REVIEW PAPER
Neurological manifestations of primary Sjögren’s syndrome
Submission date: 2018-03-05
Final revision date: 2018-03-29
Acceptance date: 2018-04-03
Online publication date: 2018-05-09
Publication date: 2018-04-30
Reumatologia 2018;56(2):99-105
KEYWORDS
TOPICS
ABSTRACT
Primary Sjögren’s syndrome (pSS) is an autoimmune connective tissue disease affecting the exocrine glands, leading to damage of their structure and impairment of their function. In the course of pSS the internal organs may be involved and the symptoms may concern any system. Neurological disorders are one of the most common extraglandular manifestations of pSS. Available literature data estimate the prevalence of neurological symptoms as about 8.5–70% of patients diagnosed with pSS. The most common neurological complication of pSS is peripheral neuropathy, and in particular sensory polyneuropathy. Central nervous system involvement is much less common. There are also reports of various symptoms connected with damage to cranial nerves and the autonomic nervous system. A careful neurological evaluation, combined with neurophysiological tests, is recommended in patients with pSS. This review summarizes the neurological manifestations of pSS, their possible pathogenic mechanisms, diagnostic evaluation and potential treatment.
REFERENCES (60)
1.
Alexander E, Provost T, Stevens M, et al. Neurologic complications of primary Sjögren’s syndrome. Medicine (Baltimore) 1982; 61: 247-257.
2.
Fauchais AL, Magy L, Vidal E. Central and peripheral neurological complications of primary Sjögren’s syndrome. Presse Med 2012; 41: 485-493.
3.
Griffin JW, Cornblath DR, Alexander E, et al. Ataxic sensory neuropathy and dorsal root ganglionitis associated with Sjögren’s syndrome. Ann Neurol 1990; 27: 304-315.
4.
Chai J, Logigian EL. Neurological manifestations of primary Sjögren syndrome. Curr Opin Neurol 2010; 23: 509-513.
5.
Moutsopoulos HN, Sarmas JH, Talal N. Is central nervous system involvement a systemic manifestation of primary Sjögren syndrome? Rheum Dis Clin North Am 1993; 19: 909-912.
6.
Andonopoulos AP, Lagos G, Drosos AA, et al. The spectrum of neurological involvement in Sjögren syndrome. Br J Rheumatol 1990; 29: 21-23.
7.
Delalande S, de Seze J, Fauchais AL, et al. Neurologic manifestations in primary Sjögren syndrome: a study of 82 patients. Medicine (Baltimore) 2004; 83: 280e91.
8.
Mori K, Iijima M, Koike H, et al. The wide spectrum of clinical manifestations in Sjögren’s syndrome-associated neuropathy. Brain 2005; 128: 2518-2534.
9.
Le Guern V, Belin C, Henegar C, et al. Cognitive function and 99mTc-ECD brain SPECT are significantly correlated in patients with primary Sjögren syndrome: a case-control study. Ann Rheum Dis 2010; 69: 132-137.
10.
Mataró M, Escudero D, Ariza M, et al. Magnetic resonance abnormalities associated with cognitive dysfunction in primary Sjögren syndrome. J Neurol 2003; 250: 1070-1076.
11.
Alexander EL, Malinow K, Lejewski JE, et al. Primary Sjögren’s syndrome with central nervous system disease mimicking multiple sclerosis. Ann Intern Med 1986; 104: 323-330.
12.
Thong BY, Venketasubramanian N. A case of Sjögren’s syndrome or multiple sclerosis? A diagnostic and therapeutic dilemma. Rheumatol Int 2002; 22: 256-258.
13.
Pillemer SR, Mendelsohn AB, Morgen KE. Central Nervous System involvement. In: Sjögren’s Syndrome. Ramos-Casals M (ed.). Springer-Verlag London Ltd, London 2012: 281-291.
14.
de Seze J, Devos D, Castelnovo G, et al. The prevalence of Sjögren syndrome in patients with primary progressive multiple sclerosis. Neurology 2001; 57: 1359-1363.
15.
de Seze J, Delalande S, Fauchais AL, et al. Myelopathies secondary to Sjögren’s syndrome: treatment with monthly intravenous cyclophosphamide associated with corticosteroids. J Rheumatol 2006; 33: 709-711.
16.
Zahlane S, Louhab N, El Mellakh M, et al. Anterior horn syndrome: A rare manifestation of primary Sjögren’s syndrome. Joint Bone Spine 2016; 83: 448-450.
17.
Hagiwara K, Murai H, Ochi H, et al. Upper motor neuron syndrome associated with subclinical Sjögren’s syndrome. Intern Med 2008; 47: 1047-1051.
18.
Gøransson LG, Herigstad A, Tjensvoll AB, et al. Peripheral neuropathy in primary Sjögren syndrome: a population-based study. Arch Neurol 2006; 63: 1612-1615.
19.
Vrethem M, Lindvall B, Holmgren H, et al. Neuropathy and myopathy in primary Sjögren’s syndrome: neurophysiological, immunological and muscle biopsy results. Acta Neurol Scand 1990; 82: 126-131.
20.
Gemignani F, Marbini A, Pavesi G, et al. Peripheral neuropathy associated with primary Sjögren’s syndrome. J Neurol Neurosurg Psychiatry 1994; 57: 983-986.
21.
Barendregt PJ, van den Bent MJ, van Raaij-van den Aarssen VJ, et al. Involvement of the peripheral nervous system in primary Sjögren’s syndrome. Ann Rheum Dis 2001; 60: 876-881.
22.
Lopate G, Pestronk A, Al-Lozi M, et al. Peripheral neuropathy in an outpatient cohort of patients with Sjögren’s syndrome. Muscle Nerve 2006; 33: 672-676.
23.
Skopouli FN, Dafni U, Ioannidis JP, et al. Clinical evolution, and morbidity and mortality of primary Sjögren’s syndrome. Semin Arthritis Rheum 2000; 29: 296-304.
24.
Ioannidis JP, Vassiliou VA, Moutsopoulos HM. Long-term risk of mortality and lymphoproliferative disease and predictive classification of primary Sjögren’s syndrome. Arthritis Rheum 2002; 46: 741-747.
25.
Liao CY, Wang CC, Chen IH, et al. Hypokalemic paralysis as a presenting manifestation of primary Sjögren’s syndrome accompanied by vitamin D deficiency. Intern Med 2013; 52: 2351-2353.
26.
Garcia-Carrasco M, Ramos-Casals M, Rosas J. Primary Sjögren syndrome: Clinical and immunologic disease patterns in a cohort of 400 patients. Medicine (Baltimore) 2002; 81: 270-280.
27.
Lafitte C, Amoura Z, Cacoub P, et al. Neurological complications of primary Sjögren’s syndrome. J Neurol 2001; 248: 577-584.
28.
Sène D, Jallouli M, Lefaucheur J-P, et al. Peripheral neuropathies associated with primary Sjögren syndrome: immunologic profiles of nonataxic sensory neuropathy and sensorimotor neuropathy. Medicine (Baltimore) 2011; 90: 133-138.
29.
Tanaka K, Nakayasu H, Suto Y, et al. Acute Motor-dominant Polyneuropathy as Guillain-Barré Syndrome and Multiple Mononeuropathies in a Patient with Sjögren’s Syndrome. Intern Med 2016; 55: 2717-2722.
30.
Tobón GJ, Pers JO, Devauchelle-Pensec V, et al. Neurological Disorders in Primary Sjögren’s Syndrome. Autoimmune Dis 2012; 2012: 645967.
31.
Camdessanché JP, Jousserand G, Ferraud K, et al. The pattern and diagnostic criteria of sensory neuronopathy: a case-control study. Brain 2009; 132: 1723-1733.
32.
Jamilloux Y, Magy L, Hurtevent JF, et al. Immunological profiles determine neurological involvement in Sjögren’s syndrome. Eur J Intern Med 2014; 25: 177-181.
33.
Mochizuki H, Kamakura K, Masaki T, et al. Motor dominant neuropathy in Sjögren’s syndrome: report of two cases. Intern Med 2002; 41: 142-146.
34.
Dworkin RH, O’Connor AB, Backonja M, et al. Pharmacologic management of neuropathic pain: evidence-based recommendations. Pain 2007; 132: 237-251.
35.
Hughes RA, Donofrio P, Bril V, et al. Intravenous immune globulin (10% caprylate-chromatography purified) for the treatment of chronic inflammatory demyelinating polyradiculoneuropathy (ICE study): a randomised placebo-controlled trial. Lancet Neurol 2008; 7: 136-144.
36.
Terrier B, Lacroix C, Guillevin L, et al. Diagnostic and prognostic relevance of neuromuscular biopsy in primary Sjögren’s syndrome-related neuropathy. Arthritis Rheum 2007; 57: 1520-1529.
37.
Ramos-Casals M, Solans R, Rosas J, et al. Primary Sjögren’s syndrome in Spain: clinical and immunologic expression in 1010 patients. Medicine (Baltimore) 2008; 87: 210-219.
38.
Grant IA, Hunder GG, Homburger HA, et al. Peripheral neuropathy associated with sicca complex. Neurology 1997; 48: 855-862.
39.
England JD, Gronseth GS, Franklin G, et al. Evaluation of distal symmetric polyneuropathy: the role of autonomic testing, nerve biopsy, and skin biopsy (an evidence-based review). Muscle Nerve 2009; 39: 106-115.
41.
Pereira PR, Viala K, Maisonobe T, et al. Sjögren Sensory Neuronopathy (Sjögren Ganglionopathy): Long-Term Outcome and Treatment Response in a Series of 13 Cases. Medicine (Baltimore) 2016; 95: e3632.
42.
Souayah N, Chong PS, Cros D. Acute sensory neuronopathy as the presenting symptom of Sjögren’s syndrome. J Clin Neurosci 2006; 13: 862-865.
43.
Takahashi Y, Takata T, Hoshino M, et al. Benefit of IVIG for long-standing ataxic sensory neuronopathy with Sjögren’s syndrome. IV immunoglobulin. Neurology 2003; 60: 503-505.
44.
Chen WH, Yeh JH, Chiu HC. Plasmapheresis in the treatment of ataxic sensory neuropathy associated with Sjögren’s syndrome. Eur Neurol 2001; 45: 270-274.
45.
Yamada S, Mori K, Matsuo K, et al. Interferon alfa treatment for Sjögren’s syndrome associated neuropathy. J Neurol Neurosurg Psychiatry 2005; 76: 576-578.
47.
Mori K, Iijima M, Sugiura M, et al. Sjögren’s syndrome associated painful sensory neuropathy without sensory ataxia. J Neurol Neurosurg Psychiatry 2003; 74: 1320-1322.
48.
Amoura Z, Salachas F, Cacoub B, et al. Pseudosclerose laterale amyotrophiqueau tours du syndrome de Gougerot-Sjögren primitif. Rev Med Interne 1999; 20: 83s.
49.
Attout L, Rahmeh F, Ziegler L. Syndrome de Gougerot-Sjögren simulant unesclerose laterale amyotrophique. Rev Med Interne 2000; 21: 708-710.
50.
Katz JS, Houroupian D, Ross MA. Multisystem neuronal involvement and sicca complex: broadening the spectrum of complications. Muscle Nerve 1999; 22: 404-407.
51.
Rafai MA, Boulaajaj FZ, Moutawakil F, et al. Neurological manifestations revealing primitive Gougerot-Sjögren syndrome: 9 cases. Joint Bone Spine 2009; 76: 139-145.
52.
Lindvall B, Bengtsson A, Ernerudh J, et al. Subclinical myositis is common in primary Sjögren’s syndrome and is not related to muscle pain. J Rheumatol 2002; 29: 717-725.
53.
Kanellopoulos P, Baltoyiannis C, Tzioufas AG. Primary Sjögren’s syndrome associated with inclusion body myositis. Rheumatology (Oxford) 2002; 41: 440-444.
54.
Misterska-Skóra M, Sebastian A, Dzięgiel P, et al. Inclusion body myositis associated with Sjögren’s syndrome. Rheumatol Int 2013; 33: 3083-3086.
55.
Hung YM, Huang NC, Wann SR, et al. Recurrent Hypokalemic Periodic Paralysis UnmasksSjögren Syndrome without Sicca Symptoms. J Coll Physicians Surg Pak 2015; 25 Suppl 1: S36-S38.
56.
Andonopoulos AP, Christodoulou J, Ballas C, et al. Autonomic cardiovascular neuropathy in Sjögren’s syndrome. A controlled study. J Rheumatol 1998; 25: 2385-2388.
57.
Waterman SA, Gordon TP, Rischmueller M. Inhibitory effects of muscarinic receptor autoantibodies on parasympathetic neurotransmission in Sjögren’s syndrome. Arthritis Rheum 2000; 43: 1647-1654.
58.
Dawson L, Tobin A, Smith P, et al. Antimuscarinic antibodies in Sjögren’s syndrome: where are we, and where are we going? Arthritis Rheum 2005; 52: 2984-2995.
59.
Fox RI, Stern M. Sjögren’s syndrome: mechanisms of pathogenesis involve interaction of immune and neurosecretory systems. Scand J Rheumatol 2002; 116: 3-13.
60.
Dawson LJ, Caulfield VL, Stanbury JB, et al. Hydroxychloroquine therapy in patients with primary Sjögren’s syndrome may improve salivary gland hypofunction by inhibition of glandular cholinesterase. Rheumatology 2005; 44: 449-455.
Copyright: © Narodowy Instytut Geriatrii, Reumatologii i Rehabilitacji w Warszawie. This is an Open Access journal, all articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International (CC BY-NC-SA 4.0) License (https://creativecommons.org/licenses/by-nc-sa/4.0/), allowing third parties to copy and redistribute the material in any medium or format and to remix, transform, and build upon the material, provided the original work is properly cited and states its license.
Share
RELATED ARTICLE
| eISSN: | 2084-9834 |
| ISSN: | 0034-6233 |
We process personal data collected when visiting the website. The function of obtaining information about users and their behavior is carried out by voluntarily entered information in forms and saving cookies in end devices. Data, including cookies, are used to provide services, improve the user experience and to analyze the traffic in accordance with the Privacy policy. Data are also collected and processed by Google Analytics tool (more).
You can change cookies settings in your browser. Restricted use of cookies in the browser configuration may affect some functionalities of the website.
You can change cookies settings in your browser. Restricted use of cookies in the browser configuration may affect some functionalities of the website.
