Central nervous system involvement in Sjogren's syndrome: unusual, but not unremarkable--clinical, serological characteristics and outcomes in a large cohort of Italian patients

Massara, Alfonso; Bonazza, Sara; Castellino, Gabriella; Caniatti, Luisa; Trotta, Francesco; Borrelli, Massimo; Feggi, Luciano; Govoni, Marcello

doi:10.1093/rheumatology/keq111

Cited by 124 publications

(121 citation statements)

References 54 publications

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“…CNS involvement is a rare manifestation in primary Sjögren's syndrome (pSS) [80], and, in the majority of cases, is due to vasculopathy with endothelial dysfunction, leading to small infarcts or microaneurysms [3,81]. Transverse myelitis has also been described in patients with pSS [3,82,83], and in a small percentage it may be accompanied by brain MS-like lesions or by optic neuritis making, at times, the distinction from a primary demyelinating disease such as MS or NMO rather difficult [31,80].…”

Section: Sjögren's Syndromementioning

confidence: 99%

“…Transverse myelitis has also been described in patients with pSS [3,82,83], and in a small percentage it may be accompanied by brain MS-like lesions or by optic neuritis making, at times, the distinction from a primary demyelinating disease such as MS or NMO rather difficult [31,80]. The meninges can also be affected, alone or in the context of meningoencephalitis [84], while in some cases a subacute encephalopathy presenting with memory loss, cognitive dysfunction, visual disturbances, and reduced concentration and attention has been noted [80]. Symptoms such as seizures, headaches, psychiatric disturbances, and cognitive dysfunction have also been described but their true prevalence and association with the underlying syndrome is questionable [3,82].…”

Section: Sjögren's Syndromementioning

confidence: 99%

“…Treatment is similar to CNS lupus and mainly consists of immunosuppressive agents (high oral dose or IV pulse steroids, cyclophosphamide, AZA, mycophenolate mofetil) and possibly IVIg and rituximab [80,[85][86][87][88]. All these treatment options are, apart from our own experience, mostly documented in small case series.…”

Section: Sjögren's Syndromementioning

confidence: 99%

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Immunotherapies for Neurological Manifestations in the Context of Systemic Autoimmunity

et al. 2016

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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.

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Section: Sjögren's Syndromementioning

confidence: 99%

Section: Sjögren's Syndromementioning

confidence: 99%

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Immunotherapies for Neurological Manifestations in the Context of Systemic Autoimmunity

et al. 2016

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“…PNS involvement is characterized by sensory polyneuropathy and mononeuritis multiplex [198]. CNS manifestations occur in 6% to 25% of patients with Sjögren's syndrome, depending on the study and the definitions used, and these include encephalopathy, aseptic meningitis, optic neuritis, and MS-like disease [198,201,202]. Involvement of the CNS is more likely in patients with lung involvement [201].…”

Section: Sjögren's Syndromementioning

confidence: 99%

“…CNS manifestations occur in 6% to 25% of patients with Sjögren's syndrome, depending on the study and the definitions used, and these include encephalopathy, aseptic meningitis, optic neuritis, and MS-like disease [198,201,202]. Involvement of the CNS is more likely in patients with lung involvement [201]. Although most of the neurological manifestations of Sjögren's syndrome are not severe enough to warrant ICU admission, acute myelopathy can occur and potentially necessitate ventilatory support [203,204].…”

Section: Sjögren's Syndromementioning

confidence: 99%

Immune Mediated Diseases and Immune Modulation in the Neurocritical Care Unit

Geldern¹,

McPharlin

Becker

2012

Neurotherapeutics

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This chapter will review the spectrum of immunemediated diseases that affect the nervous system and may result in an admission to the neurological intensive care unit. Immunomodulatory strategies to treat acute exacerbations of neurological diseases caused by aberrant immune responses are discussed, but strategies for long-term immunosuppression are not presented. The recommendations for therapeutic intervention are based on a synthesis of the literature, and include recommendations by the Cochrane Collaborative, the American Academy of Neurology, and other key organizations. References from recent publications are provided for the disorders and therapies in which randomized clinical trials and large evidenced-based reviews do not exist. The chapter concludes with a brief review of the mechanisms of action, dosing, and side effects of commonly used immunosuppressive strategies in the neurocritical care unit.

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A 62‐Year‐Old Man With Progressive Weakness, Multiple Neurologic Deficits, and Hypernatremia

Lim

Chia

Lim

et al. 2013

Arthritis Care & Research

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A 62-year-old Chinese man was admitted for progressive weakness, loss of weight and appetite, and multiple neurologic deficits. History of the presenting symptomThe patient had a 6-month history of unsteady gait and generalized weakness with constitutional symptoms of loss of weight and appetite. He consulted his general practitioner 2 months later. Laboratory investigations then showed normal complete blood count and liver and thyroid function tests. Magnetic resonance imaging (MRI) of the brain with contrast fluid-attenuated inversion recovery (FLAIR) sequences and diffusion-weighted imaging showed multiple nonspecific foci of an abnormal signal seen in the white matter of both cerebral hemispheres. VDRL serology was nonreactive. The only abnormal investigation was an elevated serum sodium level at 148 mmoles/liter (reference interval 135-145). He was referred to a neurologist, who made a diagnosis of multiple system atrophy based on features of cerebellar dysfunction and dysautonomia, which included orthostatic hypotension and erectile dysfunction. His serum vitamin E level was 11.3 mg/liter (reference interval 5.5-18.0). He was commenced on highdose oral vitamin E therapy as an antioxidant for multiple system atrophy. He subsequently presented to the general medicine department and was admitted for progression of symptoms. Medical historyHe had been attending long-term followup visits with his general practitioner for hyperlipidemia and type 2 diabetes mellitus for Ͼ10 years. Glycosylated hemoglobin (HbA1c) 2 months prior to admission was 11.8%. His regular medications included lovastatin, glipizide, and vitamin E tablets. He had no recent exposure to anyone with tuberculosis or history of pulmonary tuberculosis. Medications and allergiesHe did not consume any over-the-counter medications or traditional herbal preparations. He was adherent to his medications, but not to dietary control for diabetes mellitus and hyperlipidemia. Social and family historyThe patient was born in Singapore and was the fourth of 10 children. There was a family history of hypertension and hyperlipidemia, but no history of neurologic disease or malignancy. His younger sister had died of an unknown complication of systemic lupus erythematosus (SLE) 2 decades before. He was single and living with his father. He was retired and used to work as a taxi driver. He did not smoke tobacco or consume any ethanol or illicit drugs. Review of systemsThe patient experienced a week's duration of change in bowel habit in the form of intermittent constipation. There was no per rectal bleeding, blood mixed with stools, chest pain, shortness of breath, abdominal pain, chronic cough, or any change in micturition. There was no history of joint pain, oral ulcers, alopecia, photosensitivity, Raynaud's phenomenon, or fever. Physical examinationExamination upon admission revealed a thin man who weighed 45 kg. He appeared lethargic, but was alert, coherent, nontoxic, and apyrexial. He was noted to have significant postural hypotension: his supine blood press...

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Central nervous system involvement in Sjogren's syndrome: unusual, but not unremarkable--clinical, serological characteristics and outcomes in a large cohort of Italian patients

Cited by 124 publications

References 54 publications

Immunotherapies for Neurological Manifestations in the Context of Systemic Autoimmunity

Immunotherapies for Neurological Manifestations in the Context of Systemic Autoimmunity

Immune Mediated Diseases and Immune Modulation in the Neurocritical Care Unit

A 62‐Year‐Old Man With Progressive Weakness, Multiple Neurologic Deficits, and Hypernatremia

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