Background/Purpose

Primary Sjögren’s syndrome (pSS) is a systemic autoimmune disease, where musculoskeletal manifestations are usually treated by symptomatic measures and different conventional DMARDs by off-label use. Cyclosporine A (CyA), as an approved DMARD for treatment of arthritic joint involvement in different rheumatic disorders, is mainly used for local treatment of keratoconjunctivitis sicca in pSS, whereas reports on systemic effects are limited to small cohorts so far. Since low-dose CyA has shown promising effects and improved tolerability in different autoimmune disorders, the aim of this pilot study was to investigate the effects of a reduced dosage on articular involvement in patients with pSS. 

Methods

For this single-center, open-label, non-controlled phase II trial n=30 patients with pSS (29 females, average age 54.9 years, mean disease duration of 6 years) fulfilling the European-American Classification Criteria were included. All patients had active articular involvement with a minimum of 3 tender and/or 3 swollen joints. Oral NSAIDs and systemic steroids (£10mg/d prednisone equivalent) were permitted at stable doses for at least 4 weeks prior to inclusion. In 18 patients, at least one DMARD (not CyA) had failed prior to study entry. Primary endpoint was to evaluate the therapeutic effects of low-dose CyA (2mg/kg divided in two intakes a day) on articular involvement (reduction of tender and swollen joint counts) after 16 weeks of treatment.

Results

Introduction of low-dose CyA improved significantly joint involvement in patients with pSS. At week 16, the DAS28 decreased from 5.1 to 4.2 (p=0.003), the Tender Joint Count (out of 68) decreased from 17±13 to 10±11 (p=0.003), the Swollen Joint Count (out of 66) decreased from 3±3 to 1±3 (p<0.001) Furthermore, a clear treatment effect was observed on overall disease activity by the EULAR Sjögren’s Syndrome Disease Activity Index (ESSDAI) reduction from 5.3±3.3 to 3.0±3.1 (p=0.001). Ultrasonography of the three predominanty affected joints confirmed improvement of joint effusion and synovitis (p<0.05) at week 16. There was no significant effect on HAQ or sicca-symptoms measured by Schirmer- and Saxon-tests. Overall, the treatment was well tolerated and adverse events were consistent with the known safety profile of CyA (e. g. hypertension, headache, grade I creatinine increase).

Conclusion

In this pilot-study, promising effects of low-dose CyA treatment on articular involvement and disease activity were observed in patients with pSS. The safety profile was comparable to known side effects of CyA.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/effectiveness-and-safety-of-low-dose-cyclosporine-a-in-patients-with-primary-sjogrens-syndrome-pss-with-articular-involvement-results-of-a-pilot-study/