Immune‐mediated axonal dysfunction in seropositive and seronegative primary Sjögren’s syndrome
Abstract Objective The present study investigates the peripheral neuropathy in Primary Sjögren'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 ant...
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| Format: | Article |
| Language: | English |
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Wiley
2020-05-01
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| Series: | Annals of Clinical and Translational Neurology |
| Online Access: | https://doi.org/10.1002/acn3.51053 |
| _version_ | 1818608389241962496 |
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| author | Jowy Tani Hsien‐Tzung Liao Hui‐Ching Hsu Lung‐Fang Chen Tsui‐San Chang Cindy Shin‐Yi Lin Jia‐Ying Sung |
| author_facet | Jowy Tani Hsien‐Tzung Liao Hui‐Ching Hsu Lung‐Fang Chen Tsui‐San Chang Cindy Shin‐Yi Lin Jia‐Ying Sung |
| author_sort | Jowy Tani |
| collection | DOAJ |
| description | Abstract Objective The present study investigates the peripheral neuropathy in Primary Sjögren'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. |
| first_indexed | 2024-12-16T14:41:52Z |
| format | Article |
| id | doaj.art-c421a0dcb32f4a6fac318f28a5085e57 |
| institution | Directory Open Access Journal |
| issn | 2328-9503 |
| language | English |
| last_indexed | 2024-12-16T14:41:52Z |
| publishDate | 2020-05-01 |
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| series | Annals of Clinical and Translational Neurology |
| spelling | doaj.art-c421a0dcb32f4a6fac318f28a5085e572022-12-21T22:27:53ZengWileyAnnals of Clinical and Translational Neurology2328-95032020-05-017581982810.1002/acn3.51053Immune‐mediated axonal dysfunction in seropositive and seronegative primary Sjögren’s syndromeJowy Tani0Hsien‐Tzung Liao1Hui‐Ching Hsu2Lung‐Fang Chen3Tsui‐San Chang4Cindy Shin‐Yi Lin5Jia‐Ying Sung6Department of Neurology Wan Fang Hospital Taipei Medical University Taipei TaiwanDivision of Allergy, Immunology and Rheumatology Department of Internal Medicine Taipei Veterans General Hospital Taipei TaiwanDivision of Allergy, Immunology and Rheumatology Department of Internal Medicine Wan Fang Hospital Taipei Medical University Taipei TaiwanDivision of Allergy, Immunology and Rheumatology Department of Internal Medicine Wan Fang Hospital Taipei Medical University Taipei TaiwanDepartment of Neurology School of Medicine College of Medicine Taipei Medical University Taipei TaiwanNeural Regenerative Medicine College of Medical Science and Technology Taipei Medical University and National Health Research Institutes Taipei TaiwanDepartment of Neurology Wan Fang Hospital Taipei Medical University Taipei TaiwanAbstract Objective The present study investigates the peripheral neuropathy in Primary Sjögren'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.https://doi.org/10.1002/acn3.51053 |
| spellingShingle | Jowy Tani Hsien‐Tzung Liao Hui‐Ching Hsu Lung‐Fang Chen Tsui‐San Chang Cindy Shin‐Yi Lin Jia‐Ying Sung Immune‐mediated axonal dysfunction in seropositive and seronegative primary Sjögren’s syndrome Annals of Clinical and Translational Neurology |
| title | Immune‐mediated axonal dysfunction in seropositive and seronegative primary Sjögren’s syndrome |
| title_full | Immune‐mediated axonal dysfunction in seropositive and seronegative primary Sjögren’s syndrome |
| title_fullStr | Immune‐mediated axonal dysfunction in seropositive and seronegative primary Sjögren’s syndrome |
| title_full_unstemmed | Immune‐mediated axonal dysfunction in seropositive and seronegative primary Sjögren’s syndrome |
| title_short | Immune‐mediated axonal dysfunction in seropositive and seronegative primary Sjögren’s syndrome |
| title_sort | immune mediated axonal dysfunction in seropositive and seronegative primary sjogren s syndrome |
| url | https://doi.org/10.1002/acn3.51053 |
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