Vol.19 No.4

Case Report

Extreme efficacy of intravenous immunoglobulin therapy for severe burning pain in a patient with small fiber neuropathy associated with primary Sjögren’s syndrome

Authors

Daisuke Wakasugi1 , Takashi Kato1 , Takahisa Gono1 , Eiichi Ito2 , Hiroyuki Nodera3 , Yasushi Kawaguchi1 , Hisashi Yamanaka1 , Masako Hara1

  • Department of Rheumatology, Tokyo Women’s Medical University, 10-22 Kawada-cho, Shinjuku-ku, Tokyo 162-0054, Japan
  • Department of Neurology, Tokyo Women’s Medical University, 8-1 Kawada-cho, Shinjuku-ku, Tokyo 162-8666, Japan
  • Department of Neurology, The University of Tokushima, 3-8-15 Kuramoto-cho, Tokushima 770-8503, Japan
Received:

26 January 2009

Accepted:

7 April 2009

Published online:

22 May 2009

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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.

Key words

Sjögren’s syndrome - Small fiber neuropathy - Burning pain - Intravenous immunoglobulin