Background/Purpose: Aim of this study was to assess indications, effectiveness, clinical and ultrasonographic response to rituximab (RTX) therapy in primary Sjögren’s syndrome (pSS), focusing in particular on the safety of multiple courses of B-cell depletion therapy .

Methods: Out of a single centre cohort of 378 pSS patients (AECG 2002) we retrieved the clinical charts of patients treated with RTX, focusing particularly on those that received multiple courses. In addition to patients’ demographics, clinical and serological features, we analyzed the following data: indications for RTX therapy, changes in the ESSDAI, lab parameters and in salivary gland ultrasonography (SGUS), number of RTX courses, regimens, time to re-treatment and adverse events.

Results: We included in the study 34 patients (M/F = 3/31) who received RTX during their disease course for the following indications: lymphoproliferative disease (16/34), cryoglobulinemic vasculitis (7/34), systemically active disease 5≤ ESSDAI<13 with at least 1 extraglandular domain moderate (7/34), and highly active disease in the haematological (2/34), articular (1/34) or pulmonary (1/34) ESSDAI domains. B-depletion therapy resulted in a significant reduction of disease activity (p <0.05) producing a partial response only for lung involvement. SGUS was performed in 7 patients, showing a significant improvement of the score in one case and stable findings in the others. Six female received at least a second infusion of RTX: in 3/6 only a second cycle was administered “on demand” (after 1, 3 and 4 years, respectively), in the other 3 cases multiple maintenance courses were administered (6, 6 and 11, respectively). Each course of re-treatment consisted in 1 or 2 g bi-weekly infusion; median interval between courses was 9 months (IQR 6-31.5). Multiple courses of RTX resulted in significant improvement of ESSDAI (median ESSDAI before RTX re-treatment =13.5 (IQR 11.25-25.25) vs median ESSDAI post-retreatment =3.5 (IQR 2-5), p=0.008). Re-treatment with RTX was well-tolerated in five cases; one patient developed allergic drug reaction. Infectious events included: recurrent urinary tract infection (2/6), skin infection and Herpes Zoster reactivation (1/6). One patient who received multiple courses of RTX (in association with plasma-exchange therapy) developed a severe hypogammaglobulinemia complicated with recurrent infections that required Ig replacement therapy.

Conclusion: Single or multiple courses of B-depletive therapy with RTX seem to be effective, relatively safe and tolerated in pSS. Allergic reactions and infections remain still an issue. Further studies are needed to optimize repeated depletion therapy with RTX for pSS remission maintenance.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/b-lymphocyte-depletion-therapy-with-rituximab-in-primary-sjogrens-syndrome-indications-effectiveness-and-ultrasonographic-response/