Background/Purpose: Primary Sjögren syndrome (pSS) is a systemic autoimmune disease involving mainly the exocrine glandular system. Nevertheless, its clinical spectrum includes the development of multiple extra-glandular manifestations that will be determinant for the prognosis and the use of systemic therapy, including biological treatments. The aim of our study was to describe the biological treatments used in a pSS cohort of patients assisted in Spanish Rheumatology Departments.

Methods: This is a multicenter descriptive transversal study of pSS patients fulfilling European/American consensus criteria. Patients were included by randomization from thirty-three Rheumatology departments. Data were collected by reviewing clinical records and interviewing the patients. Signed informed consent was obtained and local ethics committees approved the study. Variables were analyzed by descriptive statistical methods, using means, medians, and rates. Chi-square was used to establish the statistical associations, being considered significant a p<0.05.

Results: Four hundred and thirty-seven patients were included. Ninety-five percent of them were women. The median age of the cohort was 58 years. Ten patients had been treated (currently or previously) with anti-TNF agents (2.29%); 31 patients had been treated with rituximab (7.1%); 3 patients had been treated with abatacept (0.69%) and 1 patient had been treated with tocilizumab (0.23%). Patients receiving anti-TNF agents had the following clinical manifestations: articular involvement (4 patients), pulmonar involvement (2 patients), central nervous system (CNS) involvement (4 patients) and cytopenia (2 patients). Patients receiving rituximab had: articular involvement (13 patients), pulmonar involvement (9 patients), renal involvement (6 patients), CNS involvement (5 patients), peripheral nervous system (PNS) involvement (8 patients) and cytopenia (16 patients). Patients receiving abatacept had: articular involvement (1 patient), pulmonar involvement (1 patient), CNS involvement (1 patient) and cytopenia (1 patient). The patient who received tocilizumab had: CNS involvement and cytopenia. All patients receiving biological therapy were ANA positive. Sixty percent of the patients receiving anti-TNF were anti-Ro+; 40% of the patients had anti-La; 10% of the patients had low C3 and C4, respectively; and 30% of the patients had hypergammaglobulinemia. Ninety-four percent of the patients receiving rituximab were anti-Ro+; 61% of the patients had anti-La; 29% had low C3 and C4, respectively; and 64% of the patients had hypergammaglobulinemia. Thirty-three percent of patients receiving abatacept were anti-Ro+; 33% of the patients had anti-La; 33% of the patients had low C3; and 66.7% of the patients had hypergammaglobulinemia. The patient who received tocilizumab was anti-Ro+, anti-La+ and had hypergammaglobulinemia. Hospitalization due to pSS activity was needed in 5 patients receiving anti-TNF agents; in 19 patients receiving rituximab; in 2 patients receiving abatacept and in the patient treated with tocilizumab. Biological treatments were significantly used more frequently in patients with a hospitalization for disease activity (p<0.05). The mean of EULAR Sjogren´s Syndrome Disease Activity Index (ESSDAI) was 8.78 in patients receiving anti-TNF agents; 10.39 in patients receiving rituximab; 11 in patients receiving abatacept and 14 in the patient treated with tocilizumab.

Conclusion: Despite the absence of clinical controlled studies demonstrating efficacy and safety, biological frequently used in patients with SSp. The most widely used biological agent is rituximab. Its use is associated with the presence of musculoskeletal, neurological, pulmonary and haematological manifestations, hospitalization for disease activity and a high ESSDAI.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/biological-treatments-in-primary-sjogren-syndrome/