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.

Tincani et al.

Tincani et al. BMC Medicine 2013 11:93   doi:10.1186/1741-7015-11-93

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