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.
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.
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.
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.
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- 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
- 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
- Peripheral neuropathy in primary sjogren syndrome: a population-based study.Arch Neurol. 2006; 63: 1612-1615https://doi.org/10.1001/archneur.63.11.1612
- Painful small-fiber neuropathy in Sjogren syndrome.Neurology. 2005; 65: 925-927https://doi.org/10.1212/01.wnl.0000176034.38198.f9
- Small fiber neuropathy: Diagnosis, causes, and treatment.Joint Bone Spine. 2018; 85: 553-559https://doi.org/10.1016/j.jbspin.2017.11.002
- The wide spectrum of clinical manifestations in Sjögren's syndrome-associated neuropathy.Brain. 2005; 128: 2518-2534https://doi.org/10.1093/brain/awh605
- Small-fibre neuropathies–advances in diagnosis, pathophysiology and management.Nat Rev Neurol. 2012; 8: 369-379https://doi.org/10.1038/nrneurol.2012.97
- Scientific Advances in and Clinical Approaches to Small-Fiber Polyneuropathy: A Review.JAMA Neurol. 2019; https://doi.org/10.1001/jamaneurol.2019.2917
- 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
- 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
- The diagnostic criteria for small fibre neuropathy: from symptoms to neuropathology.Brain. 2008; 131: 1912-1925https://doi.org/10.1093/brain/awn093
- Benefit of IVIG for long-standing ataxic sensory neuronopathy with Sjögren's syndrome.IV immunoglobulin. Neurology. 2003; 60: 503-505
- 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
- Needle electromyography: basic concepts and patterns of abnormalities.Neurol Clin. 2012; 30: 429-456https://doi.org/10.1016/j.ncl.2011.12.009
- Small fiber neuropathy: Diagnosis, causes, and treatment.Joint Bone Spine. 2017; https://doi.org/10.1016/j.jbspin.2017.11.002
- 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
- [Small fibre neuropathy in primary Sjögren syndrome].Rev Med Interne. 2011; 32: 142-148https://doi.org/10.1016/j.revmed.2010.08.022
- 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
- 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
- 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
- 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
- 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
Published online: May 27, 2020
Accepted: May 3, 2020
Received: December 2, 2019
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