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https://www.readbyqxmd.com/read/28748510/clinical-characteristics-of-autoimmune-rheumatic-disease-related-organizing-pneumonia
#1
Xin Dong, Yi Zheng, Li Wang, Wen-Hui Chen, Yun-Gang Zhang, Qiang Fu
To study the clinical characteristics of autoimmune rheumatic disease-related organizing pneumonia (AIRD-OP), the clinical presentation, radiological findings, treatment, and outcome of AIRD-OP patients were analyzed, in comparison with patients with cryptogenic organizing pneumonia (COP). A total of 131 OP patients were identified, including 57 cases of AIRD-OP, 35 cases of COP, and 39 cases of other disease-related OPs. Among AIRD-OP patients, 36 (63%) presented the symptoms of OP at onset. The primary disease of AIRDs included Sjogren's syndrome (38%), polymyositis/dermatomyositis (23%), rheumatoid arthritis (23%), and undifferentiated AIRD...
July 26, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28748269/presence-of-bpifb1-in-saliva-from-non-obese-diabetic-mice
#2
Tomoko Nashida, Junko Shimomura-Kuroki, Fumi Mizuhashi, Maiko Haga-Tsujimura, Ken Yoshimura, Sachiko Hayashi-Sakai
We previously showed that mRNA expression of BPIFB1 (Bpifb1), an antibacterial protein in the palate, lung, and nasal epithelium clone protein family, was increased in parotid acinar cells in non-obese diabetic (NOD, NOD/ShiJcl) mice, which is an animal model for Sjögren's syndrome. However, we did not previously assess the protein levels. In this report, we confirmed the expression of BPIFB1 protein in the parotid glands of NOD mice. Immunoblotting of subcellular fractions revealed that BPIBB1 was localised in secretory granules in parotid glands from NOD mice, and was almost not in parotid glands from the control mice...
July 26, 2017: Odontology
https://www.readbyqxmd.com/read/28747448/movement-disorder-in-gnao1-encephalopathy-associated-with-gain-of-function-mutations
#3
Huijie Feng, Benita Sjögren, Behirda Karaj, Vincent Shaw, Aysegul Gezer, Richard R Neubig
OBJECTIVE: To define molecular mechanisms underlying the clinical spectrum of epilepsy and movement disorder in individuals with de novo mutations in the GNAO1 gene. METHODS: We identified all GNAO1 mutations reported in individuals with epilepsy (early infantile epileptiform encephalopathy 17) or movement disorders through April 2016; 15 de novo mutant alleles from 25 individuals were introduced into the Gαo subunit by site-directed mutagenesis in a mammalian expression plasmid...
July 26, 2017: Neurology
https://www.readbyqxmd.com/read/28747284/comorbid-autoimmune-diseases-in-patients-with-pemphigus-a-nationwide-case-control-study-in-taiwan
#4
Yun-Wen Chiu, Yen-Da Chen, Tuan-Chun Hua, Chi-Hung Wu, Han-Nan Liu, Yun-Ting Chang
Previous studies have proposed the association between pemphigus and several autoimmune diseases, but no large-scale study has been reported. To delineate the association between pemphigus and autoimmune diseases including psoriasis. A total of 1,998 patients with pemphigus and 7,992 control subjects were enrolled from the National Health Insurance Research Database in Taiwan from 1997 to 2010. The odds of comorbidities between these two groups were analysed by multivariate logistic regression. Compared with control subjects, patients with pemphigus were much more likely to have Sjögren's syndrome (odds ratio [OR]: 15...
July 27, 2017: European Journal of Dermatology: EJD
https://www.readbyqxmd.com/read/28744788/extended-high-frequency-audiometry-as-early-detection-of-hearing-loss-in-primary-sj%C3%A3-gren-syndrome
#5
José Luis Treviño González, Janett Riega Torres, Yolisa Hinojosa Ríos, Mario Jesús Villegas González, Marco A Mendez Saenz, German A Soto-Galindo
The aim of the study is to evaluate hearing function by extended high-frequency audiometry in primary Sjögren Syndrome (pSS) and to correlate it with disease activity. All patients diagnosed with pSS were recruited within 1 year. Patients underwent otologic evaluation, extended high-frequency audiometry, tympanogram, and the ESSPRI scale evaluation. A total of 63 (60 female and 3 male) patients with mean age 49.5 ± 9.9 years with pSS were included. We detected a hearing loss prevalence of 95.2% (60 patients) in both ears in very high frequencies (10000-16,000 Hz)...
July 26, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28743413/corneal-melting-after-cataract-surgery-in-a-patient-with-autoimmune-disease
#6
G García de Oteyza, A Gómez, M de la Paz
CASE REPORT: A 78-year-old woman with rheumatoid arthritis and secondary Sjögren's syndrome presented with corneal melting three days after cataract extraction that required penetrating keratoplasty. By the fourth month, a second corneal transplant was needed due to a new descemetocele associated with her systemic disease. DISCUSSION: The underlying disease, together with the surgical history, was responsible for the complication presented. The correct anamnesis prior to cataract surgery, a refined technique, and a close post-operative follow-up can avoid such a serious complication...
July 22, 2017: Archivos de la Sociedad Española de Oftalmología
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
#7
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/28742141/bruton-s-tyrosine-kinase-inhibitor-bms-986142-in-experimental-models-of-rheumatoid-arthritis-enhances-efficacy-of-agents-representing-clinical-standard-of-care
#8
Kathleen M Gillooly, Claudine Pulicicchio, Mark A Pattoli, Lihong Cheng, Stacey Skala, Elizabeth M Heimrich, Kim W McIntyre, Tracy L Taylor, Daniel W Kukral, Shailesh Dudhgaonkar, Jignesh Nagar, Dana Banas, Scott H Watterson, Joseph A Tino, Aberra Fura, James R Burke
Bruton's tyrosine kinase (BTK) regulates critical signal transduction pathways involved in the pathobiology of rheumatoid arthritis (RA) and other autoimmune disorders. BMS-986142 is a potent and highly selective reversible small molecule inhibitor of BTK currently being investigated in clinical trials for the treatment of both RA and primary Sjögren's syndrome. In the present report, we detail the in vitro and in vivo pharmacology of BMS-986142 and show this agent provides potent and selective inhibition of BTK (IC50 = 0...
2017: PloS One
https://www.readbyqxmd.com/read/28740437/the-larynx-ruler-to-measure-height-and-profile-of-vocal-folds-a-proof-of-concept
#9
Gauthier Desuter, Benjamin Mertens, Alain Delchambre, Julie van Lith-Bijl, Peter Paul van Benthem, Elisabeth Sjögren
INTRODUCTION: Glottic leakage during phonation is a direct consequence of unilateral vocal fold (VF) paralysis. This air leakage can be in the horizontal plane and in the vertical plane. Presently, there is no easily applicable medical device allowing noninvasive, office-based measurement of the relative vertical position of the VFs. The larynx ruler (LR) is a laser-based measuring device that could meet the previously stated need, using a flexible endoscope. This study represents a proof of concept regarding the use of the LR in assessing VF relative positions in the vertical plane...
2017: Medical Devices: Evidence and Research
https://www.readbyqxmd.com/read/28737406/preclinical-effect-of-absorption-modifying-excipients-on-rat-intestinal-transport-of-five-model-compounds-and-the-intestinal-barrier-marker-51-cr-edta
#10
David Dahlgren, Carl Roos, Anders Lundqvist, Peter Langguth, Christer Tannergren, Markus Sjöblom, E Sjögren, H Lennernas
There is a renewed interest from the pharmaceutical field to develop oral formulations of compounds such as peptides, oligonucleotides, and polar drugs. However, these often suffer from insufficient absorption across the intestinal mucosal barrier. One approach to circumvent this problem is the use of absorption modifying excipient(s) (AME). This study determined the absorption enhancing effect of four AMEs (sodium dodecyl sulfate, caprate, chitosan, N-acetylcysteine) on five model compounds in a rat jejunal perfusion model...
July 24, 2017: Molecular Pharmaceutics
https://www.readbyqxmd.com/read/28737398/in-vivo-mechanisms-of-intestinal-drug-absorption-from-aprepitant-nanoformulations
#11
Carl Roos, David Dahlgren, Staffan Berg, Jan Westergren, Bertil Abrahamsson, Christer Tannergren, E Sjögren, H Lennernas
Over recent decades there has been an increase in the proportion of BCS class II and IV drug candidates in industrial drug development. To overcome the biopharmaceutical challenges associated with the less favorable properties of solubility and/or intestinal permeation of these substances, the development of formulations containing nanosuspensions of the drugs has been suggested. The intestinal absorption of aprepitant from two nanosuspensions (20 µM and 200 µM total concentrations) in phosphate buffer, one nanosuspension (200 µM) in fasted-state simulated intestinal fluid (FaSSIF), and one solution (20 µM) in FaSSIF was investigated in the rat single-pass intestinal perfusion model...
July 24, 2017: Molecular Pharmaceutics
https://www.readbyqxmd.com/read/28735431/a-systematic-review-of-primary-sj%C3%A3-gren-s-syndrome-in-male-and-paediatric-populations
#12
Simrun Virdee, James Greenan-Barrett, Coziana Ciurtin
Primary Sjögren's syndrome (pSS) is a chronic multisystem autoimmune rheumatic disease characterised by female predominance. Although the disease is rare in the male and paediatric populations, it has been suggested that it may have a different disease phenotype, which has not been investigated before using a systematic approach. A systematic literature search of PubMed databases (updated to December 2016) was performed to identify all published data on the epidemiological, clinical and laboratory manifestations of pSS in the male and paediatric populations...
July 22, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28730224/histopathological-and-immunohistochemical-profile-in-primary-sj%C3%A3-gren-s-syndrome
#13
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/28729155/incidence-mortality-and-causes-of-death-in-physician-diagnosed-primary-sj%C3%A3-gren-s-syndrome-in-korea-a-nationwide-population-based-study
#14
Hyun Jung Kim, Kyoung Hoon Kim, Hoo Jae Hann, Seungjin Han, Yuri Kim, Sang Hyuk Lee, Dong Sook Kim, Hyeong Sik Ahn
OBJECTIVES: The objective of this study was to investigate the epidemiological features of primary Sjögren's syndrome (pSS) in Korea at a national level, including the incidence, mortality, and causes of death. METHODS: We used a national, population-based registry database called the Rare Intractable Disease Registration Program from the Health Insurance Review and Assessment Service to obtain pSS patient data for the period between 2010 and 2014. pSS was diagnosed by a physician based on uniform criteria...
March 8, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/28728856/achaete-scute-complex-homologue-2-accelerates-the-development-of-sj%C3%A3-gren-s-syndrome-like-disease-in-the-nod-shiltj-mouse
#15
Sung-Min Kim, Jeong-Eun Kwon, Jin-Sil Park, Hyeon-Beom Seo, Kyung-Ah Jung, Young-Mee Moon, Jennifer Lee, Seung-Ki Kwok, Mi-La Cho, Sung-Hwan Park
Achaete-scute complex homologue 2 (Ascl2) has been reported to induce the differentiation and activation of follicular helper T (TFH) cells, which are essential for development of Sjögren's syndrome (SS). This study examined whether Ascl2 plays a role in the development of SS. NOD/ShiLtJ mice were injected with an Ascl2-overexpression vector, and the infiltration of lymphocytes into salivary and lacrimal glands was assessed. The expression of inflammatory cytokines and chemoattractants for T or B cells was measured...
July 17, 2017: Immunology Letters
https://www.readbyqxmd.com/read/28727987/associations-of-autoimmunity-immunodeficiency-lymphomagenesis-and-gut-microbiota-in-mice-with-knockins-for-a-pathogenic-autoantibody
#16
Shweta Jain, Jerrold M Ward, Dong-Mi Shin, Hongsheng Wang, Zohreh Naghashfar, Alexander L Kovalchuk, Herbert C Morse
A number of mouse strains transgenic for B-cell receptors specific for nucleic acids or other autoantigens have been generated to understand how autoreactive B cells are regulated in normal and autoimmune mice. Previous studies of nonautoimmune C57BL/6 mice heterozygous for both the IgH and IgL knockins of the polyreactive autoantibody, 564, produced high levels of autoantibodies in a largely Toll-like receptor 7-dependent manner. Herein, we describe studies of mice homozygous for the knockins that also expressed high levels of autoantibodies but, unlike the heterozygotes, exhibited a high incidence of mature B-cell lymphomas and enhanced susceptibility to bacterial infections...
July 17, 2017: American Journal of Pathology
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
#17
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/28722699/added-value-of-parotid-r2-values-for-evaluation-of-sj%C3%A3-gren-syndrome-a-preliminary-study
#18
Chen Chu, Nan Zhou, Huayong Zhang, Xin Dou, Ming Li, Song Liu, Jie Wang, Weibo Chen, Queenie Chan, Lingyun Sun, Jian He, Zhengyang Zhou
OBJECTIVE: To explore the application of parotid R2* values for evaluating Sjögren syndrome (SS). METHODS: Twenty-four consecutive SS patients and 24 sex-matched and age-matched healthy volunteers underwent bilateral parotid 3.0 T magnetic resonance (MR) imaging, including blood oxygenation level dependent sequence. Parotid R2* values of SS patients and volunteers were compared. A receiver operating characteristic analysis was used to evaluate the diagnostic performance of parotid R2* value alone and in combination with MR nodular grade...
July 2017: Journal of Computer Assisted Tomography
https://www.readbyqxmd.com/read/28722669/hydroxychloroquine-and-risk-of-cancer-in-patients-with-primary-sj%C3%A3-gren-syndrome-propensity-score-matched-landmark-analysis
#19
Yao-Fan Fang, Yen-Fu Chen, Ting-Ting Chung, Lai-Chu See, Kuang-Hui Yu, Shue-Fen Luo, Chang-Fu Kuo, Jenn-Haung Lai
Hydroxychloroquine inhibits systemic inflammation and autophagy and may thus have antineoplastic effects [1]. We investigated the effect of hydroxychloroquine on cancer risk in patients with primary Sjögren syndrome(pSS). We used the Taiwan National Health Insurance Database to compare cancer incidence between incident pSS patients with or without at least 6-month hydroxychloroquine use within a 1- or 3-year period. Propensity score matched landmark analysis was used. We included 4194 alive patients without cancer 1 year after pSS diagnosis from 2000 through 2005...
July 6, 2017: Oncotarget
https://www.readbyqxmd.com/read/28722506/diffusion-weighted-magnetic-resonance-imaging-of-parotid-glands-before-and-after-abatacept-therapy-in-patients-with-sj%C3%A3-gren-s-syndrome-associated-with-rheumatoid-arthritis-utility-to-evaluate-and-predict-response-to-treatment
#20
Hiroyuki Takahashi, Hiroto Tsuboi, Masahiro Yokosawa, Hiromitsu Asashima, Tomoya Hirota, Yuya Kondo, Isao Matsumoto, Takayuki Sumida
OBJECTIVE: To compare parotid diffusion-weighted images (DWIs) taken before and after abatacept therapy in patients with Sjögren's syndrome (SS) associated with rheumatoid arthritis (RA) and to examine the utility in evaluation and prediction of response to therapy. METHODS: DWIs of the parotid glands taken at baseline and 52 weeks after initiation of abatacept were analyzed in nine SS patients with RA using relative standard deviation (RSD) of the entire glands and signal intensity ratio (SIR) within the residual parenchyma...
July 19, 2017: Modern Rheumatology
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