Table 1.
B cell-targeted therapies in SS patients.
| Drug | Target | Dose | No. of Pats | Type of Study | Efficacy | Side Effects | Refs |
|---|---|---|---|---|---|---|---|
| Rituximab | Chimeric mAb against CD20 | Twice 1 g on days 1 and 15 | 17 | Randomized, double-blind, Placebo-controlled pilot study | Improvement after 6 months, sicca symptoms did not improve | IRR, SSR | [196] |
| 1 g with an interval of 2 weeks or placebo | 30 | Prospective, single center, randomized, double-blind, placebo-controlled trial | Stimulated saliva flow rate and lacrimal gland function improvement | SSR | [197] | ||
| 375 mg/m2/week for 4 weeks or 1 g on days 1 and 15 | 78 | Prospective study (AIR registry) | 1st cycle efficacy in 47 patients (60 %) After 6 m ESSDAI decrease |
IRR, SSR | [198] | ||
| 1 g with an interval of 15 days. patients received 6 courses of therapy | 41 | Prospective, multicenter, follow-up study | ESSDAI decrease. Reduction of infiltrate and GCs after treatment |
No adverse effects | [199] | ||
| Twice 1 g, 15 days apart | 28 | Prospective single-center study | ESSDAI and ESSPRI score improved. | Not reported | [200] | ||
| Twice 1 g, two weeks apart | 120 | Randomized, double-blind, Placebo-controlled, parallel-group trial (TEARS) |
No significant difference | Few patients had IRR | [201] | ||
| two doses of rituximab (1 g) or placebo, two weeks apart | 110 | A randomized double-blind placebo-controlled clinical trial | No significant difference | Not reported | [202] | ||
| Two courses of rituximab (1 g) at weeks 0, 2, 24, and 26 or placebo. | 133 | A multicenter, randomized, double-blind, placebo-controlled, parallel-group trial | No significant improvement in any outcome except unstimulated saliva flow | Few serious adverse events were reported but there were no deaths | [203] | ||
| Epratuzumab | Humanized anti-CD22 monoclonal antibody | 4 infusions of 360 mg/m2 biweekly | 16 | An open-label phase I/II study | Improvements in fatigue. B-cell reduction, T cells did not change | Not reported | [204] |
| 600 mg every week, or epratuzumab 1200 mg every other week for 4 weeks | 1584 | Randomized, double-blind, placebo-controlled, multicenter studies | Disease activity in patients with SLE and associated SS showed improvements | Adverse events were comparable in the treated and placebo group | [205] | ||
| Belimumab | Human IgG1ʎ mAb targeting BAFF | 10 mg/kg, monthly dose | 30 | Phase II open-label | In 60% of patients improvement in dryness, fatigue, and musculoskeletal pain | One patient develops pneumococcal meningitis | [206] |
| Ianalumab (VAY736) | a B cell-depleting, BAFF-R blocking, monoclonal antibody | single infusion at either 3 mg/kg, 10 mg/kg or placebo. | 27 | Double-blind, placebo-controlled, phase II, single-center study | Both doses lead to depletion of B cells for a long time | Moderate infusion related side effects | [207] |
| BAFF-R | Monthly s.c. doses (5, 50, 300 mg) or placebo. | 190 | Phase 2b Study | Primary endpoint achieved, improvement for 300 mg dose | Safety profile looked good | [208] | |
| Baminercept | Lymphotoxin-β receptor Fusion protein, reduces B cell infiltration | s.c. injections of 100 mg of baminercept every week for 24 weeks or placebo | 52 | Phase II multicenter, randomized, double-blind, placebo-controlled trial | No significant difference in ESSDAI, no difference in salivary gland secretion and ocular dryness | Higher incidence of liver toxicity | [209] |
This table displays B cell targeted therapies for SS. The table displays drugs and drug’s dose, targets number of patients (Pats), study type, efficacy, and side effects. Abbreviations: mAb—monoclonal antibody, CD20—cluster of differentiation 20, IRR—infusion-related reaction, SSR—serum sickness-related, AIR airway intervention registry, ESSDAI—the EULAR Sjögren’s syndrome disease activity index, ESSPRI the EULAR SS patient reported index, MSG—minor salivary gland, TEARS -tolerance and efficacy of Rituximab in primary SS, SLE—Systemic lupus erythematosus, BAFF—B-cell activating factor.