Immunological profiles determine neurological involvement in Sjögren's syndrome

Jamilloux, Yvan; Magy, Laurent; Hurtevent, Jean-François; Gondran, G.; Seze, J. de; Launay, David; Ly, Kim-Heang; Lambert, Marc; Hachulla, É.; Hatron, Pierre‐Yves; Vidal, É.; Fauchais, Anne-Laure

doi:10.1016/j.ejim.2013.10.005

Cited by 55 publications

(58 citation statements)

References 13 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…However, when of vasculitic origin, multiple mononeuropathies have the best response to immunotherapies, such as steroids and cyclophosphamide [82,99], followed by maintenance treatment preferably with mycophenolate mofetil. Axonal sensorimotor neuropathies have also been associated with extraglandular pSS [89,100] and although treatment is similar to that of multiple mononeuropathies (grade 3D) [101], the results are, in our experience, disappointing. In nonvasculitis-related neuropathies, the efficacy of immunosuppression is expected to be rather low [101], and most clinicians apply symptomatic therapy [20,91].…”

Section: Sjögren's Syndromementioning

confidence: 76%

“…Peripheral neuropathies are well documented in pSS, even though their prevalence varies greatly among studies (ranging from 2 % to 60 %) [83,89,90]. Some studies have found that symmetric axonal sensory or sensorimotor polyneuropathy is the most common form of neuropathy (34.0 %), followed by cranial neuropathies (19.5 %), multiple mononeuropathies (8.5 %), ganglionopathies (<5 %), and CIDP (1.3 %) [20,82,90].…”

Section: Sjögren's Syndromementioning

confidence: 99%

See 1 more Smart Citation

Immunotherapies for Neurological Manifestations in the Context of Systemic Autoimmunity

et al. 2016

View full text Add to dashboard Cite

Neurological involvement is relatively common in the majority of systemic autoimmune diseases and may lead to severe morbidity and mortality, if not promptly treated. Treatment options vary greatly, depending on the underlying systemic pathophysiology and the associated neurological symptoms. Selecting the appropriate therapeutic scheme is further complicated by the lack of definite therapeutic guidelines, the necessity to differentiate primary neurological syndromes from those related to the underlying systemic disease, and to sort out adverse neurological manifestations caused by immunosuppressants or the biological agents used to treat the primary disease. Immunotherapy is a sine qua non for treating most, if not all, neurological conditions presenting in the context of systemic autoimmunity. Specific agents include classical immune modulators such as corticosteroids, cyclophosphamide, intravenous immunoglobulin, and plasma exchange, as well as numerous biological therapies, for example antitumor necrosis factor agents and monoclonal antibodies that target various immune pathways such as B cells, cytokines, and co-stimulatory molecules. However, experience regarding the use of these agents in neurological complications of systemic diseases is mainly empirical or based on small uncontrolled studies and case series. The aim of this review is to present the state-of-the-art therapies applied in various neurological manifestations encountered in the context of systemic autoimmune diseases; evaluate all treatment options on the basis of existing guidelines; and compliment these data with our personal experience derived from a large number of patients.

show abstract

Section: Sjögren's Syndromementioning

confidence: 76%

Section: Sjögren's Syndromementioning

confidence: 99%

Immunotherapies for Neurological Manifestations in the Context of Systemic Autoimmunity

et al. 2016

View full text Add to dashboard Cite

show abstract

“…Studies reported that the pathogenesis of ALS may be related to the following factors: Cu/Zn superoxide dismutase (SOD1) gene mutation, glutamate toxicity theory, mitochondrial dysfunction, immune response, astrocyte dysfunction and so on. Primary Sjögren's syndrome (pSS) is a chronic autoimmune disorder characterized by sicca syndrome due to progressive lymphocytic infiltration of exocrine glands [3]. The mechanism of Sjogren's syndrome leading to central nervous system disease is still unknown, may be associated with T and B lymphocyte infiltration led to vasculitis or autoantibody-mediated related neuronal damage.…”

Section: Discussionmentioning

confidence: 99%

Treatment and Rehabilitation of Motor Neuron Syndrome Associated with Sjögren's Syndrome: A Case Report

Haixia¹,

Du²

2017

J Speech Pathol Ther

View full text Add to dashboard Cite

We report a patient with motor neuron syndrome accompanying Sjögren's syndrome. The patient showed progressive quadriparesis and slurred speech, but neither sensory involvement nor detrusor dysfunction was noted. EMG displayed upper and lower motor neuron damage. Although sicca symptom was not too obvious, parotid gland and submandibular gland ultrasound showed diffuse lesions. The Laboratory tests also displayed markedly elevated levels of Anti-SSA and anti-Ro 52 antibody. After a course of intravenous immunoglobulin therapy and 3 weeks of comprehensive rehabilitation, the patient showed significant improvements in terms of speech, muscle strength, trunk balance, posture control and ability of daily life. We suggested that the possibility of autoimmune-mediated motor neuron damage should be considered, when patients with motor neuron syndrome accompanying Sjögren's syndrome. And they may benefit from intravenous immunoglobulin therapy and appropriate rehabilitation.

show abstract

“…It is commonly acknowledged that about 20% of pSS patients will present neurological manifestations. In a recent publication, Jamilloux et al [3] presented a cohort of 420 patients followed between 1985 and 2012. All patients met the pSS diagnostic criteria according to ACR and EULAR definition (American-European classification criteria for the diagnosis of pSS).…”

Section: Frequency Of Neurological Manifestationsmentioning

confidence: 98%