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Keywords = Neuro-Sjögren

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15 pages, 670 KiB  
Review
Therapeutic Targets in the Management of Dry Eye Disease Associated with Sjögren’s Syndrome: An Updated Review of Current Insights and Future Perspectives
by Abdulmohsen Almulhim
J. Clin. Med. 2024, 13(6), 1777; https://doi.org/10.3390/jcm13061777 - 20 Mar 2024
Cited by 5 | Viewed by 3639
Abstract
Dry eye disease (DED) is a continuing medical challenge, further worsened in the autoimmune inflammatory hyperactivation milieu of Sjögren’s syndrome (SS) due to disturbances to innate and adaptive immunity with malfunctioning neuro-endocrine control. However, the pathogenetic mechanisms of SS DED are not fully [...] Read more.
Dry eye disease (DED) is a continuing medical challenge, further worsened in the autoimmune inflammatory hyperactivation milieu of Sjögren’s syndrome (SS) due to disturbances to innate and adaptive immunity with malfunctioning neuro-endocrine control. However, the pathogenetic mechanisms of SS DED are not fully established. This review summarized the available evidence, from systematic reviews, meta-analyses, and randomized clinical trials, for the efficacy and safety of the available ocular therapeutics for the management of SS DED. Relevant studies were obtained from major databases using appropriate keywords. The available largely empirical symptomatic, supportive, and restorative treatments have significant limitations as they do not alter local and systemic disease progression. Topical therapies have expanded to include biologics, surgical approaches, scleral lens fitting, the management of lid margin disease, systemic treatments, nutritional support, and the transplantation of stem cells. They are not curative, as they cannot permanently restore the ocular surface’s homeostasis. These approaches are efficacious in the short term in most studies, with more significant variability in outcome measures among studies in the long term. This review offers an interdisciplinary perspective that enriches our understanding of SS DED. This updated review addresses current knowledge gaps and identifies promising areas for future research to overcome this medical challenge. Full article
(This article belongs to the Special Issue Corneal Disease: Clinical Insights and Management Approaches)
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17 pages, 757 KiB  
Review
Cerebrospinal Fluid Analysis in Rheumatological Diseases with Neuropsychiatric Complications and Manifestations: A Narrative Review
by Massimiliano Castellazzi, Raffaella Candeloro, Maura Pugliatti, Marcello Govoni, Ettore Silvagni and Alessandra Bortoluzzi
Diagnostics 2024, 14(3), 242; https://doi.org/10.3390/diagnostics14030242 - 23 Jan 2024
Cited by 3 | Viewed by 4936
Abstract
The analysis of cerebrospinal fluid (CSF) remains a valuable diagnostic tool in the evaluation of inflammatory and infectious conditions involving the brain, spinal cord, and meninges. Since many rheumatic inflammatory diseases can involve the central and peripheral nervous system, the aims of this [...] Read more.
The analysis of cerebrospinal fluid (CSF) remains a valuable diagnostic tool in the evaluation of inflammatory and infectious conditions involving the brain, spinal cord, and meninges. Since many rheumatic inflammatory diseases can involve the central and peripheral nervous system, the aims of this narrative review were to summarize the latest evidence on the use of CSF analysis in the field of neuropsychiatric manifestations of rheumatic diseases. Routine CSF parameters were taken into consideration for this review: appearance; total protein and cellular content (pleocytosis); lactate and/or glucose; CSF/serum albumin quotient; intrathecal synthesis of IgG. Data regarding the role of CSF analysis in the clinical management of neuropsychiatric systemic lupus erythematosus, primary Sjogren’s syndrome, rheumatoid arthritis, and Behçet’s syndrome are presented. Although no disease-specific picture has been identified, CSF analysis remains a useful diagnostic tool to confirm the presence of a neuro-inflammatory state or, conversely, to exclude the concomitant presence of other inflammatory/infectious diseases affecting the CNS in the context of systemic rheumatologic conditions. Full article
(This article belongs to the Section Pathology and Molecular Diagnostics)
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12 pages, 2409 KiB  
Article
Saliva Free Light Chains in Patients with Neuro-Sjögren
by Franz Felix Konen, Tabea Seeliger, Philipp Schwenkenbecher, Stefan Gingele, Konstantin Fritz Jendretzky, Kurt-Wolfram Sühs, Diana Ernst, Torsten Witte and Thomas Skripuletz
Biomedicines 2022, 10(10), 2470; https://doi.org/10.3390/biomedicines10102470 - 3 Oct 2022
Cited by 3 | Viewed by 2657
Abstract
Background: Sjögren’s syndrome (SS) is an autoimmune disease characterized by sicca symptoms and various extra-glandular manifestations. The diagnosis of SS requires sicca symptoms, anti-SSA(Ro)-antibody positivity, and/or pathological focus scores on a minor salivary gland biopsy. Previous studies have investigated different biomarkers in order [...] Read more.
Background: Sjögren’s syndrome (SS) is an autoimmune disease characterized by sicca symptoms and various extra-glandular manifestations. The diagnosis of SS requires sicca symptoms, anti-SSA(Ro)-antibody positivity, and/or pathological focus scores on a minor salivary gland biopsy. Previous studies have investigated different biomarkers in order to avoid invasive diagnostic procedures. It was found that kappa and lambda free light chains (KFLC and LFLC) in saliva are specific for SS. Methods: FLC concentrations in saliva and serum were determined in 130 patients—50 with SS and neurological involvement (Neuro-Sjögren) and 80 neurological controls. The EULAR SS disease activity index and patient reported index (ESSPRI) were determined in patients with SS. Results: Patients with SS revealed increased pain and decreased saliva production according to the ESSPRI and Saxon test, respectively, with increasing FLC concentrations in the saliva. No significant differences in serum and salivary protein concentrations were observed between patients with SS and controls. Conclusion: KFLC and LFLC concentrations in saliva are not suitable to distinguish patients with Neuro-Sjögren and neurological control subjects, thus a diagnostic biopsy is still required. The association of salivary KFLC and LFLC concentrations with saliva production and ESSPRI pain score suggests a complex relationship between dryness and pain in patients with SS. Full article
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13 pages, 1663 KiB  
Article
Trigeminal Nerve Affection in Patients with Neuro-Sjögren Detected by Corneal Confocal Microscopy
by Tabea Seeliger, Marten A. Gehlhaar, Irene Oluwatoba-Popoola, Franz F. Konen, Melanie Haar, Emilia Donicova, Marija Wachsmann, Amelie Pielen, Stefan Gingele, Nils K. Prenzler, Diana Ernst, Torsten Witte, Carsten Framme, Anna Bajor and Thomas Skripuletz
J. Clin. Med. 2022, 11(15), 4484; https://doi.org/10.3390/jcm11154484 - 1 Aug 2022
Cited by 8 | Viewed by 2630
Abstract
Background: Patients with Sjögren’s syndrome and polyneuropathy more frequently develop cranial nerve affection when compared to patients with chronic inflammatory demyelinating polyneuropathy (CIDP). We therefore aimed to analyze trigeminal corneal nerve fibre characteristics in both patient groups. Methods: A total of 26 patients [...] Read more.
Background: Patients with Sjögren’s syndrome and polyneuropathy more frequently develop cranial nerve affection when compared to patients with chronic inflammatory demyelinating polyneuropathy (CIDP). We therefore aimed to analyze trigeminal corneal nerve fibre characteristics in both patient groups. Methods: A total of 26 patients with Sjögren’s syndrome associated neuropathy and 29 patients with CIDP were recruited at our university hospital and compared to 6 healthy controls. Dry eye symptoms and signs were assessed via clinical examination and the Ocular Disease Surface Index questionnaire. Trigeminal corneal nerve fibres were analyzed via corneal confocal microscopy (CCM) as a non-invasive in vivo microscopy. Results: CCM revealed significantly reduced corneal nerve fibre density and corneal nerve fibre main branch density in the Neuro-Sjögren group when compared with healthy controls. There were no significant group differences between the Neuro-Sjögren and the CIDP group for any of the microscopic parameters. Dry eye assessment showed similarly reduced scores for both patient groups, while healthy controls showed better results for objective dry eye signs. There was no correlation between microscopic parameters of the corneal confocal microscopy and parameters of dry eye assessment. Conclusions: Our data revealed trigeminal corneal nerve affection in patients with neuropathy associated with Sjögren’s syndrome and patients with CIDP detected by CCM. No difference was found between both neuropathy groups indicating that CCM is not able to distinguish between both entities. Full article
(This article belongs to the Special Issue Corneal Confocal Microscopy and the Nervous System)
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17 pages, 400 KiB  
Review
Aseptic Meningitis in Oral Medicine: Exploring the Key Elements for a Challenging Diagnosis: A Review of the Literature and Two Case Reports
by Stefania Leuci, Noemi Coppola, Tiziana Cantile, Elena Calabria, Laurenta Lelia Mihai and Michele Davide Mignogna
Int. J. Environ. Res. Public Health 2022, 19(7), 3919; https://doi.org/10.3390/ijerph19073919 - 25 Mar 2022
Cited by 8 | Viewed by 4490
Abstract
Aseptic meningitis (AM) is a potentially severe and life-threatening disease characterized by meningeal inflammation, usually with mononuclear pleocytosis. It represents a challenging and controversial issue in medicine for multiple etiologies, classification, and difficult diagnosis in the face of nonspecific sets of signs and [...] Read more.
Aseptic meningitis (AM) is a potentially severe and life-threatening disease characterized by meningeal inflammation, usually with mononuclear pleocytosis. It represents a challenging and controversial issue in medicine for multiple etiologies, classification, and difficult diagnosis in the face of nonspecific sets of signs and symptoms. In the area of interest of oral medicine, in specific clusters of patients, even if rare, the occurrence of aseptic meningitis can pose a diagnostic and management dilemma in the following potential etiologies: (i) systemic diseases with oral and meningeal involvement, which include Behçet’s disease and Sjögren syndrome; (ii) drug-induced aseptic meningitis; (iii) aseptic viral meningitis, mostly related to herpes simplex virus infection and hand, foot, and mouth disease, caused by enteroviruses. In this review, clinical manifestations, diagnostic methodologies, incidence, treatment, and prognosis for each of these clinical entities are provided. Furthermore, two illustrative case reports are described: a patient suffering from recurrent oral ulcers, in which a sudden onset of AM allows us to diagnose Neuro Behçet’s disease, and a patient affected by pemphigus vulgaris, manifesting a drug-induced AM. Exploring this complex clinical entity scenario, it is clear that an oral medicine specialist has a place on any multidisciplinary team in making such a challenging diagnosis. Full article
(This article belongs to the Collection Relationships between Oral Health Management and Systemic Health)
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