Date: Tuesday, November 15, 2016
Session Title: Sjögren's Syndrome I: Clinical Insights
Session Type: ACR Concurrent Abstract Session
Session Time: 2:30PM-4:00PM
Background/Purpose: Very limited data is available regarding predictors of systemic disease activity in primary Sjögren’s syndrome. The ESSDAI, the international systemic disease activity score of the disease, is mainly used in clinical trials, as an inclusion criteria and primary end point. The ESSDAI takes into account some routine laboratory parameters included in the haemotological (disease-related anemia, lymphopenia, neutropenia and thrombocytopenia) and biological domain (IgG levels, monoclonal component, cryoglobulinemia, complement levels). We therefore investigated whether abnormalities of either the haematological or the biological domain could predict the evolution of systemic disease activity.
Methods: The ASsessment of Systemic complications and Evolution in primary Sjögren’s Syndrome (ASSESS) cohort is a prospective ongoing cohort of 395 patients. All patients have a medical examination every year by a trained physician, who notably collects the ESSDAI (Eular Sjögren’s Syndrome Disease Activity Index), which scores systemic disease activity and clinical ESSDAI (clin ESSDAI), which scores systemic disease activity without the biological domain of the ESSDAI.
Results: At enrollment, 1, 2, 3 and 4 years of follow-up, 55%, 44.3%, 48.1%, 46.9%, and 46.5% had at least one biological abnormality (i.e. a positive biological domain of the ESSDAI), respectively. The mean (SEM) ESSDAI 1 year after: at 1, 2, 3, 4 and 5 years of follow-up were 5.0 (0.4), 5.3 (0.4), 6.2 (0.5), 5.6 (0.5), and 5.4 (0.5), respectively, compared to 3.9 (0.4), 3.7 (0.3), 3.7 (0.4), 3.5 (0.4), and 3.4 (0.3), in patients with no biological abnormality, respectively. Overall, mean (SEM) ESSDAI the year after, in patients with at one least biological abnormality the year before ESSDAI assessment, was 5.5 (0.2) compared to 3.6 (0.2) in patients with no biological abnormality (p< 0.0001). Significant associations were also observed between biological abnormalities and clinESSDAI the year after (clinESSDAI of 4.7 (0.2) with a positive biological domain the year before and 3.9 (0.2 without, p= 0.007) as well as between hypergammaglobulinemia, a frequent abnormal parameter in the biological domain, and ESSDAI and clinESSDAI the year after (5.8 (0.3) and 4.8 (0.3) versus 4.1(0.1) and 4.0 (0.1) in patients without hypergammaglobulinemia, p<0.0001 and p= 0.002, respectively). At enrollment, 1, 2, 3 and 4 years of follow-up, 26.7%, 25.3%, 24.2%, 25.7%, and 24.1% had at least haematological abnormality (i.e a positive haematological domain of the ESSDAI), respectively. The mean (SEM) ESSDAI 1 year after: at 1, 2, 3, 4 and 5 years of follow-up were 5.6 (0.6), 5.9 (0.6), 7.0 (0.7), 6.6 (0.9), and 6.5 (0.7), respectively, compared to 4.1 (0.3), 3.8 (0.3), 4.0 (0.3), 3.7 (0.4), and 3.6 (0.3), in patients with no haematological abnormality, respectively. Overall, mean (SEM) ESSDAI the year after in patients with at one least haematological abnormality the year before ESSDAI assessment, was 5.7 (0.3) compared to 3.7 (0.1) in patients with no haematological abnormality (p< 0.0001). Significant associations were also observed between haematological abnormalities and clinESSDAI the year after (5.7 (0.3) versus 3.7 (0.1) in patients with no haematological abnormality, p< 0.0001) and between lymphopenia, a frequent abnormal parameter in the haematological domain, and ESSDAI and clinESSDAI the year after (6.5 (0.3) and 5.9 (0.4) vs 3.9 (0.1) and 3.8 (0.1) in patients without lymphopenia, p< 0.0001 and p< 0.0001, respectively).
Conclusion: Abnormalities in the biological or haematological domain of the ESSDAI, prevalent in nearly one half or one fourth of patients, predict a significant increase in systemic disease score the year after. Therefore, in pSS, some routinely assessed parameters, such as serum IgG levels or lymphocyte blood count, can be considered, not only as part of this systemic disease activity score, but also as relevant biomarkers of future systemic disease activity in common practice.
To cite this abstract in AMA style:Gottenberg JE, Seror R, Saraux A, Devauchelle V, Dernis E, Dieudé P, Dubost JJ, Fauchais AL, Goeb V, Larroche C, Le-Guern V, Hachulla E, Hatron PY, Morel J, Perdriger A, Rist Bouillon S, Sène D, Vittecoq O, Sibilia J, Ravaud P, Mariette X. Abnormalities in the Biological or Haematological Domain of the Essdai Predict an Increase in Systemic Disease Activity the Year after: 5-Year Data from the Prospective Multicenter Assess Cohort [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/abnormalities-in-the-biological-or-haematological-domain-of-the-essdai-predict-an-increase-in-systemic-disease-activity-the-year-after-5-year-data-from-the-prospective-multicenter-assess-cohort/. Accessed March 21, 2023.
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