Table 1

 Main characteristics of 16 patients with primary Sjögren’s syndrome, treated with rituximab (first treatment)

PatientSex/age/disease duration (years)pSS status: anti-SSA/SSB antibodies/Chisolm–Mason scorePrior Immunosuppressive treatmentsClinical involvement at the beginning of RTXNumber of infusions × doseAssociated immunosuppressor treatmentsAdverse event (Y/N)Efficacy for lymphomaEfficacy for systemic featuresEfficacy for dryness ocular/oral (Y/N)Follow-up(months)Prednisone (mg/day) first/lastRF (IU/l)first/lastγ-globulinaemia (g/l) first/last
SubjectiveObjective
AZA, azathioprine; CHOP, cyclophosphamide, doxorubicin, vincristine, prednisone; CPH, cyclophosphamide; d-peni, d penicillamine; ETA, etanercept; F, female; HQ, hydroxy-chloroquine; IFLX, infliximab; IRR, infusion-related reaction; iv, intravenous; Leflu, leflunomide; MALT, mucosa-associated lymphoid tissue; MMF, mycophenolate mofetil; MTX, methotrexate; N, no; NA, not available; NR, not relevant; pSS, primary Sjögren’s syndrome; RF, rheumatoid factor; RTX, rituximab; SSA, Sjögren’s syndrome antigen; SSB, Sjögren’s syndrome B; SSR, serum sickness-like reaction; SZP, salazopyrine; Y, yes.
*Four patients had already been reported in a previous study.11
1F/59/25SSA SSB/IVNoPolysynovitis and glandular swelling (cutaneous nodules)4×375 msNoY(IRR)NRYN/NN/N1530/01040/41535.4/26.7
Persitant keratitis
Salivary flow = 0
2F/44/10SSA SSB/IVHQ/MTX/Pulmonary, renal4×375 msNoNNRYN/NN/N1730/101250/49987/38
Leflu/IFLX/involvements andStable Schirmer
ETA/MMFpolysynovitisSalivary flow = 0
3*F/43/4SSA SSB/NANoSalivary lymphoma (MALT)4×375 msNoY(SSR)NNRN/NNA/NA140/0499/42331/26
4F/71/100/IIIMTXNodal marginal zone lymphoma4×375 msNoY(IRR)YNRY/NY/N130/031.5/015.8/13.6
Regression of keratitis
Stable salivary flow
5*F/71/5SSA/IVCPH/HQ/Cryoglobulinaemia4×375 msNoNNRYN/NN/N2730/7.5109/1138.8/4.6
AZAStable Schirmer
Salivary flow = 0
6F/53/13SSA SSB/IVCPH/AZACryoglobulinaemia4×375 msNoNNRYN/NN/N2115/055/59.2/7
Stable Schirmer
Salivary flow = 0
7F/67/18SSA/NACPHGastric, pulmonary lymphoma (MALT)6×375 msMini-CHOPNYNRN/NNA/NA487.5/7.5270/08.6/7.5
8*F/58/15SSA/IVHQGastric lymphoma (MALT)4×375 msHQNYNRY/NY/NA2417.5/544/010.7/6.5
Regression of keratitis
9F/54/4SSA SSB/IVCPH/MMFMononeuritis multiplex4×375 msNoNNRYY/YN/NA138/7139/2213.4/9.6
Stable Schirmer
10F/69/12No/IIIHQ/MTX/Leflu/D-peni/IL1RAPolysynovitis4×375msLefluNNRYN/NN/NA1210/7.50/024.9/14
Stable Schirmer
11F/63/6SSA SSB/IVSZP/goldsaltsImmune mediated thrombocytopenia4×375 msNoNNRNN/NN/N85/544/2237.5/26
Stable Schirmer
Salivary flow = 0
12*F/58/2SSA/NACPHCryoglobulinaemia4×375 msNoNNRYNA/NANA/NA2510/0170/04.8/4.6
13F/41/9No/IVCHP/AZA/Diffuse large B cell lymphoma4×375 msCHOPNYNRY/NN/N1510/0NA/02.3/4.0
MTX/HCQStable Schirmer
Salivary flow = 0
14F/66/20No/IVCPH/AZA/Cryoglobulinaemia4×375 msNoNNRYN/NN/N1230/10NA/NA4.5/5.3
MTXWorsened Schirmer
Salivary flow = 0
15F/66/0SSA/IVNoCryoglobulinaemia4×375 msNoNNRNY/NANA/NA140/0512/NANA/NA
16F/57/3SSA/IIHQ/MTXPolysynovitis and pulmonary involvement2×1 gNoNNRYNA/NANA/NA230/50/016.7/15.2