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Original article| Volume 79, P58-62, September 2020

Differences in clinical features between small fiber and sensitive large fiber neuropathies in Sjögren's syndrome

      Abstract

      Background

      To distinguish large (LFN) and small fiber neuropathies (SFN) in Sjögren's syndrome (SS) requires electroneuromyography (EMG) first, but this is time-consuming and has sometimes a limited accessibility, which can lead to a diagnostic delay. We aimed to identify clinical features that could distinguish SFN from sensitive LFN in SS.

      Methods

      The study included patients with SS who were monitored in the internal medicine and neurology departments at Angers University Hospital between 2010 and 2016, and who were tested for suspected peripheral neuropathy. Patients with clinical motor involvement were excluded. LFN diagnosis was based on EMG. SFN diagnosis was based on intraepidermal nerve fiber density on skin biopsies in patients with no abnormality on EMG.

      Results

      LFN and SFN were diagnosed respectively in 22 (6.9%) and 17 (5.4%) patients among 317 patients with SS. Prevalence of anti-SSA antibodies was lower in the SFN group compared to the LFN group (p=0.002). The types of paresthesia did not differ between the 2 groups. After adjustment for age and sex, SFN was associated with dysautonomia (p=0.01, OR 8.4 [CI 95%: 1.7-42.4]) and without length-dependent topography (p=0.03, OR 0.2 [0.04-0.8] in comparison with the LFN group.

      Conclusions

      An association of non-length-dependent pattern and dysautonomia seems to predict the absence of LFN in SS and encourages the search for SFN. In contrary, patients with length-dependent involvement and without dysautonomia should be prioritized for EMG.

      Keywords

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      References

        • Seror R
        • Ravaud P
        • Bowman SJ
        • Baron G
        • Tzioufas A
        • Theander E
        • et al.
        EULAR Sjogren's syndrome disease activity index: development of a consensus systemic disease activity index for primary Sjogren's syndrome.
        Ann Rheum Dis. 2010; 69: 1103-1109https://doi.org/10.1136/ard.2009.110619
        • Birnbaum J.
        Peripheral nervous system manifestations of Sjögren syndrome: clinical patterns, diagnostic paradigms, etiopathogenesis, and therapeutic strategies.
        Neurologist. 2010; 16: 287-297https://doi.org/10.1097/NRL.0b013e3181ebe59f
        • Gøransson LG
        • Herigstad A
        • Tjensvoll AB
        • Harboe E
        • Mellgren SI
        • Omdal R
        Peripheral neuropathy in primary sjogren syndrome: a population-based study.
        Arch Neurol. 2006; 63: 1612-1615https://doi.org/10.1001/archneur.63.11.1612
        • Chai J
        • Herrmann DN
        • Stanton M
        • Barbano RL
        • Logigian EL
        Painful small-fiber neuropathy in Sjogren syndrome.
        Neurology. 2005; 65: 925-927https://doi.org/10.1212/01.wnl.0000176034.38198.f9
        • Sène D
        Small fiber neuropathy: Diagnosis, causes, and treatment.
        Joint Bone Spine. 2018; 85: 553-559https://doi.org/10.1016/j.jbspin.2017.11.002
        • Mori K
        • Iijima M
        • Koike H
        • Hattori N
        • Tanaka F
        • Watanabe H
        • et al.
        The wide spectrum of clinical manifestations in Sjögren's syndrome-associated neuropathy.
        Brain. 2005; 128: 2518-2534https://doi.org/10.1093/brain/awh605
        • Hoeijmakers JG
        • Faber CG
        • Lauria G
        • Merkies IS
        • Waxman SG
        Small-fibre neuropathies–advances in diagnosis, pathophysiology and management.
        Nat Rev Neurol. 2012; 8: 369-379https://doi.org/10.1038/nrneurol.2012.97
        • Oaklander AL
        • Nolano M.
        Scientific Advances in and Clinical Approaches to Small-Fiber Polyneuropathy: A Review.
        JAMA Neurol. 2019; https://doi.org/10.1001/jamaneurol.2019.2917
        • Terkelsen AJ
        • Karlsson P
        • Lauria G
        • Freeman R
        • Finnerup NB
        • Jensen TS
        The diagnostic challenge of small fibre neuropathy: clinical presentations, evaluations, and causes.
        Lancet Neurol. 2017; 16: 934-944https://doi.org/10.1016/S1474-4422(17)30329-0
        • Lauria G
        • Hsieh ST
        • Johansson O
        • Kennedy WR
        • Leger JM
        • Mellgren SI
        • et al.
        European Federation of Neurological Societies/Peripheral Nerve Society Guideline on the use of skin biopsy in the diagnosis of small fiber neuropathy. Report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society.
        Eur J Neurol. 2010; 17 (e44-49): 903-912https://doi.org/10.1111/j.1468-1331.2010.03023.x
        • Devigili G
        • Tugnoli V
        • Penza P
        • Camozzi F
        • Lombardi R
        • Melli G
        • et al.
        The diagnostic criteria for small fibre neuropathy: from symptoms to neuropathology.
        Brain. 2008; 131: 1912-1925https://doi.org/10.1093/brain/awn093
        • Takahashi Y
        • Takata T
        • Hoshino M
        • Sakurai M
        • Kanazawa I
        Benefit of IVIG for long-standing ataxic sensory neuronopathy with Sjögren's syndrome.
        IV immunoglobulin. Neurology. 2003; 60: 503-505
        • Shiboski CH
        • Shiboski SC
        • Seror R
        • Criswell LA
        • Labetoulle M
        • Lietman TM
        • et al.
        American College of Rheumatology/European League Against Rheumatism classification criteria for primary Sjögren's syndrome: A consensus and data-driven methodology involving three international patient cohorts.
        Ann Rheum Dis. 2016; 76 (2017): 9-16https://doi.org/10.1136/annrheumdis-2016-210571
        • Rubin DI
        Needle electromyography: basic concepts and patterns of abnormalities.
        Neurol Clin. 2012; 30: 429-456https://doi.org/10.1016/j.ncl.2011.12.009
        • Sène D
        Small fiber neuropathy: Diagnosis, causes, and treatment.
        Joint Bone Spine. 2017; https://doi.org/10.1016/j.jbspin.2017.11.002
        • Ramos-Casals M
        • Brito-Zerón P
        • Seror R
        • Bootsma H
        • Bowman SJ
        • Dörner T
        • et al.
        Characterization of systemic disease in primary Sjögren's syndrome: EULAR-SS Task Force recommendations for articular, cutaneous, pulmonary and renal involvements.
        Rheumatology (Oxford). 2015; 54: 2230-2238https://doi.org/10.1093/rheumatology/kev200
        • Fauchais A-L
        • Richard L
        • Gondran G
        • Ghorab K
        • Palat S
        • Bezanahary H
        • et al.
        [Small fibre neuropathy in primary Sjögren syndrome].
        Rev Med Interne. 2011; 32: 142-148https://doi.org/10.1016/j.revmed.2010.08.022
        • Birnbaum J
        • Lalji A
        • Saed A
        • Baer AN
        Biopsy-Proven Small-Fiber Neuropathy in Primary Sjögren's Syndrome:Neuropathic Pain Characteristics, Autoantibody Findings, and Histopathological Features.
        Arthritis Care Res (Hoboken). 2018; https://doi.org/10.1002/acr.23762
        • Sène D
        • Cacoub P
        • Authier F-J
        • Haroche J
        • Créange A
        • Saadoun D
        • et al.
        Sjögren Syndrome-Associated Small Fiber Neuropathy: Characterization From a Prospective Series of 40 Cases.
        Medicine (Baltimore). 2013; 92: e10-e18https://doi.org/10.1097/MD.0000000000000005
        • Ng Wing Tin S
        • Zouari HG
        • Wahab A
        • Sène D
        • Lefaucheur J-P
        Characterization of Neuropathic Pain in Primary Sjögren's Syndrome with Respect to Neurophysiological Evidence of Small-Fiber Neuropathy.
        Pain Med. 2018; https://doi.org/10.1093/pm/pny183
        • Lefaucheur J-P
        • Wahab A
        • Planté-Bordeneuve V
        • Sène D
        • Ménard-Lefaucheur I
        • Rouie D
        • et al.
        Diagnosis of small fiber neuropathy: A comparative study of five neurophysiological tests.
        Neurophysiol Clin. 2015; 45: 445-455https://doi.org/10.1016/j.neucli.2015.09.012
        • Kemp HI
        • Petropoulos IN
        • Rice ASC
        • Vollert J
        • Maier C
        • Sturm D
        • et al.
        Use of Corneal Confocal Microscopy to Evaluate Small Nerve Fibers in Patients With Human Immunodeficiency Virus.
        JAMA Ophthalmol. 2017; 135: 795-800https://doi.org/10.1001/jamaophthalmol.2017.1703