Drs. Rist and Sellam contributed equally to this work.
Experience of intravenous immunoglobulin therapy in neuropathy associated with primary Sjögren's syndrome: A national multicentric retrospective study
Version of Record online: 29 AUG 2011
Copyright © 2011 by the American College of Rheumatology
Arthritis Care & Research
Volume 63, Issue 9, pages 1339–1344, September 2011
How to Cite
Rist, S., Sellam, J., Hachulla, E., Sordet, C., Puéchal, X., Hatron, P.-y., Benhamou, C.-l., Sibilia, J., Mariette, X. and Club Rhumatismes et Inflammation (2011), Experience of intravenous immunoglobulin therapy in neuropathy associated with primary Sjögren's syndrome: A national multicentric retrospective study. Arthritis Care Res, 63: 1339–1344. doi: 10.1002/acr.20495
- Issue online: 29 AUG 2011
- Version of Record online: 29 AUG 2011
- Accepted manuscript online: 16 MAY 2011 11:46AM EST
- Manuscript Accepted: 20 APR 2011
- Manuscript Received: 15 DEC 2010
Sjögren's syndrome (SS)–related peripheral neuropathy is responsible for disability, but no treatment has been shown to improve its outcome. In some cases, intravenous immunoglobulin (IVIG) therapy has been associated with some benefit. In this study, we investigated the effectiveness of IVIG in SS-related peripheral neuropathy.
We assessed the efficacy and tolerance of IVIG in 19 patients with primary SS–related neuropathy without any necrotizing vasculitis in a retrospective national multicentric study. The evaluation of the response was assessed using the disability Modified Rankin Scale (MRS) and a global evaluation by the practitioner.
Eight patients (42%) exhibited a decrease of the MRS score corresponding to a clinical improvement, 10 patients (52%) exhibited a stable MRS score, and 1 patient (6%) showed an increase of MRS score. According to the global evaluation by the practitioner, 9 (47%) of the 19 patients were improved, 6 patients (31%) were stable, and 4 patients (21%) worsened. All the patients with sensorimotor (n = 5) or nonataxic sensory neuropathy (n = 4) were improved or stabilized. However, among the patients with ataxic neuropathy (n = 9), only 2 improved and 4 worsened. Ten of the 13 patients treated with corticosteroids could have had the prednisone dosage decreased from 15 mg/day (range 7.5–60) to 10 mg/day (range 5–20) with IVIG. Only 1 patient stopped the treatment after 1 dose because of a minor side effect and lack of initial efficacy.
IVIG may be useful in the treatment of SS-associated sensorimotor neuropathies or nonataxic sensory neuropathy without any necrotizing vasculitis. The benefit of such therapy in the SS-related ataxic neuropathy seems less clear.