International Journal of Neurology and Brain Disorders | 2015-08-03

Efficacy of intravenous immunoglobulin for small fiber neuropathy associated with primary Sjögren syndrome: a pilot study

AL Fauchais

Abstract

Objectives The specific treatment of primary Sjögren syndrome (pSS) related small fiber neuropathy (SFN) is actually non-codified. Neuropathic pain is usually resistant to conventional analgesics but might partially respond to anticonvulsive therapy and/or antidepressants, and few case reports deal with the inconstant efficiency of corticosteroid or immunomodulatory drugs in pSS-associated SFN. Methods To evaluate IVIg (intravenous immunoglobulin) efficiency in the treatment of intractable neuropathic pain associated with SFN in pSS, pain intensity, characteristics, localization, interferences with daily activities and sleep, analgesic consumption were evaluated before and after IVIg treatment. Results We describe five patients with SFN related to pSS who developed intractable neuropathic pain despite treatment with corticosteroid (n=4) and analgesics (n=5). Skin biopsy findings demonstrated SFN in all five patients. All patients were treated by 2 g/kg of body weight of IVIg, administered over 2 to 4 days, every 4 weeks for 12 (n=4) to 16 weeks (n=1). Two patients, who had active signs of degeneration at the skin biopsy, have an improvement in pain (57%, 30% respectively) with a concomitant improvement of daily activities and quality of sleep. In three patients, pain was unchanged despite the IVIg infusions. Conclusion The efficiency of IgIV is inconstant in pSS related SFN and seems to correlate with active sign of neuro-degeneration; further studies are needed to identify anatomo-pathological markers of a clinical response to immunomodulative drugs.

Keywords: PrimarySjögren syndrome, Small fiberneuropathy, Intravenous immunoglobulin