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Sicca Syndrome

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https://www.readbyqxmd.com/read/29243035/systemic-lupus-erythematosus-and-ocular-involvement-an-overview
#1
REVIEW
Rosanna Dammacco
Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease of undefined etiology and with remarkably heterogeneous clinical features. Virtually any organ system can be affected, including the eye. SLE-related eye involvement can be diagnosed in approximately one-third of the patients and is usually indicative of disease activity. An early diagnosis and the adoption of suitable therapeutic measures are necessary to prevent sight-threatening consequences, especially in patients with juvenile SLE. Periocular lesions, such as eyelid involvement and orbital inflammation, are relatively rare and, in case of orbital masses, may require a biopsy control...
December 14, 2017: Clinical and Experimental Medicine
https://www.readbyqxmd.com/read/29231165/polyvalent-immunoglobulins-with-vitamin-d3-and-vitamin-b12-in-the-treatment-of-sjogren-s-syndrome-in-a-vegetarian-with-stomatitis-glossodynia-xerostomia-and-elevated-antinuclear-antibodies-case-report%C3%A2
#2
Clemens Cuny, Barbara Vaerst, Jennis Gabrielpillai, Aykut Tahtali, Sven Balster, Reinhard Lissner, Barry G Woodcock
BACKGROUND: Sjogren's syndrome, involving sicca symptoms with xerostomia, stomatitis, and considerable pain is a difficult-to-treat autoimmune disease where the treatment options are limited and, as in the case of methotrexate, have a low therapeutic index. CASE REPORT: This case report concerns a male patient, aged 75 years and vegetarian, with Sjogren's syndrome subsequently confirmed by salivary gland biopsy. Serum antinuclear antibodies (ANA) were elevated (1 : 320)...
December 12, 2017: International Journal of Clinical Pharmacology and Therapeutics
https://www.readbyqxmd.com/read/29209436/the-potential-relationship-between-flammer-and-sj%C3%A3-gren-syndromes-the-chime-of-dysfunction
#3
REVIEW
Babak Baban, Olga Golubnitschaja
Flammer syndrome (FS) is a term to blanket a cluster of vascular and nonvascular signs and symptoms linked to primary vascular dysregulation (PVD), increased sensitivity to various stimuli (stress, drugs, etc.) and altered sense regulation such as pain, smell and thirst perception. On one hand, disruption of blood barrier and homeostasis of the body are the main targets of vascular irregularity. Inflammation and immune disorders including autoimmunity are considered as a consequence of the abnormal vascular regulation processes...
December 2017: EPMA Journal
https://www.readbyqxmd.com/read/29208023/comparison-of-2002-aecg-and-2016-acr-eular-classification-criteria-and-added-value-of-salivary-gland-ultrasonography-in-a-patient-cohort-with-suspected-primary-sj%C3%A3-gren-s-syndrome
#4
Maëlle Le Goff, Divi Cornec, Sandrine Jousse-Joulin, Dewi Guellec, Sebastian Costa, Thierry Marhadour, Rozenn Le Berre, Steeve Genestet, Béatrice Cochener, Sylvie Boisrame-Gastrin, Yves Renaudineau, Jacques-Olivier Pers, Alain Saraux, Valérie Devauchelle-Pensec
BACKGROUND: The objective was to evaluate concordance between 2002 American-European Consensus Group (AECG) and 2016 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria for primary Sjögren's syndrome (pSS) and to assess how salivary gland ultrasonography (SGUS) might improve the classification of patients. METHODS: Patients with suspected pSS underwent a standardised evaluation, including SGUS, at inclusion into the single-centre Brittany DIApSS cohort...
December 6, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/29148407/minor-salivary-gland-fibrosis-in-sj%C3%A3-gren-s-syndrome-is-elevated-associated-with-focus-score-and-not-solely-a-consequence-of-aging
#5
Kerry M Leehan, Nathan P Pezant, Astrid Rasmussen, Kiely Grundahl, Jacen S Moore, Lida Radfar, David M Lewis, Donald U Stone, Christopher J Lessard, Nelson L Rhodus, Barbara M Segal, R Hal Scofield, Kathy L Sivils, Courtney Montgomery, A Darise Farris
OBJECTIVES: Evaluate the presence of minor salivary gland (SG) fibrosis in primary Sjögren's syndrome (pSS) as a function of disease pathology or a consequence of ageing. METHODS: Subjects with sicca symptoms attending a Sjögren's research clinic were classified by American European Consensus Group (AECG) criteria as either pSS or non-SS (nSS). Discovery (n=34 pSS, n=28 nSS) and replication (n=35 pSS, n=31 nSS) datasets were evaluated. Minor SG cross-sections from haematoxylin and eosin stained slides were imaged, digitally reconstructed and analysed for percent area fibrosis...
October 23, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29130141/sj%C3%A3-gren-s-syndrome-initially-presented-as-thrombotic-thrombocytopenic-purpura-in-a-male-patient-a-case-report-and-literature-review
#6
REVIEW
Xiaohan Xu, Tienan Zhu, Di Wu, Lu Zhang
Thrombotic thrombocytopenic purpura (TTP) is a potentially lethal multisystem disorder which could be caused by autoimmune diseases. However, the concomitant occurrence of TTP and Sjögren's syndrome (SS) is an extremely uncommon scenario, especially in male patients. A 56-year-old Chinese male was admitted for the appearance of diffuse ecchymosis. Then he gradually developed transient slurred speech, progressive confusion, agitation, extremity weakness, and fever. Laboratory investigations suggested anemia, thrombocytopenia, significantly increased lactic dehydrogenase, schistocytes in peripheral blood smear, and a disintegrin-like metalloproteinase with thrombospondin motif type 1 member 13 (ADAMTS13) activity deficiency with high inhibitor titers...
November 13, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/29127143/satb1-conditional-knockout-results-in-sj%C3%A3-gren-s-syndrome-in-mice
#7
Yuriko Tanaka, Takehiko Sotome, Akiko Inoue, Takanori Mukozu, Taku Kuwabara, Tetuo Mikami, Terumi Kowhi-Shigematsu, Motonari Kondo
Sjögren's syndrome (SS) is an autoimmune disease in which exocrine tissues are affected by cellular and humoral immunity. As a result, the salivary and lacrimal glands of patients with SS are damaged, leading to xerostomia (dry mouth) and keratoconjunctivitis sicca (dry eyes). Because experimental approaches to investigate SS pathogenesis in human patients are limited, development of a mouse model is indispensable for understanding the disease. In this study, we show that special AT-rich sequence binding protein-1 conditional knockout (SATB1cKO) mice, in which the SATB1 gene is specifically deleted from hematopoietic cells, develop SS by 4 wk of age, soon after weaning...
November 10, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/29074164/defective-regulation-of-l1-endogenous-retroelements-in-primary-sjogren-s-syndrome-and-systemic-lupus-erythematosus-role-of-methylating-enzymes
#8
Clio P Mavragani, Adrianos Nezos, Irina Sagalovskiy, Surya Seshan, Kyriakos A Kirou, Mary K Crow
OBJECTIVE: To investigate whether altered DNA methylation contributes to the inappropriate expression of LINE-1 (L1) retroelements in primary Sjogren's syndrome (SS) and systemic lupus erythematosus (SLE). METHODS: Minor salivary glands (MSG) were obtained from 42 patients with primary SS [23 without adverse predictors for lymphoma development (SS-low risk), 7 SS-high risk and 12 complicated by B-cell lymphoma (SS-lymphoma)] and 17 sicca controls (SC). Additionally, kidney biopsy specimens and PBMCs were obtained from 23 and 73 lupus patients, respectively...
October 23, 2017: Journal of Autoimmunity
https://www.readbyqxmd.com/read/29049933/systemic-autoimmune-diseases-are-associated-with-an-increased-risk-of-bipolar-disorder-a-nationwide-population-based-cohort-study
#9
Ling-Yi Wang, Jen-Huai Chiang, Shih-Fen Chen, Yu-Chih Shen
BACKGROUND: Studies suggested autoimmunity plays a role in the etiology of bipolar disorder (BD). This study aimed to investigate the association between systemic autoimmune diseases (SADs) and the subsequent development of BD, and examine the potential risk factors for developing BD. METHODS: Patients with SADs were identified in the Taiwan National Health Insurance Program (NHIP). A comparison cohort was created by matching patients without SADs with age. The SADs cohort consisted of 65,498 while the comparison cohort consisted of 261,992 patients...
October 7, 2017: Journal of Affective Disorders
https://www.readbyqxmd.com/read/29018565/health-related-quality-of-life-and-depression-among-participants-in-the-sj%C3%A3-gren-s-international-collaborative-clinical-alliance-registry
#10
Annie Chou, John A Gonzales, Troy E Daniels, Lindsey A Criswell, Stephen C Shiboski, Caroline H Shiboski
OBJECTIVE: To examine health-related quality of life (HRQoL) and depression among participants in an international Sjögren's syndrome (SS) registry, comparing those with and without SS. METHODS: Cross-sectional study of participants in the Sjögren's International Collaborative Clinical Alliance (SICCA) registry. The 2016 American College of Rheumatology/European League Against Rheumatism SS classification criteria were used to determine disease status. HRQoL was assessed using the Short Form 12, version 2 Health Survey to derive scores for physical component summary (PCS) and mental component summary (MCS)...
2017: RMD Open
https://www.readbyqxmd.com/read/28988489/fatty-infiltration-of-the-minor-salivary-glands-is-a-selective-feature-of-aging-but-not-sj%C3%A3-gren-s-syndrome
#11
Kerry M Leehan, Nathan P Pezant, Astrid Rasmussen, Kiely Grundahl, Jacen S Moore, Lida Radfar, David M Lewis, Donald U Stone, Christopher J Lessard, Nelson L Rhodus, Barbara M Segal, C Erick Kaufman, R Hal Scofield, Kathy L Sivils, Courtney Montgomery, A Darise Farris
OBJECTIVE: Determine the presence and assess the extent of fatty infiltration of the minor salivary glands (SG) of primary SS patients (pSS) as compared to those with non-SS sicca (nSS). METHODS: Minor SG biopsy samples from 134 subjects with pSS (n = 72) or nSS (n = 62) were imaged. Total area and fatty replacement area for each glandular cross-section (n = 4-6 cross-sections per subject) were measured using Image J (National Institutes of Health, Bethesda, MD)...
October 8, 2017: Autoimmunity
https://www.readbyqxmd.com/read/28980905/clinical-and-epidemiological-differences-between-men-and-women-with-systemic-sclerosis-a-study-in-a-spanish-systemic-sclerosis-cohort-and-literature-review
#12
Mayka Freire, Alberto Rivera, Bernardo Sopeña, Carles Tolosa Vilella, Alfredo Guillén-Del Castillo, Dolores Colunga Argüelles, Jose Luis Callejas Rubio, Manuel Rubio Rivas, Luis Trapiella Martínez, Jose Antonio Todolí Parra, Mónica Rodríguez Carballeira, Nerea Iniesta Arandia, Francisco José García Hernández, María Victoria Egurbide Arberas, Luis Sáez Comet, Jose Antonio Vargas Hitos, Juan José Ríos Blanco, Adela Marín Ballvé, Xavier Pla Salas, Ana Belén Madroñero Vuelta, Manuel Ruiz Muñoz, Vicent Fonollosa Pla, Carmen Pilar Simeón Aznar
OBJECTIVES: The low overall prevalence of systemic sclerosis (SSc) and the low proportion of male patients have resulted in a scarcity of studies assessing sex differences in Ssc patients, and contradictory results have often been show among those studies that have been performed. METHODS: A prospective study was conducted with the Spanish RESCLE register to analyse the influence of gender on survival of SSc patients. RESULTS: In total, 1506 SSc patients (1341 women, 165 men) were recruited from 21 centres...
September 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28963698/hereditary-lysozyme-amyloidosis-with-sicca-syndrome-digestive-arterial-and-tracheobronchial-involvement-case-based-review
#13
REVIEW
Audrey Benyamine, Fanny Bernard-Guervilly, Céline Tummino, Nicolas Macagno, Laurent Daniel, Sophie Valleix, Brigitte Granel
Lysozyme amyloidosis (ALys) is a rare autosomal dominant hereditary systemic amyloidosis associated with a large spectrum of clinical manifestations. ALys phenotype mainly involves the digestive tract, liver and spleen, kidneys, lymph nodes, skin, and lachrymal and salivary glands. Very recently, cardiac involvement and peripheral neuropathy associated with a new p.Leu102Ser variant of lysozyme have been documented. In the present observation, we extend the phenotypic heterogeneity of ALys to the tracheobronchial tree with histologically proven bronchial ALys-amyloid deposits...
November 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28913747/primary-sj%C3%A3-gren-s-syndrome-extraglandular-manifestations-and-hydroxychloroquine-therapy
#14
J Demarchi, S Papasidero, M A Medina, D Klajn, R Chaparro Del Moral, O Rillo, V Martiré, G Crespo, A Secco, A Catalan Pellet, C Amitrano, C Crow, C Asnal, P Pucci, F Caeiro, N Benzanquen, J P Pirola, M Mayer, F Zazzetti, S Velez, J Barreira, N Tamborenea, L Santiago, L Raiti
The use of hydroxychloroquine (HCQ) in Primary Sjögren's Syndrome (pSS) has been assessed in different studies over the last years, with conflicting results regarding its efficacy in sicca syndrome and extraglandular manifestations (EGM). The goal of this study was to compare the incidence rate of EGM in pSS patients with and without HCQ therapy.We performed a multicenter retrospective study, including patients with pSS (European classification criteria) with at least 1 year of follow-up. Subjects with concomitant fibromyalgia, autoimmune hepatitis, primary biliary cirrhosis, and primary sclerosing cholangitis were excluded...
November 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28883442/interdisciplinary-comprehensive-oral-and-ocular-evaluation-of-patients-with-primary-sj%C3%A3-gren-s-syndrome
#15
Behzod Tashbayev, Shermin Rusthen, Alix Young, Bente Brokstad Herlofson, Lene Hystad Hove, Preet Bano Singh, Morten Rykke, Lara Adnan Aqrawi, Xiangjun Chen, Øygunn Aass Utheim, Tor Paaske Utheim, Øyvind Palm, Janicke Liaaen Jensen
A comprehensive evaluation of oral and ocular symptoms and findings in primary Sjögren's syndrome (pSS) patients may provide valuable information for management. Medical history was obtained from female pSS patients, and sex- and age-matched non-SS patients with sicca symptoms (non-SS sicca controls) as well as healthy subjects without sicca complaints (healthy controls). Oral (Summated Xerostomia Inventory, SXI) and ocular (McMonnies Dry Eye questionnaire, MDEIS, and Ocular Surface Disease Index, OSDI) subjective complaints were recorded...
September 7, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28871434/cutaneous-and-mucosal-manifestations-of-sj%C3%A3-gren-s-syndrome
#16
REVIEW
Elena Generali, Antonio Costanzo, Carlo Mainetti, Carlo Selmi
Sjögren's syndrome is currently considered an "autoimmune epithelitis," as exocrine glands, especially salivary and lacrimal, are progressively destructed by an immune-mediated process associated with specific serum autoantibodies and local lymphocyte infiltrate. Xerostomia remains a key complain in patients with Sjögren's syndrome but should be evaluated also for other causes such as xerogenic medications, followed by radiation and chemotherapy for head and neck cancers, hormone disorders, infections, or other connective tissue diseases...
September 4, 2017: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/28844048/traumatic-brain-injury-dry-eye-and-comorbid-pain-diagnoses-in-us-veterans
#17
Charity J Lee, Elizabeth R Felix, Roy C Levitt, Christopher Eddy, Elizabeth A Vanner, William J Feuer, Constantine D Sarantopoulos, Anat Galor
AIMS: The purpose of the study is to evaluate the relationship between dry eye (DE) and pain diagnoses in US veterans with and without traumatic brain injury (TBI). METHODS: Retrospective cohort study of veterans who were seen in the Veterans Administration Hospital (VA) between 1 January 2010 and 31 December 2014. Veterans were separated into two groups by the presence or absence of an International Classification of Diseases, Ninth Revision diagnosis of TBI and assessed for DE and other comorbidities...
August 26, 2017: British Journal of Ophthalmology
https://www.readbyqxmd.com/read/28839447/hypokalemic-paralysis-a-hidden-card-of-several-autoimmune-diseases
#18
Yelitza Velarde-Mejía, Rocío Gamboa-Cárdenas, Manuel Ugarte-Gil, César Pastor Asurza
Acute hypokalemic paralysis is a rare and potentially fatal condition, with few related causes, one of which highlights distal renal tubular acidosis (dRTA). Distal renal tubular acidosis is a rare complication of several autoimmune diseases such as systemic lupus erythematosus, Sjögren's syndrome, and Hashimoto thyroiditis. We report a case of a lupic patient who presented rapidly progressive quadriparesis in the context of active renal disease. Research revealed severe refractory hypokalemia, metabolic acidosis, and alkaline urine suggestive of dRTA...
2017: Clinical Medicine Insights. Arthritis and Musculoskeletal Disorders
https://www.readbyqxmd.com/read/28804486/first-association-of-interleukin-12-receptor-beta-1-deficiency-with-sj%C3%A3-gren-s-syndrome
#19
Georgios Sogkas, Faranaz Atschekzei, Vivien Schacht, Christian von Falck, Alexandra Jablonka, Roland Jacobs, Matthias Stoll, Torsten Witte, Reinhold E Schmidt
INTRODUCTION: Interleukin 12 receptor beta 1 (IL12Rβ1) deficiency is a primary immunodeficiency resulting mainly in susceptibility to opportunistic infection by non-tuberculous, environmental mycobacteria and severe infection caused by Salmonella spp. Till now, less than 300 patients with IL12Rβ1 deficiency have been reported. Among them, only three have been described to develop autoimmunity. CASE PRESENTATION: We present the case of a 50-year-old male with IL12Rβ1 deficiency due to compound heterozygosity [c...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28743286/cd4-t-lymphocyte-autophagy-is-upregulated-in-the-salivary-glands-of-primary-sj%C3%A3-gren-s-syndrome-patients-and-correlates-with-focus-score-and-disease-activity
#20
Cristiano Alessandri, Francesco Ciccia, Roberta Priori, Elisa Astorri, Giuliana Guggino, Riccardo Alessandro, Aroldo Rizzo, Fabrizio Conti, Antonina Minniti, Cristiana Barbati, Marta Vomero, Monica Pendolino, Annacarla Finucci, Elena Ortona, Tania Colasanti, Marina Pierdominici, Walter Malorni, Giovanni Triolo, Guido Valesini
BACKGROUND: Primary Sjögren's syndrome (pSS) is a common chronic autoimmune disease characterized by lymphocytic infiltration of exocrine glands and peripheral lymphocyte perturbation. In the current study, we aimed to investigate the possible pathogenic implication of autophagy in T lymphocytes in patients with pSS. METHODS: Thirty consecutive pSS patients were recruited together with 20 patients affected by sicca syndrome and/or chronic sialoadenitis and 30 healthy controls...
July 25, 2017: Arthritis Research & Therapy
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