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Table 3 Association between organ-by-organ baseline ESSDAI activity at diagnosis and the risk of development of the three main subtypes of hematological cancer

From: Characterization and risk estimate of cancer in patients with primary Sjögren syndrome

Variables B-cell MALT (n = 27) B-cell non-MALT (n = 19) Non-B-cell neoplasms (n = 15)
Dry eye 0.90 [0.12–6.83] 0.76 [0.10–5.94]
Altered ocular tests 1.33 [0.17–10.17]
Altered parotid scintigraphy 1.10 [0.25–4.86] 0.49 [0.13–1.87]
Positive salivary gland biopsy 1.84 [0.42–8.04] 0.37 [0.07–2.12] 0.83 [0.17–3.98]
Anemia (Hb < 110 g/L) 0.89 [0.31–2.61] 2.58 [1.01–6.58] 4.02 [1.42–11.38]
Leukopenia (<4000/mm3) 0.51 [0.15–1.69] 0.84 [0.24–2.89] 2.54 [0.86–7.52]
Thrombocytopenia (<150000/mm3) 0.47 [0.06–3.48] 4.85 [1.64–14.34]
Neutropenia (<1500/mm3) 1.24 [0.42–3.63] 0.51 [0.07–3.92] 5.86 [1.89–18.14]
Lymphopenia (<1000/mm3) 2.12 [0.79–5.68] 0.32 [0.04–2.48] 2.14 [0.65–7.02]
Antinuclear antibodies+ 1.15 [0.35–3.84] 0.86 [0.25–2.97] 1.14 [0.26–5.10]
Rheumatoid factor+ 0.94 [0.43–2.03] 1.16 [0.47–2.86] 1.28 [0.46–3.53]
Anti-Ro/SS-A+ 1.07 [0.42–2.71] 1.63 [0.53–5.03] 1.26 [0.39–4.09]
Anti-La/SS-B+ 0.66 [0.30–1.46] 2.35 [0.88–6.32] 1.34 [0.47–3.83]
Monoclonal gammopathy 1.30 [0.38–4.43] 3.34 [1.14–9.83] 2.16 [0.58–8.04]
Cryoglobulins+ 6.32 [2.47–16.15] 3.31 [1.06–10.36] 4.36 [1.12–16.99]
Low C3 levels (<0.82 g/L) 3.25 [1.33–7.93] 1.14 [0.26–5.06] 1.43 [0.31–6.48]
Low C4 levels (<0.11 g/L) 1.76 [0.65–4.78] 3.83 [1.31–11.21] 1.36 [0.30–6.11]
Baseline ESSDAI 1.07 [1.03–1.11] 1.04 [0.98–1.09] 1.08 [1.03–1.13]
Baseline DAS (REF = low)
Moderate 0.91 [0.33–2.51] 1.20 [0.42–3.47] 3.37 [0.84–13.52]
High 4.80 [1.99–11.62] 2.60 [0.84–8.07] 8.29 [2.05–33.55]
ESSDAI domains†
Constitutional 1.31 [0.39–4.44] 1.62 [0.37–7.12] 7.20 [2.43–21.32]
Lymphadenopathy 9.39 [4.20–21.02] 8.99 [3.35–24.14] 4.74 [1.32–17.02]
Glandular 7.35 [3.36–16.10] 1.34 [0.44–4.13] 1.38 [0.38–5.04]
Articular 1.09 [0.50–2.39] 2.07 [0.82–5.18] 1.00 [0.34–3.00]
Cutaneous 1.16 [0.35–3.87] 1.28 [0.29–5.56] 0.78 [0.10–6.00]
Pulmonary 4.98 [1.50–16.59]
Central nervous System 1.12 [0.15–8.29]
Hematological 1.10 [0.50–2.42] 0.61 [0.22–1.69] 4.99 [1.58–15.76]
Biological 1.35 [0.60–3.06] 6.57 [1.51–28.55] 1.68 [0.57–4.95]
  1. Values are represented as the HRs (95% CIs). B-cell non-MALT lymphomas included diffuse large B-cell lymphoma (7), marginal zone lymphoma (4), myeloma multiple (2), and other B-cell lymphomas (6). Non-B-cell neoplasms included myelodysplastic syndromes (4), leukemia (4), Hodgkin lymphoma (4), and T/NK cell neoplasias (3)
  2. Set in italics, statistically significant (p < 0.05) SS-related features and ESSDAI domains associated with cancer in the univariate Cox proportional hazards regression analysis adjusted for age at diagnosis and gender
  3. REF reference level, ESSDAI EULAR-Sjögren Syndrome Disease Activity Index, DAS disease activity states
  4. †Level of activity is recoded as no versus any type of activity (low/moderate/high) in the analysis. There were not enough observations to fit models for the renal, muscular, and peripheral nervous system domains