Vol.23 No.5

Original Article

Diagnosis and treatment of primary Sj?gren syndrome-associated peripheral neuropathy: a six-case series

Authors

Hiroyuki Yamashita¹ , Toshiki Eri¹ , Yo Ueda¹ , Takashi Ozaki¹ , Hiroyuki Takahashi¹ , Takahiro Tsuno¹ , Yuko Takahashi¹ , Toshikazu Kano¹ , Akio Mimori¹

Received:

6 July 2012

Accepted:

30 August 2012

Published online:

28 September 2012

Full Text

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Abstract

Objectives The clinical and therapeutic aspects of primary
Sjo¨gren syndrome (PSS) in patients with peripheral
neuropathy were analyzed and the specifics of individual
case studies are discussed.
Methods We retrospectively studied six patients (four
women, two men; mean age 64.5 years) presenting with PSS
with peripheral neurological involvement over a five-year
period (2008?2012). All patients had neurological examinations,
including nerve conduction studies, somatosensory
evoked potentials, and sural nerve biopsies. Treatment regimens
included corticosteroids, intravenous gammaglobulin,
or immunosuppressive treatment.
Results Peripheral neuropathy was observed in six
(7.9 %) of 76 patients with SS as the underlying disease;
three were cases of multiple mononeuropathy, two cases
had sensory ataxic neuropathy, one of which was autonomic
neuropathy, and one case was diagnosed as painful
sensory neuropathy without sensory ataxia. Four of the six
patients were diagnosed with SS after the onset of neurological
symptoms. Individual peripheral neuropathies had
distinct neurological, electrophysiological, and pathological
characteristics. The effect of steroids and intravenous
gammaglobulin differed depending on the case.
Conclusions In PSS patients, a precise diagnosis is
important, because the therapeutic strategy and response
varies depending on the type of neuropathy. In clinical
practice, it is important to consider a diagnosis

Key words

Sj?gren syndrome, Peripheral neuropathy, Corticosteroids, Intravenous gammaglobulin