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Sicca Syndrome

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https://www.readbyqxmd.com/read/28804486/first-association-of-interleukin-12-receptor-beta-1-deficiency-with-sj%C3%A3-gren-s-syndrome
#1
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
#2
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
https://www.readbyqxmd.com/read/28730224/histopathological-and-immunohistochemical-profile-in-primary-sj%C3%A3-gren-s-syndrome
#3
Ştefan Cristian Dinescu, Mircea Cătălin ForŢofoiu, Ana Maria Bumbea, Paulina Lucia Ciurea, Cristina Jana Busuioc, Anca Emanuela Muşetescu
Sjögren's syndrome (SS) is an autoimmune disease characterized by hypofunction of the salivary and lachrymal glands. Main clinical features of SS are sicca symptoms, due to the altered glandular function. Also, in advanced stages, bilateral swelling of the parotid glands can be noted, indicative of severe glandular involvement. Phenotypic expression of various mononuclear cells present in the affected tissue offers additional insight into cellular proliferation, survival, migration, antibody secretion and also the potential of forming tertiary lymphoid tissue, i...
2017: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
https://www.readbyqxmd.com/read/28725949/tubulointerstitial-nephritis-induced-hypophosphatemic-osteomalacia-in-sj%C3%A3-gren-s-syndrome-a-case-report-and-review-of-the-literature
#4
REVIEW
Yan Geng, Youlu Zhao, Zhuoli Zhang
Sjögren's syndrome (SS) is a chronic autoimmune inflammatory disease that typically affects the salivary and lacrimal glands. Renal involvement is relatively uncommon and may precede other complaints. Tubulointestitial nephritis (TIN) is the most common renal involvement in SS. Osteomalacia occurring as the first manifestation of renal tubular disorder due to SS is very rare. We report a 39-year-old male who presented with polydipsia, polyuria, and multiple bone pain. Bone density test showed severe osteoporosis, and laboratory findings suggested hypokalemia, hypophosphatemia, and vitamin D deficiency, which supported the diagnosis of hypophosphatemic osteomalacia...
July 20, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28689585/rheumatologic-manifestations-of-hepatitis-c-virus-infection
#5
REVIEW
Patrice Cacoub, Cloé Comarmond, Anne Claire Desbois, David Saadoun
Hepatitis C virus (HCV) infection is associated with a morbidity and mortality due to liver complications. HCV infection is also frequently associated with rheumatic disorders, such as arthralgia, myalgia, cryoglobulinemia vasculitis, and sicca syndrome, as well as the production of autoantibodies. The treatment of HCV infection with interferon alpha (IFN) has been contraindicated for a long time in many rheumatologic autoimmune/inflammatory disorders. New oral IFN-free combinations offer an opportunity for HCV-infected patients with extrahepatic manifestations, including rheumatologic autoimmune/inflammatory disorders, to be cured with a short treatment duration and a low risk of side effects...
August 2017: Clinics in Liver Disease
https://www.readbyqxmd.com/read/28686485/impaired-salivary-gland-activity-in-patients-with-autoimmune-polyendocrine-syndrome-type-i
#6
Bergithe E Oftedal, Mihaela Cuida Marthinussen, Martina M Erichsen, Maria K Tveitarås, Anja Kjellesvik-Kristiansen, Daniel Hammenfors, Malin V Jonsson, Kai Kisand, Roland Jonsson, Anette S B Wolff
Autoimmune polyendocrine syndrome type I (APS-I) is a severe disease caused by mutations in the autoimmune regulator (AIRE) gene. We hypothesized that salivary gland dysfunction could be a possible unexplored component of these patients and here aimed to investigate salivary and lachrymal symptoms in the Norwegian cohort of APS-I patients (N = 41) and the aetiology behind it. Sicca symptoms and possible corresponding underlying factors were assessed by subjective reports combined with objective measures of saliva and tear flow, serological testing, immune fluorescence microscopy, ultrasonography and searching for putative autoantibodies in the salivary glands...
July 7, 2017: Autoimmunity
https://www.readbyqxmd.com/read/28685938/acquired-gitelman-syndrome-in-a-primary-sj%C3%A3-gren-syndrome-patient-with-a-slc12a3-heterozygous-mutation-a-case-report-and-literature-review
#7
Xiangchen Gu, Zheling Su, Min Chen, Yanqiu Xu, Yi Wang
Acquired Gitelman's syndrome (GS) associated with Sjögren syndrome (SS) is rare. A 50-year-old woman was admitted to our department because of nausea, acratia and sicca complex. Laboratory tests after admission showed renal failure, hypokalaemia, metabolic alkalosis, hypomagnesaemia and hypocalciuria, all of which met the diagnostic criteria for GS. Diagnostic evaluation identified primary SS as the cause of the acquired GS. Light microscopy of the renal tissue from the patient showed severe membranoproliferative glomerunephritis and tubulointerstitial nephritis...
August 2017: Nephrology
https://www.readbyqxmd.com/read/28640813/identification-of-a-sj%C3%A3-gren-s-syndrome-susceptibility-locus-at-oas1-that-influences-isoform-switching-protein-expression-and-responsiveness-to-type-i-interferons
#8
He Li, Tove Ragna Reksten, John A Ice, Jennifer A Kelly, Indra Adrianto, Astrid Rasmussen, Shaofeng Wang, Bo He, Kiely M Grundahl, Stuart B Glenn, Corinne Miceli-Richard, Simon Bowman, Sue Lester, Per Eriksson, Maija-Leena Eloranta, Johan G Brun, Lasse G Gøransson, Erna Harboe, Joel M Guthridge, Kenneth M Kaufman, Marika Kvarnström, Deborah S Cunninghame Graham, Ketan Patel, Adam J Adler, A Darise Farris, Michael T Brennan, James Chodosh, Rajaram Gopalakrishnan, Michael H Weisman, Swamy Venuturupalli, Daniel J Wallace, Kimberly S Hefner, Glen D Houston, Andrew J W Huang, Pamela J Hughes, David M Lewis, Lida Radfar, Evan S Vista, Contessa E Edgar, Michael D Rohrer, Donald U Stone, Timothy J Vyse, John B Harley, Patrick M Gaffney, Judith A James, Sean Turner, Ilias Alevizos, Juan-Manuel Anaya, Nelson L Rhodus, Barbara M Segal, Courtney G Montgomery, R Hal Scofield, Susan Kovats, Xavier Mariette, Lars Rönnblom, Torsten Witte, Maureen Rischmueller, Marie Wahren-Herlenius, Roald Omdal, Roland Jonsson, Wan-Fai Ng, Gunnel Nordmark, Christopher J Lessard, Kathy L Sivils
Sjögren's syndrome (SS) is a common, autoimmune exocrinopathy distinguished by keratoconjunctivitis sicca and xerostomia. Patients frequently develop serious complications including lymphoma, pulmonary dysfunction, neuropathy, vasculitis, and debilitating fatigue. Dysregulation of type I interferon (IFN) pathway is a prominent feature of SS and is correlated with increased autoantibody titers and disease severity. To identify genetic determinants of IFN pathway dysregulation in SS, we performed cis-expression quantitative trait locus (eQTL) analyses focusing on differentially expressed type I IFN-inducible transcripts identified through a transcriptome profiling study...
June 2017: PLoS Genetics
https://www.readbyqxmd.com/read/28622456/elevated-ccl25-and-ccr9-expressing-t-helper-cells-in-salivary-glands-of-primary-sj%C3%A3-gren-s-syndrome-patients-potential-new-axis-in-lymphoid-neogenesis
#9
Sofie L M Blokland, Maarten R Hillen, Aike A Kruize, Stephan Meller, Bernhard Homey, Glennda M Smithson, Timothy R D J Radstake, Joel A G van Roon
Introduction T follicular helper (Tfh)-cells play a critical role in germinal center formation and B-cell activation, both hallmarks of primary Sjögren's syndrome (pSS). CCR9-expressing Th-cells have "Tfh-like" characteristics and are increased at mucosa-associated sites in several inflammatory conditions. Because of their unique characteristics and limited evaluation we investigated the local and systemic CCL25/CCR9-axis in pSS. Methods CCL25 protein and mRNA levels and CCR9+ Th-cells were assessed in labial salivary glands (LSG) of pSS and non-Sjögren's sicca (nSS) patients and their correlation with inflammatory and clinical parameters was evaluated...
June 16, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28610655/the-diagnosis-and-treatment-of-sj%C3%A3-gren-s-syndrome
#10
Ana-Luisa Stefanski, Christian Tomiak, Uwe Pleyer, Thomas Dietrich, Gerd Rüdiger Burmester, Thomas Dörner
BACKGROUND: Sjögren's syndrome is one of the more common inflammatory rheumatological diseases, with a prevalence of at least 0.4% in Germany. METHODS: This review is based on pertinent articles retrieved by a selective search in PubMed. Special attention is drawn to updated classification criteria and current treatment recommendations. RESULTS: Sjögren's syndrome has a wide variety of presentations, ranging from the local involvement of exocrine glands with keratoconjunctivitis sicca and xerostomia (the leading signs of the disease) to the systemic, extraglandular involvement of multiple organs...
May 26, 2017: Deutsches Ärzteblatt International
https://www.readbyqxmd.com/read/28598869/graft-versus-host-disease-what-should-the-oculoplastic-surgeon-know
#11
Cynthia I Tung
PURPOSE OF REVIEW: To provide a concise review of the oculoplastic manifestations of ocular graft versus host disease (GVHD), and to discuss their management. RECENT FINDINGS: Ocular GVHD occurs as a common immune-mediated complication of hematopoietic stem cell transplantation that presents as a Stevens-Johnson-like syndrome in the acute phase or a Sjögren-like syndrome in the chronic phase. Cicatricial conjunctivitis may be underreported in ocular GVHD. The spectrum of oculoplastic manifestations includes GVHD of the skin, cicatricial entropion, nasolacrimal duct obstruction, and lacrimal gland dysfunction...
June 8, 2017: Current Opinion in Ophthalmology
https://www.readbyqxmd.com/read/28596045/comorbidity-in-chronic-fatigue-syndrome-myalgic-encephalomyelitis-a-nationwide-population-based-cohort-study
#12
Jesús Castro-Marrero, Mónica Faro, Luisa Aliste, Naia Sáez-Francàs, Natalia Calvo, Alba Martínez-Martínez, Tomás Fernández de Sevilla, Jose Alegre
BACKGROUND: Previous studies have shown evidence of comorbid conditions in chronic fatigue syndrome/myalgic encephalomyelitis (CFS/ME). OBJECTIVE: To estimate the prevalence of comorbidities and assess their associations using a nationwide population-based database of a Spanish CFS/ME cohort. METHOD: A nationally representative, retrospective, cross-sectional cohort study (2008-2015) assessed 1757 Spanish subjects who met both the 1994 Centers for Disease Control and Prevention/Fukuda definition and 2003 Canadian Criteria for CFS/ME...
April 21, 2017: Psychosomatics
https://www.readbyqxmd.com/read/28538612/scleral-contact-lenses-in-an-academic-oculoplastics-clinic-epidemiology-and-emerging-considerations
#13
Harinder S Chahal, Marcela Estrada, Christine W Sindt, Jacob A Boehme, Mark A Greiner, Jeffrey A Nerad, Keith D Carter, Richard C Allen, Erin M Shriver
PURPOSE: To describe the role and efficacy of scleral contact lenses (SCLs) in the treatment of progressive keratopathy in patients who have undergone periocular surgical procedures, to investigate the financial impact of these surgical interventions, and to demonstrate the role of oculoplastic surgery in improving scleral contact lens fit. METHODS: A retrospective medical record review was performed to identify patients who both received SCLs and were examined by the oculoplastics service at the University of Iowa between January 1990 and December 2015...
May 22, 2017: Ophthalmic Plastic and Reconstructive Surgery
https://www.readbyqxmd.com/read/28528128/comparison-of-the-diagnostic-value-of-four-scoring-systems-in-primary-sj%C3%A3-gren-s-syndrome-patients
#14
Xuan Qi, Chao Sun, Yu Tian, Yuxiang Han, Chenxing Peng, Hongtao Jin, Lixia Gao, Huifang Guo
PURPOSE: We attempted to evaluate the diagnostic value of different salivary gland ultrasonography (SGUS) scoring systems for primary sjögren's syndrome (pSS). METHODS: Total 134 patients with pSS and 109 non-SS sicca controls were included in our study. All the cases were evaluated by four scoring systems (such as 0-16 SGUS, 0-3 SGUS, Parotid glands and Submandibular salivary glands scoring system). The receiver operating characteristic (ROC) analysis was performed for the four scoring systems...
August 2017: Immunology Letters
https://www.readbyqxmd.com/read/28515613/secondary-sjogren-s-syndrome-presenting-with-distal-tubular-acidosis-and-quadriparesis
#15
Kartik Munta, Manimala Rao Surath, K Seshikiran
A 52-year-old female patient was admitted to Intensive Care Unit with complaints of quadriparesis. Investigations revealed distal renal tubular acidosis (DRTA) secondary to Sjogren's syndrome with involvement of the parotid and thyroid glands. Laboratory investigations showed hyperchloremic metabolic acidosis and an alkaline urine pH with clinical signs of sicca syndrome. Sjogren's syndrome is associated with DRTA and occurrences of quadriparetic hypokalemia, nephrolithiasis, and osteomalacia can be prevented with early diagnosis and lifelong treatment with potassium and alkali replacement...
April 2017: Indian Journal of Critical Care Medicine
https://www.readbyqxmd.com/read/28507447/managing-refractory-cryoglobulinemic-vasculitis-challenges-and-solutions
#16
REVIEW
Predrag Ostojic, Ivan R Jeremic
Cryoglobulinemia is thought to be a rare condition. It may be an isolated disorder or secondary to a particular disease. According to immunoglobulin composition, cryoglobulinemia is classified into three types. In mixed cryoglobulinemia (types II and III), vascular deposition of cryoglobulin-containing immune complexes and complement may induce a clinical syndrome, characterized by systemic vasculitis and inflammation - cryoglobulinemic vasculitis (CryoVas). Most common clinical manifestations in CryoVas are skin lesions (orthostatic purpura and ulcers), weakness, peripheral neuropathy, Raynaud's phenomenon, sicca syndrome, membranoproliferative glomerulonephritis, and arthralgia and seldom arthritis...
2017: Journal of Inflammation Research
https://www.readbyqxmd.com/read/28499370/is-hydroxychloroquine-effective-in-treating-primary-sjogren-s-syndrome-a-systematic-review-and-meta-analysis
#17
Shi-Qin Wang, Li-Wei Zhang, Pan Wei, Hong Hua
BACKGROUND: To systematically review and assess the efficacy and safety of hydroxychloroquine (HCQ) for treating primary Sjogren's syndrome (pSS). METHODS: Five electronic databases (Pubmed, EMBASE, Web of science, Ovid, Cochrane Library) were searched for randomized controlled trials and retrospective or prospective studies published in English that reported the effect of HCQ on pSS. The subjective symptoms (sicca symptoms, fatigue and pain) and the objective indexes (erythrocyte sedimentation rate and Schirmer test) were assessed as main outcome measures...
May 12, 2017: BMC Musculoskeletal Disorders
https://www.readbyqxmd.com/read/28484851/-long-term-outcome-of-keratoprosthesis-with-biological-support
#18
K Hille
BACKGROUND: In severe Sicca syndrome and limbal stem cell deficiency, vision can be restored by an artificial optical implant into the cornea (keratoprosthesis, KPro). After multiple efforts to achieve long-lasting corneal fixation of such an optical implant, two methods have become established: KPro with biological support, such as osteo-odonto-keratoprosthesis (OOKP) made from the root of patient's own tooth or osteo-keratoprosthesis (OKP), made from cortical bone of the patient's tibia; and the Boston KPro...
May 8, 2017: Der Ophthalmologe: Zeitschrift der Deutschen Ophthalmologischen Gesellschaft
https://www.readbyqxmd.com/read/28466806/hcv-unrelated-cryoglobulinaemic-vasculitis-the-results-of-a-prospective-observational-study-by-the-italian-group-for-the-study-of-cryoglobulinaemias-gisc
#19
MULTICENTER STUDY
Massimo Galli, Letizia Oreni, Francesco Saccardo, Laura Castelnovo, Davide Filippini, Piero Marson, Maria Teresa Mascia, Cesare Mazzaro, Laura Origgi, Elena Ossi, Maurizio Pietrogrande, Piero Pioltelli, Luca Quartuccio, Salvatore Scarpato, Salvatore Sollima, Agostino Riva, Paolo Fraticelli, Roberta Zani, Dilia Giuggioli, Marco Sebastiani, Piercarlo Sarzi Puttini, Armando Gabrielli, Anna Linda Zignego, Patrizia Scaini, Clodoveo Ferri, Salvatore De Vita, Giuseppe Monti
OBJECTIVES: To investigate the clinical and laboratory patterns of HCV-unrelated cryoglobulinaemic vasculitis (CV), and the factors influencing its outcome. METHODS: Prospective study of all anti-HCV and HCV-RNA negative patients with CV who have been observed since January 2004 in 17 centres participating in the Italian Group for the Study of Cryoglobulinaemias (GISC). RESULTS: 175 enrolled were followed up for 677 person-years. The associated conditions were primary Sjögren's syndrome (21...
March 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28444575/erratum-to-prevalence-and-characterization-of-non-sicca-onset-primary-sj%C3%A3-gren-syndrome-with-interstitial-lung-involvement
#20
Andreina Manfredi, Marco Sebastiani, Stefania Cerri, Giulia Cassone, Pietrantonio Bellini, Giovanni Della Casa, Fabrizio Luppi, Clodoveo Ferri
No abstract text is available yet for this article.
April 25, 2017: Clinical Rheumatology
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