Diagnosis and treatment of primary Sjögren syndrome-associated peripheral neuropathy: a six-case series.
OBJECTIVE
The clinical and therapeutic aspects of primary Sjögren 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 of SS when patients present with peripheral neuropathy.
Division of Rheumatic Diseases, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinju-ku, Tokyo, 162-8655, Japan, hiroyuki_yjp2005@yahoo.co.jp.
Hiroyuki
H
Yamashita
Toshiki
T
Eri
Yo
Y
Ueda
Takashi
T
Ozaki
Hiroyuki
H
Takahashi
Takahiro
T
Tsuno
Yuko
Y
Takahashi
Toshikazu
T
Kano
Akio
A
Mimori
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D004568:Electrodiagnosis; D005073:Evoked Potentials, Somatosensory / Q000502:physiology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009431:Neural Conduction / Q000502:physiology; D010523:Peripheral Nervous System Diseases / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy; D012189:Retrospective Studies; D012859:Sjogren's Syndrome / Q000150:complications* / Q000503:physiopathology