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Sicca Syndrome

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https://www.readbyqxmd.com/read/28324200/prevalence-and-characterization-of-non-sicca-onset-primary-sj%C3%A3-gren-syndrome-with-interstitial-lung-involvement
#1
Manfredi Andreina, Sebastiani Marco, Cerri Stefania, Cassone Giulia, Bellini Pietrantonio, Della Casa Giovanni, Luppi Fabrizio, Ferri Clodoveo
Primary Sjögren syndrome (pSS)-related interstitial lung disease (ILD) involved about 10-20% of patients. In 20% of cases, ILD can be diagnosed before pSS; anyway, few studies have investigated the frequency of ILD as the first clinically relevant manifestation of pSS, generally referred to retrospective studies. Aim of our prospective study was to describe prevalence, clinical, serological, and instrumental features of non-sicca onset pSS patients with interstitial lung involvement. During a period of 48 months, all consecutive patients diagnosed as pSS were enrolled...
March 21, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28293537/a-rare-case-of-concomitant-sicca-keratopathy-and-ipsilateral-central-facial-palsy-in-wallenberg-s-dorsolateral-medullary-syndrome
#2
Deborah De Bruyn, Elisabeth Van Aken, Kristien Herman
Objective: To describe a patient with a right-sided supranuclear facial palsy and concomitant sicca keratopathy of the right eye following right-sided dorsolateral medullary infarction. Methods: Our patient underwent a complete ophthalmologic and neurologic examination including biomicroscopy, fundus examination, cranial nerve examination, Shirmer I test, and magnetic resonance imaging of the brain. Results: A 61-year-old woman presented in emergency with a central facial nerve palsy on the right side and truncal ataxia...
2017: GMS Ophthalmology Cases
https://www.readbyqxmd.com/read/28284773/extranodal-marginal-zone-b-cell-lymphoma-an-unexpected-complication-in-children-with-sj%C3%A3-gren-s-syndrome
#3
Paz Collado, Aml Kelada, Maria Cámara, Andrew Zeft, Aron Flagg
Sjögren's syndrome (SS) is a systemic autoimmune disease characterized by the infiltration of lymphocytes into exocrine glands, resulting in the typical sicca symptoms. Unlike adults, primary SS is a very rare condition in childhood, and the risk of malignancy in juvenile SS (JSS) has not been defined. We report the detection of extranodal marginal zone B-cell lymphoma (EMZL) occurring in two children with SS. Fine needle aspiration of the salivary glands (SG) showed nonspecific findings that led to delayed diagnosis of SS...
March 8, 2017: Reumatología Clinica
https://www.readbyqxmd.com/read/28283891/the-potential-role-for-early-biomarker-testing-as-part-of-a-modern-multidisciplinary-approach-to-sj%C3%A3-gren-s-syndrome-diagnosis
#4
REVIEW
Kenneth A Beckman, Jodi Luchs, Mark S Milner, Julian L Ambrus
Sjögren's syndrome (SS) is a chronic and progressive multisystem autoimmune disease typically managed by rheumatologists. Diagnostic delays are common, due in large part to the non-specific and variable nature of SS symptoms and the slow progression of disease. The hallmark characteristics of SS are dry eye and dry mouth, but there are a broad range of other possible symptoms such as joint and muscle pain, skin rashes, chronic dry cough, vaginal dryness, extremity numbness or tingling, and disabling fatigue...
March 10, 2017: Advances in Therapy
https://www.readbyqxmd.com/read/28282148/alterations-in-the-salivary-proteome-and-n-glycome-of-sj%C3%A3-gren-s-syndrome-patients
#5
Steven C Hall, Maria E Hassis, Katherine E Williams, Matthew E Albertolle, Akraporn Prakobphol, Andrew B Dykstra, Megan Laurance, Katherine Ona, Richard K Niles, Namrata Prasad, Matthew Gormley, Caroline H Shiboski, Lindsey A Criswell, H Ewa Witkowska, Susan J Fisher
We used isobaric mass tagging (iTRAQ) and lectin affinity capture mass spectrometry (MS)-based workflows for global analyses of parotid saliva (PS) and whole saliva (WS) samples obtained from patients diagnosed with primary Sjögren's Syndrome (pSS) who were enrolled in the Sjögren's International Collaborative Clinical Alliance (SICCA) as compared to two control groups. The iTRAQ analyses revealed up- and down-regulation of numerous proteins that could be involved in the disease process (e.g., histones) or attempts to mitigate the ensuing damage [e...
March 10, 2017: Journal of Proteome Research
https://www.readbyqxmd.com/read/28275496/thymic-hyperplasia-associated-with-primary-sjogren-s-syndrome-cured-by-thymectomy
#6
Yanzhong Xin, Hongfei Cai, Yang Li, Youbin Cui
Thymus hyperplasia associated with Sjogren's syndrome is a rare morbid state. The present study described a 55-year-old woman who presented with a dryness of the oral cavity, and itchy eyes. Chest computed tomography identified a mass, measuring 4×2.5×2.5 cm, located at the anterior mediastinum. The mass was suspected as thymoma, thymic cyst, or teratoma, and resected by thymectomy. The postoperative pathological diagnosis was thymic lymphoid hyperplasia. After 1-year follow-up period, her sicca syndrome has been resolved...
February 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28272192/optic-neuropathy-associated-with-primary-sj%C3%A3-gren-s-syndrome-a-case-series
#7
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/28217953/b7-h4-deficiency-in-salivary-gland-of-patients-with-primary-sj%C3%A3-gren-s-syndrome-impairs-the-regulatory-effect-on-t-cells
#8
Xiaomei Li, Daliang Yu, Ning Yu, Ximei Wang, Xiangpei Li, David C H Harris, Yiping Wang
AIM: Our previous study confirmed the defect of B7-H4 expression in peripheral blood and salivary glands of patients with primary Sjögren's syndrome (pSS). The aim of this study was to analyze the effect of the deficit expression of B7-H4 on CD4(+) T cells. METHODS: CD4(+) T cells were purified by magnetic-activated cell sorting MACS. The proliferation and cytokine production of CD4(+) T cells co-cultured with purified salivary gland epithelial cells (SGECs) from pSS or non-SS sicca syndrome were detected...
February 20, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28207432/spontaneous-canalicular-plug-erosion-after-punctal-plug-placement
#9
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/28202706/clinical-and-genetic-factors-predicting-dravet-syndrome-in-infants-with-scn1a-mutations
#10
Valentina Cetica, Sara Chiari, Davide Mei, Elena Parrini, Laura Grisotto, Carla Marini, Daniela Pucatti, Annarita Ferrari, Federico Sicca, Nicola Specchio, Marina Trivisano, Domenica Battaglia, Ilaria Contaldo, Nelia Zamponi, Cristina Petrelli, Tiziana Granata, Francesca Ragona, Giuliano Avanzini, Renzo Guerrini
OBJECTIVE: To explore the prognostic value of initial clinical and mutational findings in infants with SCN1A mutations. METHODS: Combining sex, age/fever at first seizure, family history of epilepsy, EEG, and mutation type, we analyzed the accuracy of significant associations in predicting Dravet syndrome vs milder outcomes in 182 mutation carriers ascertained after seizure onset. To assess the diagnostic accuracy of all parameters, we calculated sensitivity, specificity, receiver operating characteristic (ROC) curves, diagnostic odds ratios, and positive and negative predictive values and the accuracy of combined information...
March 14, 2017: Neurology
https://www.readbyqxmd.com/read/28198156/prevalence-of-secondary-sj%C3%A3-gren-s-syndrome-in-indian-patients-with-rheumatoid-arthritis-a-single-center-study
#11
Krishna Santosh, Varun Dhir, Surjit Singh, Ashwani Sood, Amit Gupta, Aman Sharma, Shefali Sharma
AIM: To assess the prevalence of secondary Sjögren's syndrome (sSS) and its association with joint damage in patients with rheumatoid arthritis (RA) from northern India. METHODS: Patients included had RA, fulfilling the 1987 American College of Rheumatology criteria, with disease duration of more than 1 year. They were administered a sicca questionnaire that included six questions from subjective criteria of American European Consensus Group (AECG) criteria. Those who answered affirmatively to at least one question underwent Schirmer I test (ocular function) and salivary scintigraphy (salivary gland involvement)...
February 15, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28182102/a-case-report-of-nonvasculitic-autoimmune-inflammatory-meningoencephalitis-with-sensory-ganglionopathy-a-rare-presentation-of-sj%C3%A3-gren-syndrome
#12
João Peres, Simão Cruz, Rita Oliveira, Luís Santos, Ana Valverde
A 68-year-old Caucasian female was admitted to the emergency department with a progressive history of behavioural symptoms and anxiety followed by visual and auditory hallucinations, forgetfulness, and impaired gait in the previous 3 months. On examination she was psychotic and had a postural and rest tremor of the upper limbs, cogwheel rigidity of the four limbs, retropulsion on standing position, and inability to walk. During the following 2 weeks she developed xerostomia and unilateral parotiditis that improved with steroids...
2017: Case Reports in Neurological Medicine
https://www.readbyqxmd.com/read/28166540/effect-of-tobacco-smoking-on-the-clinical-histopathological-and-serological-manifestations-of-sj%C3%A3-gren-s-syndrome
#13
Donald U Stone, Dustin Fife, Michael Brown, Keith E Earley, Lida Radfar, C Erick Kaufman, David M Lewis, Nelson L Rhodus, Barbara M Segal, Daniel J Wallace, Michael H Weisman, Swamy Venuturupalli, Michael T Brennan, Christopher J Lessard, Courtney G Montgomery, R Hal Scofield, Kathy L Sivils, Astrid Rasmussen
OBJECTIVES: To assess the association of smoking habits with the clinical, serological, and histopathological manifestations of Sjögren's syndrome (SS) and non-Sjögren's sicca (non-SS sicca). METHODS: Cross-sectional case-control study of 1288 patients with sicca symptoms (587 SS and 701 non-SS sicca) evaluated in a multi-disciplinary research clinic. Smoking patterns were obtained from questionnaire data and disease-related clinical and laboratory data were compared between current, past, ever, and never smokers...
2017: PloS One
https://www.readbyqxmd.com/read/28149646/vasculitic-syndromes-in-hepatitis-c-virus-a-review
#14
REVIEW
Gaafar Ragab, Mohamed A Hussein
Vasculitis is a remarkable presentation of the extrahepatic manifestations of HCV. According to the presence or absence of cryoglobulins it is subdivided into two main types: cryoglobulinemic vasculitis and non cryoglobulinemic vasculitis based on the attribution of vasculitis to serum cryoglobulins as a pathogenic factor. The attribution of cryoglobulinemia to HCV represents a success story in the history of immunology, microbiology, and clinical medicine. HCV can bind to and invade lymphocytes, consequently triggering an immune response through different mechanisms...
March 2017: Journal of Advanced Research
https://www.readbyqxmd.com/read/28149645/new-insights-into-hcv-related-rheumatologic-disorders-a-review
#15
REVIEW
Patrice Cacoub, Cloé Comarmond
Hepatitis C virus (HCV) infected patients are known to be exposed to major liver complications i.e. cirrhosis and hepatocellular carcinoma. In addition, many extrahepatic manifestations including rheumatologic disorders have been reported in up to two-third of HCV infected patients. These manifestations include frank auto-immune and rheumatic diseases (such as arthralgia, myalgia, arthritis, sicca syndrome and vasculitis) which may dominate the course of infection. Until recently, the standard of care of HCV has been the use of interferon-alpha based regimens, which not only had limited effectiveness in HCV cure but were poorly tolerated...
March 2017: Journal of Advanced Research
https://www.readbyqxmd.com/read/28098109/visceral-leishmaniasis-in-adults-with-nephropathy
#16
H Kaaroud El Jeri, A Harzallah, S Barbouch, M M Bacha, R Kheder, S Turki, S Trabelsi, E Abderrahim, F Ben Hamida, T Ben Abdallah
The aim of this study is to evaluate the features of visceral leishmaniasis (VL) in adults with nephropathy, who were not infected with the human immunodeficiency virus. This is a retrospective study of 14 adults hospitalized between 2000 and 2014, with VL and renal involvement. Clinical, biological, and therapeutic data were collected from the patients' medical files. Eleven women and three men, most of whom were from the North of the country, with a mean age of 40.5 years were studied. Lupus was present in five cases, the Sicca syndrome in three cases, diabetes in one case, renal failure on dialysis in two cases, and there were three renal transplant recipients...
January 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28076899/genome-wide-association-analysis-reveals-genetic-heterogeneity-of-sj%C3%A3-gren-s-syndrome-according-to-ancestry
#17
Kimberly E Taylor, Quenna Wong, David M Levine, Caitlin McHugh, Cathy Laurie, Kimberly Doheny, Mi Y Lam, Alan N Baer, Stephen Challacombe, Hector Lanfranchi, Morten Schiødt, M Srinivasan, Hisanori Umehara, Frederick B Vivino, Yan Zhao, Stephen Shiboski, Troy E Daniels, John S Greenspan, Caroline H Shiboski, Lindsey A Criswell
OBJECTIVE: Sjögren's Syndrome (SS) is a systemic autoimmune disease affecting primarily the lacrimal and salivary glands. The Sjögren's International Collaborative Clinical Alliance (SICCA) is an international multisite observational study whose participants have been genotyped on the Omni 2.5M platform and undergone deep phenotyping using common protocol-directed methods, providing a unique opportunity to examine the genetic etiology of SS across ancestry and disease subsets. METHODS: We perform GWAS analyses utilizing dbGaP controls on all subjects (1405 cases, 1622 SICCA controls, 3125 external controls), European (similarly 585, 966, 2580), and Asian (similarly 460, 224, 901) with ancestry adjustments via principal component analyses...
January 11, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28050735/semi-quantitative-analysis-of-salivary-gland-scintigraphy-in-sj%C3%A3-gren-s-syndrome-diagnosis-a-first-line-tool
#18
Tiziana Angusti, Emanuela Pilati, Antonella Parente, Renato Carignola, Matteo Manfredi, Simona Cauda, Elena Pizzigati, Julien Dubreuil, Francesco Giammarile, Valerio Podio, Andrea Skanjeti
OBJECTIVE: The aim of this study was the assessment of semi-quantified salivary gland dynamic scintigraphy (SGdS) parameters independently and in an integrated way in order to predict primary Sjögren's syndrome (pSS). MATERIALS AND METHODS: Forty-six consecutive patients (41 females; age 61 ± 11 years) with sicca syndrome were studied by SGdS after injection of 200 MBq of pertechnetate. In sixteen patients, pSS was diagnosed, according to American-European Consensus Group criteria (AECGc)...
January 3, 2017: Clinical Oral Investigations
https://www.readbyqxmd.com/read/28049649/hereditary-lysozyme-amyloidosis-variant-p-leu102ser-associates-with-unique-phenotype
#19
Samih H Nasr, Surendra Dasari, John R Mills, Jason D Theis, Michael T Zimmermann, Rafael Fonseca, Julie A Vrana, Steven J Lester, Brooke M McLaughlin, Robert Gillespie, W Edward Highsmith, John J Lee, Angela Dispenzieri, Paul J Kurtin
Lysozyme amyloidosis (ALys) is a rare form of hereditary amyloidosis that typically manifests with renal impairment, gastrointestinal (GI) symptoms, and sicca syndrome, whereas cardiac involvement is exceedingly rare and neuropathy has not been reported. Here, we describe a 40-year-old man with renal impairment, cardiac and GI symptoms, and peripheral neuropathy. Renal biopsy specimen analysis revealed amyloidosis with extensive involvement of glomeruli, vessels, and medulla. Amyloid was also detected in the GI tract...
February 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/28024826/predictors-of-discordance-between-symptoms-and-signs-in-dry-eye-disease
#20
Jelle Vehof, Nicole Sillevis Smitt-Kamminga, Simone A Nibourg, Christopher J Hammond
PURPOSE: To investigate predictors of discordance between symptoms and signs in dry eye disease (DED). DESIGN: Cross-sectional association study. PARTICIPANTS: A total of 648 patients with dry eye from the Groningen LOngitudinal Sicca StudY (GLOSSY), a tertiary dry eye clinic patient cohort from the Netherlands. METHODS: Patient symptoms were assessed using the Ocular Surface Disease Index (OSDI) questionnaire. Dry eye signs were assessed by tear osmolarity, Schirmer test, tear breakup time, corneal and conjunctival staining, and meibomian gland dysfunction, all in both eyes, and a composite dry eye signs severity score was calculated from these 6 tests for each patient...
March 2017: Ophthalmology
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