Patients with early-onset primary Sjögren’s syndrome have distinctive clinical manifestations and circulating lymphocyte profiles

Abstract

Objectives To further investigate the clinical characteristics and circulating lymphocyte profiles of patients with early-onset primary Sjögren’s syndrome (pSS). Method Data of 333 patients with pSS were analyzed retrospectively. Early onset was defined as a pSS diagnosis at an age of 35 years or younger. The clinical, laboratory, and immunophenotypic profiles of peripheral blood lymphocyte subsets were compared between early- and later-onset pSS. Results Thirty-six (10.81%) patients matched the definition of early-onset pSS, with age at disease onset being 28.97 ± 5.53 years. Elevated serum IgG level (77.14% vs 31.16%, p < 0.001), low C3 (41.67% vs 20.20%, p = 0.004) and C4 levels (27.78% vs 6.40%, p < 0.001), anti-SSA positivity (91.67% vs 51.85%, p < 0.001), and anti-SSB positivity (50% vs 20.54%, p < 0.001) were more frequent in early-onset patients. The frequencies of hematological (80.56% vs 52.53%, p = 0.001), renal (19.44% vs 5.05%, p = 0.005), and mucocutaneous involvement (50% vs 22.56%, p < 0.001) were significantly higher in the early-onset pSS group, which showed a higher ESSDAI (11(6.25-17) vs 7(3-12); p = 0.003), compared with the later-onset group. In addition, profound CD4+ T-cell lymphopenia was found in patients with early-onset. Conclusions Patients with early-onset pSS have distinctive clinical manifestations and greater activation of the cellular immune system, present with more severe clinical symptoms and immunological features, have increased activation of circulating T cells, and have an unfavorable prognosis. Thus, they require more positive treatment with glucocorticoids and/or immunosuppressants and merit closer follow-up and regular monitoring.

The Predictive Role of Neutrophil-to-Lymphocyte Ratio (NLR), Platelet-to-Lymphocyte Ratio (PLR), Monocytes-to-Lymphocyte Ratio (MLR) and Gammaglobulins for the Development of Cutaneous Vasculitis Lesions in Primary Sjögren’s Syndrome

“…Furthermore, the analysis of cellular ratios—NLR, MLR and PLR—revealed higher values in pSS patients compared to controls, suggesting that, even though all cellular lines were decreased in pSS patients, the important drop in circulating lymphocytes counts is the main actor to influence these differences. These findings are supported by similar results reported from previous studies investigating the hematological profile of pSS patients [ 13 , 18 , 29 ]. Disease activity, quantified through ESSDAI and VAS scores, was higher in our group of pSS patients compared to previous studies [ 13 , 21 ], probably due to a late diagnosis of the disease.…”

Predictors for future development of systemic lupus erythematosus in Korean Sjögren’s syndrome patients

“…A recent study found that SS patients with early-onset and shorter disease duration had higher disease activity (by ESSDAI) and greater cellular immune system activity than those with late-onset and longer disease duration. 37 In our study, the HR of SS disease duration (≥3 years) at baseline was 0.38 (95% CI: 0.18-0.81; p = .0116). The results revealed that the shorter the period from SS diagnosis to baseline, the higher the risk of developing SLE in the future.…”

Association of Combined Anti‐Ro52/TRIM21 and Anti‐Ro60/SSA Antibodies With Increased Sjögren Disease Severity Through Interferon Pathway Activation

“…Patients had a significantly different age at onset of symptoms according to their subgroup, with an earlier onset of the disease in the Ro52 + /Ro60 + patients (P = 0.004), which is in agreement with the available data in the literature. 28 The time course of the disease also varied according to the subgroup (P = 0.001). Specifically, single-positive antibody patients had a shorter disease duration than the other patients.…”