Multimodal Assessment and Characterization of Sicca Syndrome

Abstract

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.

Identification of distinct subgroups of Sjögren's disease by cluster analysis based on clinical and biological manifestations: data from the cross-sectional Paris-Saclay and the prospective ASSESS cohorts

“…Over the past 5 years, attempts have been made to stratify patients with Sjögren's disease using unsupervised clustering; however, the stratification methods used were based only on subjective symptoms ( appendix pp 6–7 ). 7 , 8 , 22 , 23 , 24 In an international, cross-sectional study, Gairy and colleagues 22 used latent class analysis to identify five distinct clusters: two were characterised by a low burden, with or without articular involvement; one by a moderate burden consisting of fatigue, pain, and joint discomfort; and two clusters showed multi-organ involvement, with differing levels of burden (one moderate and one high). A symptom-based cluster analysis using five common symptoms (pain, fatigue, dryness, anxiety, and depression), conducted on data from the UKPSSR, 7 identified four subgroups and was confirmed in two independent validation cohorts: one from Norway and the ASSESS cohort in France.…”

Saliva Free Light Chains in Patients with Neuro-Sjögren

“…The lack of serological and salivary group differences may be due to the fact that our study focused explicitly on patients with SS and neurological involvement. These patients have been shown to have different clinical and paraclinical features compared with patients with SS without neurological involvement [ 3 , 25 ]. Patients with SS-associated polyneuropathy showed pathological results not only in nerve conduction, but also in ultrasound examinations of the nerves, and often meet the diagnostic criteria for CIDP [ 4 , 25 ].…”

“…Sjögren´s syndrome (SS) is an autoimmune disease characterized by lymphocytic infiltration of the exocrine glands leading to sicca symptoms, but may also cause extra-glandular manifestations such as interstitial lung disease, arthritis, cutaneous vasculitis, and central or peripheral nervous system (CNS and PNS) involvement [ 1 , 2 , 3 , 4 , 5 , 6 , 7 ]. According to the latest American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria of 2016, SS can be diagnosed in a patient with sicca symptoms and additional anti-SSA(Ro)-antibody positivity and/or pathological focus scores on a minor salivary gland biopsy [ 8 , 9 , 10 ].…”

Update zum Sjögren-Syndrom: Diagnostik & Therapie

“…Eine subjektiv hohe Krankheitslast durch Trockenheit, Fatigue und Schmerzen korrelierte invers mit laborchemischer Entzündungsaktivität oder schweren Organmanifestationen 13 . PatientInnen mit einer objektivierbaren Trockenheit und mit extraglandulären Organmanifestationen klagten hingegen seltener über eine stark beeinträchtigende Symptomlast 14 .…”