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Clinical and laboratory findings of primary Sjögren's syndrome patients without sicca symptoms
L.G. Chatzis1, V. Koulouri2, C. Baldini3, V.C. Pezoulas4, P.V. Voulgari5, F.N. Skopouli6, D.I. Fotiadis7, A.G. Tzioufas8, A.V. Goules9
- Pathophysiology Department, Athens School of Medicine, National and Kapodistrian University of Athens, Greece. lukechatzis@gmail.com
- Pathophysiology Department, Athens School of Medicine, National and Kapodistrian University of Athens, Greece.
- Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Italy.
- Unit of Medical Technology and Intelligent Information Systems, University of Ioannina, Greece.
- Rheumatology Clinic, Department of Internal Medicine, Medical School, University of Ioannina, Greece.
- Department of Nutrition and Clinical Dietetics, Harokopio University of Athens, Greece.
- Unit of Medical Technology and Intelligent Information Systems, University of Ioannina, and Department of Biomedical Research, Institute of Molecular Biology and Biotechnology, FORTH, Ioannina, Greece.
- Pathophysiology Department, Athens School of Medicine, National and Kapodistrian University of Athens, Greece.
- Pathophysiology Department, Athens School of Medicine, National and Kapodistrian University of Athens, Greece.
CER16010
2022 Vol.40, N°12
PI 2298, PF 2302
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PMID: 36200933 [PubMed]
Received: 04/07/2022
Accepted : 26/08/2022
In Press: 05/10/2022
Published: 20/12/2022
Abstract
OBJECTIVES:
Primary Sjögren’s syndrome (pSS) is a systemic autoimmune disease characterised by oral and eye dryness. A minority of patients can present without dryness but studies on their clinico-laboratory manifestations are scarce. Our purpose was to describe the clinical phenotype of pSS patients lacking sicca symptoms.
METHODS:
From a total of 1738 consecutive pSS patients fulfilling the 2016 ACR-EULAR criteria, those who presented without sicca symptoms were identified (non-dryness group). Their medical data was collected and compared with 2 control groups: a) the remaining unmatched sicca pSS patients with both oral and eye dryness (unmatched dryness group) and b) matched sicca pSS patients according to age, sex, and disease duration, in 1:2 ratio (matched dryness group).
RESULTS:
Thirty-eight (2.19%) patients lacked sicca manifestations presenting mainly with arthralgias (47%), parotid enlargement (24%), Raynaud’s phenomenon (11%) and persistent lymphadenopathy (11%) that led them to be evaluated for pSS. Non-dryness pSS patients were younger than the unmatched sicca controls, displaying a higher frequency of anti-Ro/SSA antibodies (100% vs. 79.7%, p<0.001), ANA positivity (100% vs. 90.4%, p<0.001), neutropenia (20.8% vs. 7.5%, p=0.04) and thrombocytopenia (13.8% vs. 4.2%, p=0.04). They also had lower frequency of positive ocular tests compared to both unmatched and matched dryness patients. No differences were found between non-dryness pSS patients and both control groups regarding focus score or any other extraglandular manifestation.
CONCLUSIONS:
pSS patients without sicca complaints constitute a distinct phenotype involving younger patients, sharing common immunopathologic mechanisms with typical sicca patients.
