Table 2.
Studies including patients affected with Sjögren’s syndrome (SS) treated with rituximab
| Author, year and reference | No. of patients | Study design | Involvement | Efficacy | Safety |
|---|---|---|---|---|---|
| Somer et al., 2003 [109] |
1 |
Case report |
Marginal zone lymphoma, xerophthalmia, xerostomia |
Improvement in corneal staining, Schirmer’s test, salivary flow rate, tear production, salivary pooling, diminished parotid enlargement |
No AE reported |
| Voulgarelis et al., 2004 [110] |
4 |
Case reports |
Marginal zone lymphoma, parotid gland enlargement, lymphadenopathy, cryoglobulinemia, purpura, peripheral neuropathy, arthralgia |
Improvement in lymphoma, arthralgia, cryoglobulinemia, purpura, peripheral neuropathy (50%) |
No AE reported |
| Gottenberg et al., 2005 [111] |
6 |
Retrospective |
2 MALT lymphomas, 2 vasculitis with cryoglobulinemia, 2 parotid gland enlargement and articular involvement |
Improvement in parotid swelling, subjective dryness, fatigue and vasculitis in 5 out of 6 patients |
1 serum sickness, 1 infusion reaction |
| Pijpe et al., 2005 [112] |
15 |
Open label |
8 early primary SS and 7 MALT lymphoma: 8 parotid gland swelling, 8 Raynaud’s phenomenon, 13 fatigue, 11 arthralgia, 2 pulmonary involvement, 2 vasculitis |
Remission of lymphoma in 3 of 7 patients, disease stability in 3 of 7, progression in 1. Increased salivary secretion, improvement in the rose Bengal score and tear, BUT, mouth dryness, arthralgia. No improvement on Schirmer test. |
2 infusion reactions, 1 Herpes zoster |
| Ring et al., 2006 [113] |
1 |
Case report |
Distal renal tubular acidosis, xerostomia with mouth ulcerations |
Xerostomia improvement |
No AE reported |
| Seror et al., 2007 [114] |
16 |
Retrospective |
5 lymphoma, 2 pulmonary involvement, 2 polysynovitis, 5 mixed cryoglobulinemia, 1 thrombocytopenia, and 1 mononeuritis multiplex |
Remission of lymphoma in 4 of 5 patients; improvement of systemic involvements in 9 of 11, subjective dryness in 5 of 16, and regression of keratitis in 2 of 11. No response on salivary flow and Schirmer test. |
2 infusion reactions, 1 serum sickness |
| Devauchelle-Pensec et al., 2007 [115] |
16 |
Open label |
Sicca symptoms, pain, fatigue; 1 pulmonary involvement |
Improvement in subjective fatigue, pain, dryness, and pulmonary involvement. No changes in unstimulated salivary flow, salivary gland score and ophthalmologist evaluation. |
3 infusion reactions, 1 serum sickness |
| Dass et al., 2008 [116] |
8 (PL 9) |
RCT |
Fatigue, ocular and mouth dryness; no systemic involvement |
Improvement in fatigue, general health and SF-36. No improvement on Schirmer test and salivary flow rate. |
2 infusion reactions, 1 delayed reaction with meningism, 1 gastroenteritis and palpitation |
| Galarza et al., 2008 [117] |
8 |
Open label |
Severe glandular and musculoskeletal involvement, cutaneous vasculitis |
Improvement in parotid swelling, articular involvement, fatigue, and subjective dryness in 4 of 7 patients |
3 AE: 2 infusion reactions |
| Ramos-Casals et al., 2010 [118] |
15 |
Registry |
6 lymphoma, 4 neurological involvement, 2 hematological involvement, 1 refractory glomerulonephritis, arthritis, and protein-losing enteropathy |
Complete response in 67% of patients, partial response in 20%, no response in 13% |
1 urinary tract infection, 1 interstitial pneumonitis |
| Meijer et al., 2010 [119] |
20 (PL 10) |
RCT |
15 arthralgia, 6 arthritis, 2 renal involvement, 1 peripheral neuropathy, 11 Raynaud’s phenomenon, 17 tendomyalgia, 6 vasculitis, thyroid dysfunction |
Improvement in saliva flow rate, stimulated lacrimal gland function; but not in BUT and Schirmer test. Improvement in SF-36 and MFI. Improvement in extraglandular manifestations. |
1 serum sickness, 12 infections in 11 patients on rituximab vs 7 infections in 4 patients on placebo |
| Mekinian et al., 2012 [120] | 17 | Registry | All peripheral nervous system involvement: 10 patients with cryoglobulinemia and/or vasculitis, 7 patients without cryoglobulinemia and/or vasculitis | Response in 9 of 10 patients with cryoglobulinemia and/or vasculitis, and in 2 of 7 without cryoglobulinemia and/or vasculitis | 6 (35%) AE: 2 mild arterial hypertension, 1 infusion reaction, 1 cutaneous infection, CMV infection, hypogammaglobulinemia |
AE, adverse event; BUT, break-up time; CMV, cytomegalovirus; MALT, mucose-associated lymphoid tissue; MFI, multidimensional fatigue inventory; PL, placebo; RCT, randomized controlled trial; SF-36, Short Form 36 Health Survey.