Immune-mediated axonal dysfunction in seropositive and seronegative primary Sj?gren's syndrome.

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Title:	Immune-mediated axonal dysfunction in seropositive and seronegative primary Sj?gren's syndrome.
Authors:	宋家瑩 Jowy Tani, Hsien-Tzung Liao, Hui-Ching Hsu, Lung-Fang Chen, Tsui-San Chang, Cindy Shin-Yi Lin, Jia-Ying Sung
Contributors:	醫學系神經學科
Date:	2020-05
Issue Date:	2025-03-28 16:42:43 (UTC+8)
Abstract:	Abstract Objective: The present study investigates the peripheral neuropathy in Primary Sj?gren's syndrome (pSS) using the nerve excitability test to further elucidate how peripheral nerves are affected by the autoantibodies. Methods: Each patient received clinical evaluation, examination for anti-SSA/Ro and anti-SSB/La antibodies titer, paired motor and sensory nerve excitability test, thermal quantitative sensory test (QST), and nerve conduction study (NCS). Results: A total of 40 pSS patients wasenrolled. Motor axonal study of the pSS with positive anti-SSA/Ro or anti-SSB/La antibodies (n = 28) was found to have increased stimulus for 50% compound muscle action potential (CMAP) (P < 0.05), increased rheobase (P < 0.01), increased minimum I/V slope (P < 0.01) and hyperpolarizing I/V slope (P < 0.05), increased relative refractory period (RRP, P < 0.001), decreased accommodation of threshold electrotonus toward depolarizing current (P < 0.05), and increased accommodation toward hyperpolarizing current (P < 0.05). Seronegative pSS (n = 10) showed much less prominent motor axonal changes, showing only increased minimum I/V slope (P < 0.05). Sensory axonal study in seropositive pSS patients is found to have increased stimulus for 50% sensory nerve action potential (SNAP) (P < 0.01), decreased latency (P < 0.01), increased RRP (P < 0.01), and increased subexcitability (P < 0.05). Seronegative pSS patients have shown no significant sensory axonal changes. Thermal QST showed more prominent abnormalities in seronegative pSS compared to seropositive pSS. Interpretation: Anti-SSA/Ro and anti-SSB/La autoantibodies might cause dysfunction in nodal and internodal region of the axon and small nerve fibers; meanwhile, autoreactive antibodies in seronegative pSS mainly affect small nerve fibers. Thus, the underlying pathophysiology for the two types of pSS is different.
Relation:	Ann Clin Transl Neurol. 2020 May ; 7(5) : 819-828
Description:	【111-2 升等】臺北醫學大學教師升等專門著作職別：專任教師送審等級：教授著作送審
Data Type:	article
Appears in Collections:	[Scholarly output for promotion] 111 [Department of Neurology] Periodical Article

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