To investigate the clinical efficacy and safety of low-dose rituximab (RTX) for patients in primary Sjögren's syndrome (pSS) with thrombocytopenia.
Four pSS patients, 2 with refractory thrombocytopenia and 2 with glucocorticoid-dependent thrombocytopenia, were treated with rituximab at 100 mg, intravenous, weekly for a total of two cycles, together with prednisone 1 - 2 mg×kg(-1)×d(-1), and the counts of platelets and B-cells were evaluated.
Efficacy of treatment was observed in all patients. The counts of platelets, at (3 - 39) × 10(9)/L baseline, increased in 1 - 2 weeks, and went up to (107 - 241) × 10(9)/L in 3 - 8 weeks. Sustained remission had been achieved for 27 - 52 weeks. The doses of prednisone were tappered to 3.75 - 7.50 mg/day in 12 weeks. One patient who relapsed at the 27th week (platelet count 47 × 10(9)/L), was retreated with 100 mg of RTX and still had good efficacy. The counts of B-cells reduced to (0.007 - 0.010) × 10(9)/L, but they did not achieved the depletion. There were no severe adverse events during RTX therapy.
Our study has shown good efficacy and tolerability of low-dose RTX for pSS with thrombocytopenia. Low-dose RTX allows for reduction in corticosteroid doses and B-cells, while large-scale randomized double-blind controlled trials are needed to confirm the results.