represents a heterogeneous group of conditions, such as Sjögren syndrome, causing xerophthalmiaand xerostomia. This study characterizes in depth patients with Sicca syndrome and evaluates salivary gland ultrasound (SGUS).Methods: Principal component analysis and hierarchical clustering of clinical parameters, such as ESSPRI, ESSDAI and laboratory data, were performed on all referrals for assessment of Sicca symptoms between October 2018 and March 2021. SGUS and labial gland biopsies were compared across groups.Results: A total of 583 patients were assessed. Objective dryness was confirmed in 73% of the patients. Cluster analysis identified 3 groups with post-hoc analysis confirming distinct phenotypes: Somatic Group (283/583; 49%) with more frequent symptoms but limited objective dryness; Dry Without Autoimmune Features (DAFneg, 206/584; 35%), and Dry With Autoimmune Features (DAFpos, 94/584;16%). DAFpos patients had highest autoantibody titers (anti-SSA(Ro) 240 vs. 3.6 vs. 3.8; p < 0.001), most extra-glandular manifestations (p < 0.001), and highest median SGUS Score (DAFpos: 8 [IQR 4–10] vs. SG: 2 [1–4] vs. DAFneg 4 [2–5]; p < 0.001). No tangible correlation with primary Sjögren syndrome criteria was observed.Discussion: SGUS score correlated with a subset of patients with Sjögren syndrome, identified in the DAFpos cluster. This study highlights heterogeneity within sicca and, indeed, Sjögren syndrome, highlighting the need for further studies.

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Tear Meniscus and Corneal Sub-basal Nerve Plexus Assessment in Primary Sjögren Syndrome and Sicca Syndrome Patients

Cornea ◽

10.1097/ico.0000000000001800 ◽

2018 ◽

pp. 1

Author(s):

Joana Cardigos ◽

Filipe Barcelos ◽

Helena Carvalho ◽

Diogo Hipólito ◽

Sara Crisóstomo ◽

...

Keyword(s):

Sjögren Syndrome ◽

Sicca Syndrome ◽

Nerve Plexus ◽

Sjogren Syndrome ◽

Primary Sjögren Syndrome ◽

Tear Meniscus

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Prevalence and characterization of non-sicca onset primary Sjögren syndrome with interstitial lung involvement

Clinical Rheumatology ◽

10.1007/s10067-017-3601-1 ◽

2017 ◽

Vol 36 (6) ◽

pp. 1261-1268 ◽

Cited By ~ 17

Author(s):

Andreina Manfredi ◽

Marco Sebastiani ◽

Stefania Cerri ◽

Giulia Cassone ◽

Pietrantonio Bellini ◽

...

Keyword(s):

Sjögren Syndrome ◽

Sjogren Syndrome ◽

Lung Involvement ◽

Primary Sjögren Syndrome

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Clinical characterization of annular erythema in European white patients with primary Sjögren syndrome

European Journal of Internal Medicine ◽

10.1016/j.ejim.2013.08.308 ◽

2013 ◽

Vol 24 ◽

pp. e121

Author(s):

S. Retamozo ◽

P. Brito-Zeron ◽

M. Akasbi ◽

M. Gandia ◽

M. Perez-De-Lis ◽

...

Keyword(s):

Sjögren Syndrome ◽

Sjogren Syndrome ◽

Primary Sjögren Syndrome ◽

Annular Erythema ◽

White Patients

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THU0681 Baseline ESSDAI/DAS scores in 8061 patients with primary sjÖgren syndrome: characterization of systemic disease

10.1136/annrheumdis-2017-eular.4472 ◽

2017 ◽

Author(s):

P Brito-Zerόn ◽

N Acar-Denizli ◽

M Zeher ◽

A Rasmussen ◽

X Li ◽

...

Keyword(s):

Systemic Disease ◽

Sjögren Syndrome ◽

Sjogren Syndrome ◽

Primary Sjögren Syndrome

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Women With Primary Sjögren Syndrome and With Non-Sjögren Sicca Syndrome Show Similar Vulvar Histopathologic and Immunohistochemical Changes

International Journal of Gynecological Pathology ◽

10.1097/pgp.0000000000000295 ◽

2016 ◽

Vol 35 (6) ◽

pp. 585-592 ◽

Cited By ~ 2

Author(s):

Susanna Maddali Bongi ◽

Martina Orlandi ◽

Angelina De Magnis ◽

Daniela Moncini ◽

Angela Del Rosso ◽

...

Keyword(s):

Sjögren Syndrome ◽

Sicca Syndrome ◽

Sjogren Syndrome ◽

Primary Sjögren Syndrome

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Comparison of Effect on Sicca Symptoms of Anticentromere Antibody–positive Sjögren Syndrome and Primary Sjögren Syndrome Alone

The Journal of Rheumatology ◽

10.3899/jrheum.190462 ◽

2019 ◽

Vol 47 (6) ◽

pp. 876-880

Author(s):

Yifan Li ◽

Arthur A.M. Bookman

Keyword(s):

Salivary Gland ◽

Physical Examination ◽

Minor Salivary Gland ◽

Sjögren Syndrome ◽

Sjogren Syndrome ◽

Anticentromere Antibody ◽

Primary Sjögren Syndrome ◽

European Consensus ◽

Significant Difference ◽

Sicca Symptoms

Objective.To determine whether positive anticentromere antibody (ACA) serology affects the severity of sicca symptoms in patients with primary Sjögren syndrome (pSS).Methods.Evaluation to detect subjective and objective sicca symptoms included questionnaires, physical examination, and pathology. Cases of pSS were classified according to the 2002 American-European Consensus Group (AECG) criteria. All patients were evaluated for presence of anti-Ro, anti-La, and ACA serology. Patients with pSS were categorized into ACA+ SS and ACA–SS. The groups were compared for measures of severity of oral and ocular sicca.Results.The pSS group had 446 patients, of whom 26 were ACA+ SS. Subjective ocular sicca measured 7.0 ± 2.4 (out of 10) in ACA+ SS and 6.4 ± 2.6 in ACA–SS (p = 0.197). Objective ocular sicca measured 3.2 mm ± 1.8 mm/5 min in ACA+ SS and 4.2 mm ± 4.4 mm/5 min in ACA–SS (p = 0.038). Subjective oral sicca measured 8.5 ± 1.4 in ACA+ SS and 6.7 ± 2.4 in ACA–SS (p < 0.001). Objective oral sicca measured 0.1 ml ± 0.2 ml/15 min in ACA+ SS and 0.4 ml ± 1.0 ml/15 min in ACA–SS (p < 0.001). Only 35% of ACA+ patients with SS were anti-Ro–positive or anti-La–positive compared with 77% of the ACA–patients with SS (p < 0.001). There was no significant difference in minor salivary gland fibrosis or focus scores between ACA+ SS and ACA–patients with SS.Conclusion.ACA+ SS is associated with more severe objective ocular sicca and more severe subjective and objective oral sicca compared to ACA–SS. The majority of ACA+ patients with SS meet AECG criteria for pSS despite negative serology for anti-Ro/La antibodies.

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