Anti‐CD20 treatment in Primary Sjögren’s Syndrome

Abstract

We performed a search for publications on rituximab (Rtx) in the treatment of primary Sjögren’s syndrome (pSS), and assessed the reports for the efficacy of the drug on complaints like sicca symptoms, systemic manifestations and pSS associated lymphoma. We also reviewed the effects on laboratory parameters and potential adverse effects. From the published literature there is little evidence supporting Rtx to have an effect on sicca symptoms, and there is particularly lack of objective improvements in measures of oral and ocular dryness. Systemic manifestations such as fatigue, synovitis, arthralgia, cryoglobulinaemia‐related vasculitis, neurological, renal and pulmonary involvement all seem to react favourably to Rtx treatment. The effect on pSS associated non‐Hodgkin’s lymphoma is also beneficial. Rheumatoid factor concentration is decreasing during Rtx treatment. The levels of anti‐SSA and ‐SSB antibodies are, however, unaltered according to the majority of the studies. The most common complications to Rtx treatment are mild and transient infusion related reactions. Delayed moderate‐to‐severe reactions are less common, and occur mostly in patients who develop human anti‐chimeric antibodies. In conclusion, Rtx is a promising treatment option for severe pSS with systemic complications, but more data from randomized controlled trials are warranted before conclusions on the drug’s role can be made with more accuracy.

Oral mucosal manifestations in primary and secondary Sjögren syndrome and dry mouth syndrome

“…It may show as a primary disease without any other accompanying symptoms or as a secondary disease appearing simultaneously with other autoimmune diseases such as rheumatoid arthritis, lupus erythematosus, dermatomyelitis, scleroderma, and primary cirrhosis of the liver. Sjögren syndrome also shows a tendency towards fibromyalgia, migraines, hypothyroidism, Raynaud symptom, and lymphomas [1][2][3][4][5][6][7][8][9][10][11][12]. One of the most bothering symptoms of the syndrome is xerostomia.…”

Systemic and Local Interleukin-17 and Linked Cytokines Associated with Sjögren’s Syndrome Immunopathogenesis

“…1,32 Correspondingly, anti-B-cell therapy has materialized as a potential therapy for glandular and extraglandular manifestations of pSS, as well as for management of lymphoma associated with pSS. 33 In comparison, IFN␥ mRNA, overexpressed in pSS MSG, 34 -36 is echoed by elevated IFN␥ protein in saliva. However, we do not detect heightened levels of IFN␥ by microarray, when comparing MSGs with advanced with mild disease (N. Moutsopoulos and S.M.…”

Treating Sjögren’s syndrome: insights for the clinician

“…Recently an additional randomized trial, which included a small number of patients without systemic manifestations, has shown a significant decrease of fatigue in patients treated with rituximab. It is worth noting that in these studies 10 20% of treated patients develop serum sickness disease some days after the infusion of rituximab [Dass et al 2008;Isaksen et al 2008;Mariette, 2008]. This is a largely described event after treatment with chimeric antibodies, due to the production of specific autoantibodies (human antichimeric antibodies [HACA]).…”

“…Rituximab, a chimeric monoclonal antibody, directed against CD20, a pan-B-cell surface antigen, which has been approved for the treatment of B-cell lymphoma and of anti-TNF-a refractory rheumatoid arthritis, has been tested in small open studies (Table 2) [Isaksen et al 2008;Mariette, 2008]. In addition to the expected effectiveness on the associated lymphoma, which was documented in part of the enrolled cases, rituximab demonstrated a clear effect on parotidomegaly and systemic manifestations, including vasculitis, arthritis, renal and lung disease [Seror et al 2007].…”