Quarterly Medical ReviewCentral and peripheral neurological complications of primary Sjögren's syndrome
Section snippets
Epidemiology and subtypes
Primary SS is associated with several different types of neuropathies in approximately 2 to 20% of patients [7], [8], [10], [11], [12], [14], [15], [16].
Indeed, the clinical spectrum of peripheral neuropathies encountered in pSS is wide, and sensory neuropathies are the most commonly reported [7], [8], [10], [11], [12], [13], [14], [15], [16]. These pSS-related sensory neuropathies are represented by distal, length-dependent axonal sensory neuropathy and sensory ataxic neuronopathy involving
Small fiber neuropathy and primary Sjögren's syndrome
The prevalence of small fiber neuropathy in pSS is actually unknown; however, the systematic DN4 screening of neuropathic pain in pSS is positive at least in 10 to 15% of the cases [8], [40], with abnormal electromyographic studies in 45% of the cases only. Hence, a small fiber involvement could be suspected in 5 to 8% of pSS patients [8], [16], [41]. pSS patients with SFN exhibited chronic neuropathic pain (i.e. burning sensation, prickling, dysesthesia, allodynia). Neuropathic pain involved
Epidemiology and subtypes
CNS involvement in pSS was initially controversial and seems to occur in 1 to 60% of patients depending on heterogeneous inclusion criteria [1], [3], [4], [5], [6], [29]. However, more recent reports emphasize the involvement of the CNS, often mimicking the clinical symptoms of primary progressive or relapsing-remitting multiple sclerosis although the association of pSS and multiple sclerosis is possible. Hence, pSS appears to be a great imitator of multiple sclerosis, involving the CNS more
Conclusion
Neurological complications affect 20% of pSS patients and may be inaugural. Both peripheral and central involvement could be related to a vasculitis process. Hence, systematic research of complement activation and cryoglobulinemia must be systematic in pSS patients suspect from neurologic manifestations. However, no characteristic immunogical profile has been associated with pSS neurologic manifestations.
The DN4 questionnaire may improve early detection of pSS-related neuropathies. Axonal
Disclosure of interest
the authors declare that they have no conflicts of interest concerning this article.
References (72)
- et al.
Comparison of pain syndromes associated with nervous or somatic lesions and development of a new neuropathic pain diagnostic questionnaire (dn4)
Pain
(2005) - et al.
Immunological profile in primary Sjögren's syndrome clinical significance, prognosis and long-term evolution to other autoimmune disease
Autoimmun Rev
(2010) Neurologic disease in Sjögren's syndrome: mononuclear inflammatory vasculopathy affecting central/peripheral nervous system and muscle. A clinical review and up- date of immunopathogenesis
Rheum Dis Clin North Am
(1993)- et al.
Autoimmune autonomic ganglionopathy with Sjögren's syndrome: significance of ganglionic acetylcholine receptor antibody and therapeutic approach
Auton Neurosci
(2009) - et al.
Intravenous immunoglobulin treatment for painful sensory neuropathy associated with Sjögren's syndrome
J Neurol Sci
(2009) - et al.
Treatment with rituximab in patients with mixed cryoglobulinemia syndrome: results of multicenter cohort study and review of the literature
Autoimmun Rev
(2011) - et al.
Small fiber neuropathy in primary Sjögren's syndrome–a monocentric experience
Rev Med Int 2010
(2011) - et al.
Neurological manifestations revealing primitive Gougerot-Sjögren syndrome: 9 cases
Joint Bone Spine
(2009) - et al.
Central nervous system disease in primary Sjögren's syndrome: the role of magnetic resonance imaging
Semin Arthritis Rheum
(2004) - et al.
An autopsied case of Sjögren's syndrome with massive necrotic and demyelinating lesions of the cerebellar white matter
J Neurol Sci
(2004)
Primary Sjögren's syndrome presenting as a generalized chorea
Parkinsonism Relat Disord
Peripheral neuropathy in primary Sjögren's syndrome
Neurology
Primary Sjögren's syndrome in north east England-a longitudinal study
Br J Rheumatol
Anti-neuronal antibodies in patients with neurologic complications of primary Sjögren's syndrome
Neurology
Neurological and neuropsychiatric dysfunction in primary Sjögren's syndrome
Acta Neurol Scand
Neurological involvement in primary Sjögren's syndrome: clinical and instrumental evaluation in a cohort of Italian patients
Clin Rheumatol
Primary Sjögren's syndrome in Spain: clinical and immunologic expression in 1010 patients
Medicine (Baltimore)
Clinical manifestations of neurological involvement in primary Sjögren's syndrome
Clin Rheumatol
Peripheral neuropathies in Sjögren's syndrome: a new reappraisal
J Neurol Neurosurg Psychiatry
Peripheral neuropathies associated with primary Sjögren's syndrome: Immunologic profiles of nonataxic sensory neuropathy and sensorimotor neuropathy
Medicine (Baltimore)
Neurologic complications of primary Sjögren's syndrome
Medicine (Baltimore)
Neurologic manifestations in primary Sjögren's syndrome: a study of 82 patients
Medicine (Baltimore)
Involvement of the peripheral nervous system in primary Sjögren's syndrome
Ann Rheum Dis
The wide spectrum of clinical manifestations in Sjögren's syndrome-associated neuropathy
Brain
The spectrum of neurological involvement in Sjögren's syndrome
Br J Rheumatol
Pure sensory neuropathy in primary Sjögren's syndrome. Long-term prospective follow-up and review of the literature
J Rheumatol
Peripheral neuropathy in an outpatient cohort of patients with Sjögren's syndrome
Muscle Nerve
Ataxic sensory neuropathy and dorsal root ganglionitis associated with Sjögren's syndrome
Ann Neurol
Vasculitic neuropathy in rheumatoid disease and Sjögren's syndrome
Neurology
Peripheral neuropathy associated with primary Sjögren's syndrome
J Neurol Neurosurg Psychiatry
Peripheral neuropathy in primary Sjögren's syndrome: a population-based study
Arch Neurol
Peripheral neuropathy associated with sicca complex
Neurology
Sensory neuronopathy and Sjögren's syndrome: clinical and immunologic study of two patients
Neurology
Peripheral neuropathy associated with essential mixed cryoglobulinaemia: a role for hepatitis c virus infection?
J Neurol Neurosurg Psychiatry
Peripheral neuropathy in mixed cryoglobulinemia: clinical and electrophysiologic investigations
J Rheumatol
Peripheral neuropathy in essential mixed cryoglobulinaemia
J Neurol Neurosurg Psychiatry
Cited by (58)
Saliva biomarkers in oral disease
2023, Clinica Chimica ActaPeripheral neuropathies during systemic diseases: Part I (connective tissue diseases and granulomatosis)
2023, Revue de Medecine InternePrimary Sjögren's syndrome: Autoantibodies and their relationship to clinical manifestations and histology of minor salivary glands
2023, Revista Colombiana de ReumatologiaOcular ischemic syndrome and facial palsy associated with cryoglobulinemia and primary Sjögren's syndrome
2020, Journal Francais d'OphtalmologieChronic meningitis as an initial presentation of Sjögren syndrome
2020, Revista Colombiana de Reumatologia