Abstract
Neurological involvement occurs in approximately 20% of patients with primary Sjögren’s syndrome. Although neurological symptoms can affect the peripheral nervous system and the central nervous system, the most frequent symptom is polyneuropathy. Small fiber neuropathy (SFN) is a form of painful peripheral polyneuropathy that is common in patients with diabetic neuropathy, but may also occur in toxic, infectious, or immune-mediated neuropathy. We show here a patient with Sjögren’s syndrome who developed SFN and was treated with intravenous immunoglobulin (IVIG) therapy, which was immediately and extremely effective. Because of the efficacy of IVIG therapy, we propose that direct immune-mediated mechanisms may be involved in the pathogenesis of SFN complicated by Sjögren’s syndrome.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Mori K, Iijima M, Koike H, Hattori N, Tanaka F, Watanabe H, et al. The wide spectrum of clinical manifestations in Sjögren syndrome-associated neuropathy. Brain. 2005;128:2518–34.
Gorson KC, Ropper AH. Positive salivary gland biopsy, Sjögren syndrome, and neuropathy: clinical implications. Muscle Nerve. 2003;28:553–60.
Hoitsma E, Reulen JP, de Baets M, Drent M, Spaans F, Faber CG. Small fiber neuropathy: a common and important clinical disorder. J Neurol Sci. 2004;227:119–30.
Al-Shekhlee A, Chelimsky TC, Preston DC. Review: small-fiber neuropathy. Neurologist. 2002;8:237–53.
Chai J, Herrmann DN, Stanton M, Barbano RL, Loqiqian EL. Painful small-fiber neuropathy in Sjögren syndrome. Neurology. 2005;65:925–7.
Vitali C, Bambardieri S, Jonsson R, Moutsopoulos HM, Alexander EL, Carsons SE, et al. Classification criteria for Sjögren’s syndrome: a revised version of the European criteria proposed by the American–European Consensus Group. Ann Rheum Dis. 2002;61:554–8.
Gøransson LG, Herigstad A, Tjensvoll AB, Harboe E, Mellqren SI, Omdal R. Peripheral neuropathy in primary Sjögren’s syndrome. A population-based study. Arch Neurol. 2006;63:1612–5.
Delalande S, de Seze J, Fauchais AL, Hachulla E, Stojkovic T, Ferriby D, et al. Neurologic manifestations in primary Sjögren’s syndrome. A study of 82 patients. Medicine (Baltimore). 2004;83:280–91.
Takahashi Y, Takata T, Hoshino M, Sakurai M, Kanazawa I. Benefit of IVIG for long-standing ataxic sensory neuropathy with Sjögren’s syndrome. Neurology. 2003;60:503–5.
Kizawa M, Mori K, Iijima M, Koike H, Hattori N, Sobue G. Intravenous immunoglobulin treatment in painful sensory neuropathy without sensory ataxia associated with Sjögren syndrome. J Neurol Neurosurg Psychiatry. 2006;77:967–9.
Levy Y, Uziel Y, Zandman GG, Amital H, Sherer Y, Langevitz P, et al. Intravenous immunoglobulins in peripheral neuropathy associated with vasculitis. Ann Rheum Dis. 2003;62:1221–3.
van Doorn PA, Rossi F, Brand A, van Lint M, Vermeulen M, Kazatchkine MD. On the mechanism of high-dose intravenous immunoglobulin treatment of patients with chronic inflammatory demyelinating polyneuropathy. J Neuroimmunol. 1990;29:57–64.
Saoudi A, Hurez V, de Kozak Y, Huhn J, Kaveri SV, Kazatchine MD, et al. Human immunoglobulin preparations for intravenous use prevent experimental autoimmune uveoretinitis. Int Immunol. 1990;5:1559–67.
Anderson CL. Human IgG Fc receptor. Clin Immunol Immunopathol. 1989;53:S63–71.
Conflict of interest statement
None.
Author information
Authors and Affiliations
Corresponding author
About this article
Cite this article
Wakasugi, D., Kato, T., Gono, T. et al. Extreme efficacy of intravenous immunoglobulin therapy for severe burning pain in a patient with small fiber neuropathy associated with primary Sjögren’s syndrome. Mod Rheumatol 19, 437–440 (2009). https://doi.org/10.1007/s10165-009-0180-2
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10165-009-0180-2