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https://www.readbyqxmd.com/read/28337967/one-year-in-review-2017-primary-sj%C3%A3-gren-s-syndrome
#1
REVIEW
Francesco Ferro, Elisa Marcucci, Martina Orlandi, Chiara Baldini, Elena Bartoloni-Bocci
Primary Sjögren's syndrome (pSS) is a complex and heterogeneous disease. Last year, a great deal of basic and clinical research was carried out in pSS. Following the previous reviews of this publishing series, we will herewith provide a critical digest of the most recent literature on pSS pathogenesis, clinical manifestations and treatment. More specifically, we will focus on the heterogeneity of the disease, on the underlying pathogenetic pathways and on the possible new targeted treatments on the horizon...
March 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28296253/rituximab-in-the-treatment-of-sj%C3%A3-gren-s-syndrome-is-it-the-wrong-drug
#2
EDITORIAL
Hendrika Bootsma, Frans G M Kroese, Arjan Vissink
No abstract text is available yet for this article.
March 10, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28288719/marginal-zone-lymphoma-associated-autoimmunity-and-auto-immune-disorders
#3
REVIEW
Larissa Sena Teixeira Mendes, Andrew Wotherspoon
Large epidemiological studies have shown a consistent increased risk for developing lymphoma in the setting of autoimmune disorders (AID). It is known that this link appears to be stronger for some AID and certain non-Hodgkin lymphoma subtypes e.g. Sjögren's syndrome and extra-nodal marginal zone lymphoma of the salivary gland, and thyroid MALT lymphoma in a background of Hashimoto's thyroiditis. B and T-cell hyperactivity due to chronic antigenic stimulation and the consequent presence of acquired lymphoid tissue seems to play a key role in the pathogenesis of AI-related lymphomas...
March 2017: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/28276715/inflammation-in-sj%C3%A3-gren-s-syndrome-cause-or-consequence
#4
Ana Raquel Rodrigues, Raquel Soares
Sjögren's syndrome (SS) is an autoimmune disease most commonly characterized by ocular and oral dryness. Despite the high prevalence of SS, generation and perpetuation of this disease is still unclear in many aspects. Inflammation, nonetheless, seems to play a central role in this pathology especially in the form of Th-1, Th-2 and Th-17 cytokines release within different aspects, concentrations and connections involved in the maintenance of the syndrome. Moreover, the chronically created pro-inflammatory environment appears to promote glandular atrophy and irreparable architectural modifications...
February 14, 2017: Autoimmunity
https://www.readbyqxmd.com/read/28272192/optic-neuropathy-associated-with-primary-sj%C3%A3-gren-s-syndrome-a-case-series
#5
Eunoo Bak, Hee Kyung Yang, Jeong-Min Hwang
PURPOSE: To determine the diverse clinical features of optic neuropathy associated with primary Sjögren's syndrome in Korean patients. METHODS: Five women with acute and/or chronic optic neuropathy who were diagnosed as primary Sjögren's syndrome were retrospectively evaluated. Primary Sjögren's syndrome was diagnosed by signs and symptoms of keratoconjunctivitis sicca, positive serum anti-Ro/SSA and/or anti-La/SSB antibodies, and/or minor salivary gland biopsy...
March 7, 2017: Optometry and Vision Science: Official Publication of the American Academy of Optometry
https://www.readbyqxmd.com/read/28257279/intrathecal-baclofen-therapy-for-the-treatment-of-spasticity-in-sj%C3%A3-gren-larsson-syndrome
#6
Eveline Teresa Hidalgo, Cordelia Orillac, Andrew Hersh, David H Harter, William B Rizzo, Howard L Weiner
Intrathecal baclofen therapy is widely accepted as a treatment option for patients with severe spasticity. The current treatment of spasticity in patients with Sjögren-Larsson syndrome is largely symptomatic, given that no effective causal therapy treatments are available. We report the outcome of 2 patients with Sjögren-Larsson syndrome who had pump implantation for intrathecal baclofen. We observed a positive response, with a decrease of spasticity, reflecting in the Modified Ashworth Scale, and parents and caregivers observed a functional improvement in both patients...
January 2017: Journal of Child Neurology
https://www.readbyqxmd.com/read/28245520/autoantibodies-to-box-a-of-high-mobility-group-box-1-in-systemic-lupus-erythematosus
#7
F Schaper, K de Leeuw, G Horst, F Maas, H Bootsma, P Heeringa, P C Limburg, J Westra
OBJECTIVE: Auto-antibodies to nuclear structures are a hallmark of systemic lupus erythematosus (SLE), including auto-antibodies to nuclear protein High Mobility Group Box-1 (HMGB1). HMGB1 consists of three separate domains: Box A, Box B and an acidic tail. Recombinant Box A acts as a competitive antagonist for HMGB1 and might be an interesting treatment option in SLE. However, antibodies to Box A might interfere. Therefore, levels of anti-Box A were examined in SLE patients in association with disease activity and clinical parameters...
February 28, 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/28245347/autologous-serum-eye-drops-for-dry-eye
#8
REVIEW
Qing Pan, Adla Angelina, Michael Marrone, Walter J Stark, Esen K Akpek
BACKGROUND: Theoretically, autologous serum eye drops (AS) offer a potential advantage over traditional therapies on the assumption that AS not only serve as a lacrimal substitute to provide lubrication but contain other biochemical components that allow them to mimic natural tears more closely. Application of AS has gained popularity as second-line therapy for patients with dry eye. Published studies on this subject indicate that autologous serum could be an effective treatment for dry eye...
February 28, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28240585/fibromyalgia-prevalence-and-associated-factors-in-primary-sj%C3%A3-gren-s-syndrome-patients-in-a-large-cohort-from-the-spanish-society-of-rheumatology-registry-sjogrenser
#9
Vicenç Torrente-Segarra, Hèctor Corominas, Carlos Sánchez-Piedra, Mónica Fernández-Castro, José Luis Andreu, Víctor Manuel Martínez-Taboada, Alejandro Olivé, José Rosas, Fernando Sánchez-Alonso
OBJECTIVES: To assess fibromyalgia (FM) prevalence in a large cohort of primary Sjögren's syndrome patients (pSS) from a National Database. METHODS: Data included in the national retrospective register of pSS patients of the Spanish Society of Rheumatology (SJOGRENSER) were analysed. RESULTS: 437 pSS patients were included and a 14.6% of FM prevalence was found. FM-pSS patients significantly showed more constitutional, fatigue and arthralgia symptoms, splenomegaly, genital, skin and ear involvement and dyslipidaemia (p<0...
February 24, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28229827/anti-la-positive-anti-ro-negative-subset-of-primary-sj%C3%A3-gren-s-syndrome-anti-la-is-a-reality-but-is-the-disease
#10
Debashish Danda, Rohan Sharma, Dat Truong, Kristi A Koelsch, Biji T Kurien, Harini Bagavant, Umesh Deshmukh, C Erick Kaufman, David M Lewis, Donald U Stone, Lida Radfar, Astrid Rasmussen, Kathy L Sivils, Robert H Scofield
OBJECTIVES: To characterise the serological and clinical findings in primary Sjögren's syndrome in which anti-La was found without anti-Ro. We hypothesised that a significant portion of these are falsely negative for anti-Ro60. METHODS: Twenty-nine sera from primary Sjögren's syndrome patients were tested for antibodies directed against La and Ro. Anti-La was detected using bovine La treated with or without DNAase and RNAase to identify potential false positivity...
February 20, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28229405/compact-bone-derived-multipotent-mesenchymal-stromal-cells-mscs-for-the-treatment-of-sjogren-s-like-disease-in-nod-mice
#11
Ghada Abu Elghanam, Younan Liu, Saeed Khalili, Dongdong Fang, Simon D Tran
Compact bone (cortical or dense bone) is among the organs that contain multipotent mesenchymal stromal cells (MSCs). Unlike bone marrow plugs where MSCs were initially isolated, compact bone has minimal (amount of) hematopoietic cells and thus facilitates the MSCs isolation process. In vitro, MSCs from compact bone show multipotency and differentiation into mesenchymal tissues such as bone, adipose, and cartilage, under certain conditions. MSCs therapy has been promising in preclinical and clinical studies against autoimmune diseases...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28215100/targeting-baff-and-april-in-systemic-lupus-erythematosus-and-other-antibody-associated-diseases
#12
Eileen Samy, Stephen Wax, Bertrand Huard, Henry Hess, Pascal Schneider
The B cell-stimulating molecules, BAFF (B cell activating factor) and APRIL (a proliferation-inducing ligand), are critical factors in the maintenance of the B cell pool and humoral immunity. In addition, BAFF and APRIL are involved in the pathogenesis of a number of human autoimmune diseases, with elevated levels of these cytokines detected in the sera of patients with systemic lupus erythematosus (SLE), IgA nephropathy, Sjögren's syndrome, and rheumatoid arthritis. As such, both molecules are rational targets for new therapies in B cell-driven autoimmune diseases, and several inhibitors of BAFF or BAFF and APRIL together have been investigated in clinical trials...
January 2, 2017: International Reviews of Immunology
https://www.readbyqxmd.com/read/28212920/neonatal-lupus-follow-up-in-infants-with-anti-ssa-ro-antibodies-and-review-of-the-literature
#13
REVIEW
Antonio Alberto Zuppa, Riccardo Riccardi, Simonetta Frezza, Francesca Gallini, Rita Maria Paola Luciano, Giovanni Alighieri, Costantino Romagnoli, Sara De Carolis
Neonatal Lupus Syndrome (NLS) is a distinct clinical entity caused by transplacental passage of maternal anti-SSA/Ro antibodies (Ab). Mothers may have systemic lupus erythematosus, Sjögren syndrome, or other connective tissue disease, or may be completely healthy at the time of giving birth. NLS includes several clinical manifestations: complete congenital heart block (CCHB) and cutaneous lupus are the most common, while hepatobiliary disease, hematological manifestations and central nervous system involvement may occur...
February 14, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28207432/spontaneous-canalicular-plug-erosion-after-punctal-plug-placement
#14
Xinxin Zhang, Maya Bitar, Richard M Davis
PURPOSE: To describe a case of keratoconjunctivitis sicca treated with punctal plug placement with the first reported complication of subsequent plug erosion through the canaliculus and extrusion through the lid. METHODS: Punctal plugs were placed in a patient with Sjogren syndrome for treatment of keratoconjunctivitis sicca. More than 12 years later, the plug was noted to have eroded through the canaliculus and extruded through the conjunctiva and lid. RESULTS: The extruded plug was removed, and punctal cautery was performed on 2 other puncta with improved symptom control...
February 15, 2017: Cornea
https://www.readbyqxmd.com/read/28202919/ectopic-lymphoid-neogenesis-in-rheumatic-autoimmune-diseases
#15
REVIEW
Michele Bombardieri, Myles Lewis, Costantino Pitzalis
Ectopic lymphoid neogenesis often occurs in the target tissues of patients with chronic rheumatic autoimmune diseases such as rheumatoid arthritis, Sjögren syndrome and other connective tissue disorders, including systemic lupus erythematosus and myositis. However, the mechanisms of ectopic lymphoid-like structure (ELS) formation and function are not entirely understood. For example, it is unclear whether ELSs indicate distinct disease phenotypes or whether they are evolutionary manifestations of chronic inflammation...
March 2017: Nature Reviews. Rheumatology
https://www.readbyqxmd.com/read/28199035/utility-approach-to-decision-making-in-extended-t1-and-limited-t2-glottic-carcinoma
#16
Yda van Loon, Anne M Stiggelbout, Marieke M Hakkesteegt, Ton P M Langeveld, Rob J Baatenburg de Jong, Elisabeth V Sjögren
BACKGROUND: It is still undecided if endoscopic laser surgery or radiotherapy is the preferable treatment in extended T1 and limited T2 glottic tumors. Health utilities assessed from patients can aid in decision-making. METHODS: Patients treated for extended T1 or limited T2 glottic carcinoma by laser surgery (n = 12) or radiotherapy (n = 14) assigned health utilities using a visual analog scale (VAS), time tradeoff (TTO) technique and scored their voice handicap using the Voice Handicap Index (VHI)...
February 15, 2017: Head & Neck
https://www.readbyqxmd.com/read/28197771/granulomatous-interstitial-nephritis-associated-with-primary-sj%C3%A3-gren-s-syndrome
#17
B Bitik, I I Gonul, S Haznedaroglu, B Goker, A Tufan
Primary Sjögren's syndrome (pSS) is an autoimmune disease characterized by lymphocytic and plasmacytic infiltration of the exocrine glands. Tubulointerstitial nephritis (TIN) is the most common type of renal involvement in pSS. However, clinically significant renal involvement is uncommon. Granulomatous interstitial nephritis (GIN) is a rare histopathological entity characterized by the presence of granulomas against a background of interstitial inflammation. GIN is not a typical and commonly seen form of TIN in pSS...
February 14, 2017: Zeitschrift Für Rheumatologie
https://www.readbyqxmd.com/read/28185656/subacute-cutaneous-lupus-erythematosus-clinical-characteristics-disease-associations-treatments-and-outcomes-in-a-series-of-90%C3%A2-patients-at-mayo-clinic-1996-2011
#18
Dema T Alniemi, Albert Gutierrez, Lisa A Drage, David A Wetter
OBJECTIVE: To characterize the clinical presentation, laboratory studies, disease associations, and treatments of subacute cutaneous lupus erythematosus (SCLE). PATIENTS AND METHODS: A retrospective review of 90 patients with SCLE at Mayo Clinic from January 1, 1996, through October 28, 2011, was performed. RESULTS: The mean patient age at diagnosis was 61 years; 64 patients (71%) were women, and 11 cases (12%) were drug induced (1996-2000, no drug-induced cases; 2001-2005, 2 cases; 2006-2011, 9 cases)...
March 2017: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/28177920/predictable-biomarkers-of-developing-lymphoma-in-patients-with-sj%C3%A3-gren-syndrome-a-nationwide-population-based-cohort-study
#19
Yu-Hsiang Chiu, Chi-Hsiang Chung, Kuen-Tze Lin, Chin-Sheng Lin, Jia-Hong Chen, Hsiang-Cheng Chen, Ren-Yeong Huang, Chi-Tsung Wu, Feng-Cheng Liu, Wu-Chien Chien
Sjögren syndrome (SS) is commonly known to be correlated with lymphoma. This study included 16,396 individuals in the SS cohort and 65,584 individuals in the non-SS cohort, all of whom were enrolled in the Taiwan National Health Insurance database between 2000 and 2010. We evaluated the risk factors of non-Hodgkin's lymphoma (NHL) in the primary SS cohort by applying a Cox multivariable proportional-hazards model. We increased the correlation of patients with SS and NHL, with an adjusted HR of 4.314 (95% CI 2...
February 4, 2017: Oncotarget
https://www.readbyqxmd.com/read/28150697/neutralizing-antibodies-against-adeno-associated-viruses-in-sj%C3%A3-gren-s-patients-implications-for-gene-therapy
#20
A Corden, B Handelman, H Yin, A Cotrim, I Alevizos, J A Chiorini
One potential setback to the use of gene therapy for the treatment of Sjögren's syndrome is the presence of neutralizing antibodies (nAb) against adeno-associated virus (AAV) serotypes. In order to evaluate the efficacy of this treatment option, nAb titers were measured in both healthy individuals and Sjögren's patients. Several serotypes with known transduction activity in mouse salivary glands were tested and only AAV5 showed a statistically significant change in the prevalence of nAbs between Sjögren's and healthy participants...
February 2, 2017: Gene Therapy
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