The Neurological Manifestations of Sjögren’s Syndrome: Diagnosis and Treatment

Fox, Robert I.; Birnbaum, Julius

doi:10.1007/978-1-60327-957-4_22

Cited by 3 publications

(7 citation statements)

References 158 publications

(124 reference statements)

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“…Sjögren’s syndrome is a chronic inflammatory disease characterized by lymphocytic infiltration and impaired function of the lacrimal and salivary glands. The main manifestations are keratoconjunctivitis sicca and xerostomia, although lymph node and salivary gland enlargement, non-Hodgkin’s lymphoma, myalgia, fatigue and cognitive dysfunction may also occur [ 5 ]. New diagnostic criteria proposed in 2012 by the American College of Rheumatology [ 6 ] include at least two of the following: (1) positive serum anti-SSA and/or anti-SSB antibodies or positive rheumatoid factor and ANA titers 1:320 or higher, (2) a Sjögren’s International Collaborative Clinical Alliance ocular staining score of 3 or higher and (3) lymphocytic sialadenitis with one or more foci of 50 lymphocytes per 4mm 2 .…”

Section: Discussionmentioning

confidence: 99%

Sjögren’s syndrome and acquired splenic atrophy with septic shock: a case report

et al. 2014

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IntroductionThe most frequent causes of adult-onset recurrent infections are human immunodeficiency virus infection, malignancy, and autoimmune diseases, while acquired non-surgical hyposplenism is rare. Although acquired asplenia/hyposplenism have been described in association with celiac disease and, less frequently, with autoimmune diseases such as Sjögren’s syndrome, the manifestations in this context are usually only detectable in the laboratory setting, with Howell-Jolly bodies or thrombocytosis. To the best of our knowledge, no previous case of pneumococcal septic shock in a patient with acquired hyposplenism and co-morbid Sjögren’s syndrome has been reported.Case presentationWe report a case of a 45-year-old Caucasian woman who developed pneumococcal pneumonia at age 42 years, pneumococcal meningitis at age 44 years and septic shock with Streptococcus agalactiae bacteremia at age 45 years and was subsequently diagnosed with radiological splenic atrophy and functional asplenia, as well as primary Sjögren’s syndrome. After appropriate immunizations, the patient has been free from clinically important infections.ConclusionHyposplenism should be suspected in patients with adult-onset infections caused by encapsulated bacteria, especially if autoantibodies are present. Early diagnosis can help prevent potentially life-threatening infections. Possible associations between splenic atrophy and Sjögren’s syndrome are discussed.

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Section: Discussionmentioning

confidence: 99%

Sjögren’s syndrome and acquired splenic atrophy with septic shock: a case report

et al. 2014

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“…The respective frequencies of these individual syndromes are rare: including the sensory neuronopathies (prevalence of ∼1:1000 in SS patients), 29 , 42 , 43 cerebellar degeneration (prevalence of <1:1000 in SS patients), 43 and FTD (prevalence of ∼8:10,000). 44 Therefore, the collective likelihood that these disorders jointly occurred by random chance, and were not mechanistically interrelated, has a probability of ∼1-per-billion.…”

Section: Case Presentationsmentioning

confidence: 99%

Movement and Other Neurodegenerative Syndromes in Patients with Systemic Rheumatic Diseases

et al. 2015

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Patients with rheumatic diseases can present with movement and other neurodegenerative disorders. It may be underappreciated that movement and other neurodegenerative disorders can encompass a wide variety of disease entities. Such disorders are strikingly heterogeneous and lead to a wider spectrum of clinical injury than seen in Parkinson's disease. Therefore, we sought to stringently phenotype movement and other neurodegenerative disorders presenting in a case series of rheumatic disease patients. We integrated our findings with a review of the literature to understand mechanisms which may account for such a ubiquitous pattern of clinical injury.Seven rheumatic disease patients (5 Sjögren's syndrome patients, 2 undifferentiated connective tissue disease patients) were referred and could be misdiagnosed as having Parkinson's disease. However, all of these patients were ultimately diagnosed as having other movement or neurodegenerative disorders. Findings inconsistent with and more expansive than Parkinson's disease included cerebellar degeneration, dystonia with an alien-limb phenomenon, and nonfluent aphasias.A notable finding was that individual patients could be affected by cooccurring movement and other neurodegenerative disorders, each of which could be exceptionally rare (ie, prevalence of ∼1:1000), and therefore with the collective probability that such disorders were merely coincidental and causally unrelated being as low as ∼1-per-billion. Whereas our review of the literature revealed that ubiquitous patterns of clinical injury were frequently associated with magnetic resonance imaging (MRI) findings suggestive of a widespread vasculopathy, our patients did not have such neuroimaging findings. Instead, our patients could have syndromes which phenotypically resembled paraneoplastic and other inflammatory disorders which are known to be associated with antineuronal antibodies. We similarly identified immune-mediated and inflammatory markers of injury in a psoriatic arthritis patient who developed an amyotrophic lateral sclerosis (ALS)-plus syndrome after tumor necrosis factor (TNF)-inhibitor therapy.We have described a diverse spectrum of movement and other neurodegenerative disorders in our rheumatic disease patients. The widespread pattern of clinical injury, the propensity of our patients to present with co-occurring movement disorders, and the lack of MRI neuroimaging findings suggestive of a vasculopathy collectively suggest unique patterns of immune-mediated injury.

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“…The primary target organs in pSS are exocrine glands (such as salivary, lacrimal, and sweat glands [1,2]), which are glands of the gastrointestinal and respiratory systems, which confirms the conception of generalized autoimmune epithelitis in this disease. In the absence of therapy, patients can develop systemic manifestations such as various types of lesions in the joints, blood vessels (cryoglobulinemic and hypergammaglobulinemic purpura), lungs, kidneys, and reticuloendothelial (regional and generalized lymphadenopathy, splenomegaly, and hepatomegaly), peripheral, and central nervous system, which can lead to a significant decrease in the quality and life expectancy of the patients [3,4]. According to studies, gastrointestinal involvement (dysphagia) can be observed in up to 80% of patients, and arthralgia is reported to be present in up to 75% of patients [5].…”

Section: Introductionmentioning

confidence: 99%

“…In 15% of patients, the disease debuts in childhood, and in 70% under the age of 50 years. pSS is more common in females, and the ratio to males ranges from 9:1 to 25:1 [3,4]. This phenomenon can be explained by several reasons.…”

Section: Introductionmentioning

confidence: 99%

“…In the absence of early diagnosis and treatment of the disease, due to inadequate activation and proliferation of T and B lymphocytes, ectopic lymphoid structures are formed in epithelial tissues. They are initially characterized by the synthesis of polyclonal and later oligoclonal and monoclonal immunoglobulins (Igs) with development in 5-11% of predominantly different variants of B-cell lymphomas [3,4]. Lymphoepithelial lesions (LELs) with ectopic lymphoid structures are found in 90-100% of patients with enlarged major salivary glands in their biopsy specimens, whereas their presence in MSGs is significantly lower [16].…”

Section: Introductionmentioning

confidence: 99%

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The Role of Regulatory T Cells in the Onset and Progression of Primary Sjögren’s Syndrome

Blinova

Vasilyev²,

Rodionova³

et al. 2023

Cells

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Regulatory T cells (Tregs) play a key role in maintaining immune balance and regulating the loss of self-tolerance mechanisms in various autoimmune diseases, including primary Sjögren’s syndrome (pSS). With the development of pSS primarily in the exocrine glands, lymphocytic infiltration occurs in the early stages, mainly due to activated CD4+ T cells. Subsequently, in the absence of rational therapy, patients develop ectopic lymphoid structures and lymphomas. While the suppression of autoactivated CD4+ T cells is involved in the pathological process, the main role belongs to Tregs, making them a target for research and possible regenerative therapy. However, the available information about their role in the onset and progression of this disease seems unsystematized and, in certain aspects, controversial. In our review, we aimed to organize the data on the role of Tregs in the pathogenesis of pSS, as well as to discuss possible strategies of cell therapy for this disease. This review provides information on the differentiation, activation, and suppressive functions of Tregs and the role of the FoxP3 protein in these processes. It also highlights data on various subpopulations of Tregs in pSS, their proportion in the peripheral blood and minor salivary glands of patients as well as their role in the development of ectopic lymphoid structures. Our data emphasize the need for further research on Tregs and highlight their potential use as a cell-based therapy.

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The Neurological Manifestations of Sjögren’s Syndrome: Diagnosis and Treatment

Abstract: This chapter addresses the:

Cited by 3 publications

References 158 publications

Sjögren’s syndrome and acquired splenic atrophy with septic shock: a case report

Sjögren’s syndrome and acquired splenic atrophy with septic shock: a case report

Movement and Other Neurodegenerative Syndromes in Patients with Systemic Rheumatic Diseases

The Role of Regulatory T Cells in the Onset and Progression of Primary Sjögren’s Syndrome

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