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Arthritis Care Res (Hoboken). 2018 Sep 17. doi: 10.1002/acr.23762. [Epub ahead of print]

Biopsy-Proven Small-Fiber Neuropathy in Primary Sjögren's Syndrome:Neuropathic Pain Characteristics, Autoantibody Findings, and Histopathological Features.

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Division of Rheumatology and Department of Neurology, The Johns Hopkins University School of Medicine.
Division of Rheumatology, The Johns Hopkins University School of Medicine.
Division of Rheumatology, Department of Medicine, The Johns Hopkins University School of Medicine.



Painful small-fiber neuropathies (SFN) in primary Sjögren's syndrome (SS) may present as pure or mixed with concurrent large-fiber involvement. SFN can be diagnosed by punch skin biopsies, which identifies decreased intra-epidermal nerve-fiber density (IENFD) of unmyelinated nerves.


We compared 23 consecutively evaluated SS patients with pure and mixed SFN versus 98 without. We distinguished between markers of dorsal root ganglia (DRG) degeneration (decreased IENFD proximal thigh versus distal leg) versus axonal degeneration (decreased IENFD distal leg versus proximal thigh).


There were no differences in pain intensity, quality, and treatment characteristics comparing 13 pure SFN versus 10 mixed SFN patients. Ten SFN patients (~45%) had neuropathic pain preceding sicca symptoms. Opioid analgesics were prescribed to ~45% SFN patients. When compared to 98 non-SFN patients, the 23 SFN patients had increased frequency of male sex (30% versus 9%, p<0.01), decreased frequency of anti-Ro52 (p=0.01) and anti-Ro60 antibodies (p=0.01), rheumatoid factor (p<0.01), and polyclonal gammopathy (p<0.01). Eleven patients had stocking-and-glove pain, and 12 patients had non-stocking-and-glove pain. Skin biopsy disclosed patterns of axonal (16 patients) and DRG injury (7 patients).


SS SFN had increased frequency of male sex, decreased frequency of multiple antibodies, were frequently treated with opioid analgesics, and could present with non-stocking-and-glove pain. Distinguishing between DRG versus axonal injury is significant, especially given that mechanisms targeting the DRG may result in irreversible neuronal cell death. Altogether, these findings highlight clinical, autoantibody, and pathological features that can help to define mechanisms and treatment strategies. This article is protected by copyright. All rights reserved.


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