Abstract
Primary Sjögren's syndrome (pSS)-associated neuropathy manifests a wide variety of
peripheral neuropathies that may show overlap among the neuropathic forms. In this
report, we describe histopathological findings of two autopsy cases with pSS-associated
neuropathy; one of them manifested the painful form and another showed ataxic form.
The population of dorsal root ganglion (DRG) neurons and the density of myelinated
fibers in the dorsal spinal root were variably reduced among spinal segments in both
forms. In the painful form, there was a prominent reduction of small neurons, while
in the ataxic form, large neurons were predominately lost. In accordance with the
degree of the DRG cell loss, the modality of nerve fiber loss in the dorsal spinal
roots and sural nerve correlated well with the corresponding DRG neuron loss. Prominent
CD8+ T lymphocyte infiltration was present in the DRG, sympathetic ganglion, epineurial
and perineurial space throughout the peripheral nerve trunks, and visceral organs,
including the submandibular gland of both forms.
Although the size of affected DRG neurons is different, these two forms share a similar
causal mechanism, namely, cytotoxic autoimmunity to ganglion neurons, which may be
one of a continuum of etiological factors. This hypothesis may have an impact on therapeutic
approach.
Keywords
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Article info
Publication history
Published online: June 04, 2012
Accepted:
May 9,
2012
Received in revised form:
May 8,
2012
Received:
April 2,
2012
Identification
Copyright
© 2012 Elsevier B.V. Published by Elsevier Inc. All rights reserved.
