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sjogrens disease

Siddharth Jain, Animesh Ray, Manish Soneja, S Leve Joseph Devarajan, Shyam Madabhushi, Anitha Swamy, T Naveen, S K Sharma
A known case of primary Sjogren's syndrome with chronic kidney disease presented with respiratory symptoms and subsequent altered sensorium. Chest imaging suggested cavitating lung lesions in both the lungs. Serum c-ANCA was positive without any upper respiratory tract involvement or active urinary sediments. Treatment with appropriate anti-microbials produced no improvement in the respiratory or neurological parameters. MRI brain revealed tell-tale signs of CNS vascultis. A diagnosis of GPA was made as per European Medical Agency algorithm and the patient was started on immune-suppressants to which there was dramatic response...
October 2016: Journal of the Association of Physicians of India
Young Joon Kwon, Marni J Falk, Michael J Bennett
CLN3 disease (Spielmeyer-Vogt-Sjogren-Batten disease, previously known as classic juvenile neuronal ceroid lipofuscinosis, NCL) is a pediatric-onset progressive neurodegenerative disease characterized by progressive vision loss, seizures, loss of cognitive and motor function, and early death. While no precise biochemical mechanism or therapies are known, the pathogenesis of CLN3 disease involves intracellular calcium accumulation that may trigger apoptosis. Our prior work in in vitro cell models of CLN3 deficiency suggested that FDA-approved calcium channel antagonists may have therapeutic value...
October 20, 2016: Journal of Inherited Metabolic Disease
Geraint P Williams, Peter Nightingale, Sue Southworth, Alastair K O Denniston, Paul J Tomlins, Stephen Turner, John Hamburger, Simon J Bowman, S John Curnow, Saaeha Rauz
Purpose: Ocular mucous membrane pemphigoid (OcMMP) is a rare autoimmune disorder resulting in progressive conjunctival fibrosis and ocular surface failure leading to sight loss in up to 50%. This study was designed to optimize an ocular surface sampling technique for identification of novel biomarkers associated with disease activity and/or progressive fibrosis. Methods: Fifty-seven patients with OcMMP underwent detailed examination of conjunctival inflammation and fibrosis using fornix depth measurement...
October 1, 2016: Investigative Ophthalmology & Visual Science
Arsenio Spinillo, Fausta Beneventi, Elena Locatelli, Vèronique Ramoni, Roberto Caporali, Claudia Alpini, Giulia Albonico, Chiara Cavagnoli, Carlomaurizio Montecucco
BACKGROUND: The burden of pregnancy complications associated with well defined, already established systemic rheumatic diseases preexisting pregnancy such as rheumatoid arthritis, systemic lupus erythematosus or scleroderma is well known. Systemic rheumatic diseases are characterized by a long natural history with few symptoms, an undifferentiated picture or a remitting course making difficult a timely diagnosis. It has been suggested that screening measures for these diseases could be useful but the impact of unrecognized systemic rheumatic disorders on pregnancy outcome is unknown...
October 18, 2016: BMC Pregnancy and Childbirth
Marcella Nebbioso, Valeria Fameli, Magda Gharbiya, Marta Sacchetti, Anna Maria Zicari, Alessandro Lambiase
The dry eye disease (DED) is a chronic multifactorial disorder of the tears that also involves the ocular surface, the lacrimal glands, and meibomian dysfunction. Furthermore, DED is often associated with Sjögren's syndrome (SS) and other autoimmune disorders. Sometimes, this chronic or subclinical condition is difficult to diagnose and treat, due to its heterogeneity. Areas covered: A literature search of relevant publications about treatment of DED was performed. All relevant articles published between 2011 and 2016 were identified through a computerized search for review and clinical trials using Pub Med database...
October 18, 2016: Expert Opinion on Investigational Drugs
Marcin Wirtwein, Olle Melander, Marketa Sjogren, Michal Hoffmann, Marcin Gruchala, Wojciech Sobiczewski
OBJECTIVE: The aim of this study is to report the relationship between certain single nucleotide polymorphisms (SNP) and blunted nighttime blood pressure (BP) fall in hypertensive patients with CAD confirmed by coronary angiography. DESIGN AND METHOD: According to the percentage decrease in mean systolic (SBP) and diastolic BP (DBP) during the nighttime period subjects were classified as dippers or non-dippers (nighttime relative SBP or DBP decline ≥ and <10% respectively)...
September 2016: Journal of Hypertension
Nicole C Droz, Stephanie D Mathew
After presenting with nonspecific symptoms, a 52-year-old female was found to have a 3.4 × 2.6 cm retroperitoneal mass resulting in hydronephrosis on abdominal computed tomography scan. After extensive workup including surgical debulking, excisional biopsy, and autoimmune panel, she was found to have idiopathic retroperitoneal fibrosis (IRF) with negative staining for immunoglogulin G4 (IgG4). Two years after presentation, the patient developed dyspnea, cough, and xerostomia. Repeat anti-Sjögren Syndrome A and anti-Sjögren Syndrome B titers and labial gland biopsy confirmed the diagnosis of concomitant Sjögren's syndrome (SS)...
October 2016: Military Medicine
Kang Won Lee, Jung Yong Kim, Hee Seung Chin, Kyoung Yul Seo, Tae-Im Kim, Ji Won Jung
PURPOSE: We aimed to compare the tear meniscus measurements using strip meniscometry (SM) and Keratograph5M (K5M) between 3 subtypes of dry eye disease patient groups, classified according to the presence of meibomian gland dysfunction (MGD), and normal subjects. METHODS: We enrolled 145 eyes from 145 subjects and measured the tear meniscus using SM and K5M, tear film breakup time, ocular surface staining, and ocular surface disease index; the Schirmer test; and examined lid margins and meibomian glands...
October 5, 2016: Cornea
Julie A Birt, YingMeei Tan, Neelufar Mozaffarian
OBJECTIVES: To better understand the real-world characteristics and costs of Sjögren's syndrome (SS). METHODS: Analysing the MarketScan Commercial Claims database from Jan. 1, 2006 to Dec. 31, 2011, we identified 10,414 patients ≥18 years old newly diagnosed with SS. Patient characteristics, drugs (commonly used for SS), resource utilisation, and medical costs were evaluated for 12 months pre- and post-diagnosis. RESULTS: Mean age was 55 years; 90% were female...
October 7, 2016: Clinical and Experimental Rheumatology
Gabriela M Balarini, Eliana Zandonade, Leandro Tanure, Gilda A Ferreira, Wildner M Sardenberg, Érica V Serrano, Cleia C Dias, Túlio P Navarro, Hilde H Nordal, Piotr M Mydel, Johan G Brun, Karl A Brokstad, Eva Gerdts, Roland Jonsson, Valéria Valim
OBJECTIVES: We aimed to identify the association of carotid atherosclerosis with the traditional risk factors, disease features, cytokine profile, and calprotectin in patients with primary Sjögren's syndrome (pSS). METHODS: 63 primary pSS patients and 63 age- and sex-matched healthy controls underwent carotid ultrasound, clinical and laboratory examination. The presence of carotid plaques was taken as carotid atherosclerosis. The covariates of carotid atherosclerosis were identified in univariate and multivariate regressions...
September 8, 2016: Clinical and Experimental Rheumatology
Julian Lawrence Ambrus, Lakshmanan Suresh, Ammon Peck
Sjogren's syndrome (SS) is a complex heterogeneous autoimmune disease resulting in loss of salivary gland and lacrimal gland function that may include multiple systemic manifestations including lymphoma. Multiple cell types participate in disease pathogenesis. This review discusses evidence for abnormal B cell subpopulations in patients with SS, critical roles of B cells in SS and the status of B cell-directed therapies in the management of patients with SS.
October 8, 2016: Journal of Clinical Medicine
Melis Palamar, Nazan Ozsan, Fahri Sahin
A 31-year-old female with Primary Sjögren Syndrome (pSS) presented with bilateral puffiness around the eye for 3 years. The lacrimal glands were hypertrophic and edematous bilaterally. Schirmer 1 score was 2 and 1 mm and tear-film break-up time was 3 and 4 seconds, in the right and the left eyes, respectively. An incisional biopsy from the left lacrimal gland revealed diffuse and intense CD20, CD5, and bcl-2 positivity with negative cyclin D1 and CD23 which supported lymphoma. Upon haematology consultation extranodal marginal zone lymphoma diagnosis was made...
2016: Case Reports in Ophthalmological Medicine
Mehmet Taşdemir, Chiar Hasan, Ayşe Ağbaş, Özgür Kasapçopur, Nur Canpolat, Lale Sever, Salim Çalışkan
Systemic lupus erythematosus and Sjögren's syndrome are chronic auto- inflammatory disorders which can lead to serious organ damage. Although systemic lupus erythematosus and Sjögren's syndrome were previously considered two forms of the same disease because of presence of clinical coexistence of these two conditions, the view that they are two different conditions with mutual characteristics has become prominent in recent years. In this paper, we reported a 16 year-old girl who was followed up with a diagnosis of Sjögren's syndrome for six years and then was observed to have overlap of systemic lupus erythematosus...
September 2016: Türk Pediatri Arşivi
Chang-Fu Kuo, Shue-Fen Luo, Kuang-Hui Yu, Lai-Chu See, Weiya Zhang, Michael Doherty
BACKGROUND: Systemic sclerosis (SSc) is a rare and devastating disease affecting skin and internal organs. Familial aggregation of SSc and co-aggregation with other autoimmune diseases is rarely reported. METHODS: We identified 23,658,577 beneficiaries registered with the National Health Insurance database in 2010, 1891 of whom had SSc. We identified 21,009,551 parent-child relationships and 17,168,340 full sibling pairs. The familial risks of SSc and other autoimmune diseases and familial transmission were estimated...
October 12, 2016: Arthritis Research & Therapy
Petr Husa
Extrahepatic manifestations of hepatitis C virus infection (HCV) are very common. The most common of these is mixed cryoglobulinaemia. Anti-HCV antibodies and viral ribonucleic acid, HCV RNA, can be found in the cryoprecipitates, together with the rheumatoid factor. Cryoglobulins consist of a complex of immunoglobulins that in vitro precipitate upon the cooling bellow the human body temperature. Vasculitis is caused by the deposition of such immune complexes in the small blood vessels. A link with the HCV infection is considered to be established with membranoproliferative glomerulonephritis, leukocytoclastic vasculitis, lymphoproliferative disorders (in particular B cell lymphoma), Sjögren and sicca syndrome, lichen planus, porfyria cutanea tarda and diabetes mellitus...
2016: Vnitr̆ní Lékar̆ství
Jitesh Ahuja, Deepika Arora, Jeffrey P Kanne, Travis S Henry, J David Godwin
Connective tissue diseases (CTDs) are a heterogeneous group of conditions characterized by circulating autoantibodies and autoimmune-mediated organ damage. Common CTDs with lung manifestations are rheumatoid arthritis, scleroderma or systemic sclerosis, Sjögren syndrome, polymyositis/dermatomyositis, systemic lupus erythematosis, mixed connective tissue disease, and undifferentiated connective tissue disease. The most common histopathologic patterns of CTD-related interstitial lung disease are nonspecific interstitial pneumonia, usual interstitial pneumonia, organizing pneumonia, and lymphoid interstitial pneumonia...
November 2016: Radiologic Clinics of North America
N Nikitakis, W Papaioannou, L I Sakkas, E Kousvelari
To date there is a major effort in deciphering the role of complex microbial communities, especially the oral and gut microbiomes, in the pathogenesis of various diseases. Increasing evidence indicates a key role for the oral microbiome in autoimmune diseases. In this review article, we discuss links of the oral microbiota to a group of autoimmune diseases, i.e., Sjögren's syndrome (SS), systemic lupus erythematosus (SLE), Crohn's disease (CD), and rheumatoid arthritis (RA). We particularly focus on factors that affect the balance between the immune system and the composition of microbiota leading to dysbiosis, loss of tolerance and subsequent autoimmune disease progression and maintenance...
October 7, 2016: Oral Diseases
Silvia Bosello, Giusy Peluso, Federica Iavarone, Barbara Tolusso, Irene Messana, Gavino Faa, Massimo Castagnola, Gianfranco Ferraccioli
BACKGROUND: In the present study, we investigated whether thymosin β (Tβ) in saliva and in minor salivary glands is differentially expressed in patients with primary Sjögren's syndrome (pSS) and patients with autoimmune diseases (systemic sclerosis [SSc], systemic lupus erythematosus [SLE], and rheumatoid arthritis [RA], with and without sicca syndrome [ss]). METHODS: Saliva specimens of nine patients with pSS, seven with ss/SSc, seven with ss/SLE, seven with ss/RA, seven with SSc, seven with SLE, and seven with RA, as well as ten healthy subjects, were analyzed using a high-performance liquid chromatograph coupled with a mass spectrometer equipped with an electrospray ionization source to investigate the presence and levels of Tβ4, Tβ4 sulfoxide, and Tβ10...
October 6, 2016: Arthritis Research & Therapy
Lütfi Akyol, Soner Önem, Metin Özgen, Mehmet Sayarlıoğlu
Sjögren's syndrome, an autoimmune disease characterized by lymphocytic infiltration of the lacrimal and salivary glands, leads to dryness of the mouth and eyes. Herein, we present a case of Sjögren's syndrome that developed after silicone breast implantation. A cause-effect relationship between breast implantation and Sjögren's syndrome has not been established. However, the possibility of such an association should be considered when a patient with silicone implants is admitted to the hospital for treatment of Sjögren's syndrome...
December 2015: Eur J Rheumatol
T G Coursey, F Bian, M Zaheer, S C Pflugfelder, E A Volpe, C S de Paiva
In both humans and animal models, the development of Sjögren syndrome (SS) and non-SS keratoconjunctivitis sicca (KCS) increases with age. Here, we investigated the ocular surface and lacrimal gland (LG) phenotype of NOD.B10.H2(b) mice at 7-14, 45-50, and 96-100 weeks. Aged mice develop increased corneal permeability, CD4(+) T-cell infiltration, and conjunctival goblet cell loss. Aged mice have LG atrophy with increased lymphocyte infiltration and inflammatory cytokine levels. An increase in the frequency of CD4(+)Foxp3(+) T regulatory cells (Tregs) was observed with age in the cervical lymph node (CLN), spleen, and LG...
October 5, 2016: Mucosal Immunology
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