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Neurological Complications of Sjögren’s Syndrome: Diagnosis and Management

  • Other CTD: Inflammatory Myopathies and Sjogren's (P Basharat, Section Editor)
  • Published:
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Opinion statement

Purpose of review Neurologic disease is a common extraglandular manifestation of Sjögren’s syndrome (SS), the study of which has been hampered both by the lack of uniform definitions for specific neurologic complications and by the imprecision of the tools used to diagnose SS. There is a great need to develop consensus criteria for classifying these varied neurologic manifestations, as has been done in systemic lupus erythematosus (SLE) “Arthritis and rheumatism 42:599–608, 1999”. SS patients with certain forms of neurologic involvement, such as small fiber neuropathy and sensory ataxic ganglionopathy, frequently lack anti-SSA and anti-SSB antibodies and other serologic abnormalities. In these patients, neurologic disease is often their presenting manifestation, triggering a search for underlying SS. Given the frequent seronegativity of such patients, their diagnosis of SS rests heavily on the interpretation of a labial gland biopsy. However, these biopsies are prone to misinterpretation “Vivino et al. J Rheumatol 29:938–44, 2002”, and “positive” ones are found in up to 15% of healthy volunteers “Radfar et al. Arthrit Rheumatu 47:520–4, 2002”. Better diagnostic tools are needed to determine if the frequent seronegative status of these SS patients may be related to a unique disease pathogenesis.

Recent findings Recent advances in diagnostic techniques have served to define a likely pathogenetic basis for certain neurologic manifestations of SS. The advent of punch skin biopsies to analyze intraepidermal nerve fiber density and morphology has helped define pure sensory small fiber neuropathy as common in SS and the basis for both length- and non-length-dependent patterns of neuropathic pain. New protocols for magnetic resonance imaging (MRI) have enabled the recognition of dorsal root ganglionitis, a finding originally detected in pathologic studies. The advent of the anti-aquaporin-4 (AQP4) antibody test in 2004 has led to the appreciation that demyelinating disease in SS is often related to the presence of neuromyelitis optica spectrum disorder. The anti-AQP4 antibody is considered to be directly pathogenic in the brain, targeting the primary water channel proteins in the brain, expressed prominently on astrocytic foot processes.

Summary There are no clinical trials evaluating the efficacy of systemic immunosuppressive therapy for peripheral or central nervous system involvement. With the recent increase in clinical trials of biologic agents for SS, which utilize systemic disease manifestations as standardized outcome measures, there is an urgency to develop appropriate definitions of neurologic complications of SS and clear parameters for clinical improvement.

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Authors and Affiliations

  1. School of Medicine and Public Health, University of Wisconsin, 1685 Highland Avenue, Madison, WI, 53705-2281, USA

    Sara S. McCoy & Alan N. Baer

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  1. Sara S. McCoy
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Correspondence to Sara S. McCoy.

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Sara McCoy, MD declare that they have no conflict of interest.

Alan Baer, MD declare that they have no conflict of interest.

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This article does not contain any studies with human or animal subjects performed by any of the authors.

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This article is part of the Topical Collection on Other CTD: Inflammatory Myopathies and Sjogren's

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McCoy, S.S., Baer, A.N. Neurological Complications of Sjögren’s Syndrome: Diagnosis and Management. Curr Treat Options in Rheum 3, 275–288 (2017). https://doi.org/10.1007/s40674-017-0076-9

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