IVIg for Sjogren's Neuropathy: Worth the Price?

Experience of Intravenous Immunoglobulin Therapy in Neuropathy Associated With Primary Sjögren's Syndrome: A National, Multicentric Retrospective Study

Rist S, Sellam J, Hachulla E, et al
Arthritis Care Res (Hoboken). 2011;63:1339-1344

Study Summary

Background. Peripheral and central neurologic disease may be clinical manifestations of primary Sjögren syndrome (SS). Mechanistically, SS-related neuropathy may be a consequence of vasculitis (more likely if motor neuropathy is present) or other, less well-elucidated processes, such as autoantibody-mediated nerve dysfunction.^[1] If vasculitis is the suspected cause of SS-related neuropathy, treatment typically entails potent immunosuppressive agents. Treatment for nonvasculitic SS-related neuropathy is less clear, although a variety of therapies, including corticosteroids and traditional immunosuppressive agents as well as plasmaphoresis and intravenous immunoglobulin (IVIg), have been reported in the literature. However, data to support the use of IVIg for treatment of nonvasculitic neuropathy are limited; therefore, Rist and associates sought to identify the efficacy and safety of IVIg in patients whose SS-related neuropathy was not caused by necrotizing vasculitis.

Methods. Questionnaires were mailed to 1400 French practitioners from a participating network to identify patients with SS who had received ≥ 1 dose of IVIg for peripheral neuropathy. Through this mechanism, the investigators identified 19 patients with primary SS-related, presumed nonnecrotizing vasculitic neuropathy who had been treated with IVIg. Outcomes of therapy were measured using the patient-completed Modified Rankin Score (MRS), which designates the degree of disability on the basis of symptoms and a global assessment by the care provider.

The mean age of the 19 patients was 60 years; 58% were women, and all fulfilled the American-European Consensus Group criteria for SS.^[2] They had a variety of neuropathies classified as sensorimotor (n = 5), ataxic (n = 9), non-ataxic sensory polyneuropathy (n = 4), and conduction block neuropathy (n = 1). Of note, ataxic neuropathy has been defined as impairment of position sense, with preserved muscle power and motor nerve function.^[3] Six patients had nerve biopsies, with 5 showing nonnecrotizing leukocytoclastic vasculitis or lymphocytic infiltrates, and 1 was normal. The IVIg was typically administered in monthly infusions of 2 g/kg over either 2 or 5 days, and the median duration of IVIg treatment was 7 months (range, 2-32 months); follow-up ranged from 3 to 84 months. Various other immunosuppressive agents (steroids, cyclophosphamide, azathioprine, mycophenolate mofetil, hydroxychloroquine, and rituximab) were used either before or along with IVIg.