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Sjogren's syndrome;biopsy

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https://www.readbyqxmd.com/read/27904866/sj%C3%A3-gren-syndrome-related-membranous-glomerulonephritis-progressing-to-membranoproliferative-glomerulonephritis
#1
Junko Yabuuchi, Tatsuya Suwabe, Toshiharu Ueno, Junichi Hoshino, Akinari Sekine, Noriko Hayami, Masahiko Oguro, Kyohei Kunisawa, Masahiro Kawada, Masayuki Yamanouchi, Keiichi Sumida, Hiroki Mizuno, Eiko Hasegawa, Naoki Sawa, Kenmei Takaichi, Kenichi Ohashi, Takeshi Fujii, Yoshifumi Ubara
We report a case of glomerulopathy in a 36-year-old Japanese woman with primary Sjögren syndrome (pSS). The first renal biopsy suggested membranous glomerulonephritis. However, repeat biopsy was performed after 16 years because of increased proteinuria, revealing membranoproliferative glomerulonephritis with mesangial deposits, subendothelial deposits, and subepithelial deposits. Immunofluorescent studies showed predominant deposition of IgG2 and IgG4. This patient was positive for antinuclear antibody and anti-SS-A antibody...
September 2016: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/27899373/influence-of-geolocation-and-ethnicity-on-the-phenotypic-expression-of-primary-sj%C3%A3-gren-s-syndrome-at-diagnosis-in-8310-patients-a-cross-sectional-study-from-the-big-data-sj%C3%A3-gren-project-consortium
#2
Pilar Brito-Zerón, Nihan Acar-Denizli, Margit Zeher, Astrid Rasmussen, Raphaele Seror, Elke Theander, Xiaomei Li, Chiara Baldini, Jacques-Eric Gottenberg, Debashish Danda, Luca Quartuccio, Roberta Priori, Gabriela Hernandez-Molina, Aike A Kruize, Valeria Valim, Marika Kvarnstrom, Damien Sene, Roberto Gerli, Sonja Praprotnik, David Isenberg, Roser Solans, Maureen Rischmueller, Seung-Ki Kwok, Gunnel Nordmark, Yasunori Suzuki, Roberto Giacomelli, Valerie Devauchelle-Pensec, Michele Bombardieri, Benedikt Hofauer, Hendrika Bootsma, Johan G Brun, Guadalupe Fraile, Steven E Carsons, Tamer A Gheita, Jacques Morel, Cristina Vollenveider, Fabiola Atzeni, Soledad Retamozo, Ildiko Fanny Horvath, Kathy Sivils, Thomas Mandl, Pulukool Sandhya, Salvatore De Vita, Jorge Sanchez-Guerrero, Eefje van der Heijden, Virginia Fernandes Moça Trevisani, Marie Wahren-Herlenius, Xavier Mariette, Manuel Ramos-Casals
OBJECTIVES: To analyse the influence of geolocation and ethnicity on the clinical presentation of primary Sjögren's syndrome (SjS) at diagnosis. METHODS: The Big Data Sjögren Project Consortium is an international, multicentre registry designed in 2014. By January 2016, 20 centres from five continents were participating. Multivariable logistic regression analyses were performed. RESULTS: We included 7748 women (93%) and 562 men (7%), with a mean age at diagnosis of primary SjS of 53 years...
November 29, 2016: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/27886798/multicentric-reticulohistiocytosis-rheumatology-perspective
#3
REVIEW
Bahtiyar Toz, Nesimi Büyükbabani, Murat İnanç
Multicentric reticulohistiocytosis (MRH) is a rare, multisystemic non-Langerhans cell histiocytosis characterized by skin and articular involvement, and rarely involves various other organs. There are no specific laboratory findings for MRH. Diagnosis is based on clinical findings and skin or synovial biopsy results. There is currently no consensus for the treatment of MRH. Here, we review the differential diagnosis and treatment options of MRH from the rheumatologist's perspective. We also report an index case of MRH associated with Sjögren's syndrome and pulmonary embolism...
April 2016: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/27872417/virtual-touch-quantification-of-the-salivary-glands-for-diagnosis-of-primary-sj%C3%A3-gren-syndrome
#4
Shaoqi Chen, Yukai Wang, Shaoxing Chen, Qiulin Wu, Shigao Chen
OBJECTIVES: To investigate the value of salivary gland stiffness measured by Virtual Touch quantification (VTQ; Siemens Medical Solutions, Erlangen, Germany) for assessment of primary Sjögren syndrome. METHODS: Fifty-four patients with primary Sjögren syndrome, 35 patients without primary Sjögren syndrome (patients with dry mouth and dry eye symptoms), and 52 healthy control volunteers were included in this study. Patients with primary Sjögren syndrome were classified as early or advanced stage by labial gland biopsies...
December 2016: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
https://www.readbyqxmd.com/read/27862026/unusual-presentation-of-sj%C3%A3-gren-associated-neuropathy-with-plasma-cell-rich-infiltrate
#5
Elie Naddaf, Sarah E Berini, P James B Dyck, Ruple S Laughlin
INTRODUCTION: Sjögren syndrome is thought to be a lymphocyte-driven process. Peripheral nervous system involvement occurs in about 20-25% of patients. A sensory predominant, large fiber peripheral neuropathy is most common, and it is usually associated with a subacute to chronic presentation. METHODS: We report a rare case of an acute Sjögren-associated, sensory predominant, length-dependent peripheral neuropathy mimicking Guillain-Barré syndrome. The patient presented with sensory ataxia preceded by fever and polyarthralgia...
November 16, 2016: Muscle & Nerve
https://www.readbyqxmd.com/read/27818685/primary-sjogren-s-syndrome-presenting-as-acute-interstitial-pneumonitis-hamman-rich-syndrome
#6
Abidullah Khan, Mohammad Humayun, Iqbal Haider, Maimoona Ayub, Zakir Shah, Fahad Ajmal
A previously well, 45-year-old Pakistani lady was admitted to the medical unit on-call of Khyber Teaching Hospital (KTH) Peshawar with a 5-day history of fever, productive cough with copious mucoid sputum, dyspnea, and pleuritic chest pain. She also complained of dry eyes, mouth, and vagina. Her chest X-ray showed diffuse alveolar shadowing and arterial gas analysis confirmed type 1 respiratory failure. Over the next few days, she deteriorated rapidly making an urgent transfer to the medical intensive care unit (MICU) necessary, where she was mechanically ventilated...
2016: Case Reports in Medicine
https://www.readbyqxmd.com/read/27815440/identification-of-novel-cd4-t-cell-subsets-in-the-target-tissue-of-sj%C3%A3-gren-s-syndrome-and-their-differential-regulation-by-the-lymphotoxin-light-signaling-axis
#7
Scott Haskett, Jian Ding, Wei Zhang, Alice Thai, Patrick Cullen, Shanqin Xu, Britta Petersen, Galina Kuznetsov, Luke Jandreski, Stefan Hamann, Taylor L Reynolds, Norm Allaire, Timothy S Zheng, Michael Mingueneau
Despite being one of the most common rheumatologic diseases, there is still no disease-modifying drug for primary Sjögren's syndrome (pSS). Advancing our knowledge of the target tissue has been limited by the low dimensionality of histology techniques and the small size of human salivary gland biopsies. In this study, we took advantage of a molecularly validated mouse model of pSS to characterize tissue-infiltrating CD4(+) T cells and their regulation by the lymphotoxin/LIGHT signaling axis. Novel cell subsets were identified by combining highly dimensional flow and mass cytometry with transcriptomic analyses...
November 15, 2016: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/27803305/clinical-relevance-of-ror%C3%AE-positive-and-negative-subsets-of-cd161-cd4-t-cells-in-primary-sj%C3%A3-gren-s-syndrome
#8
Linlin Zhao, Gaetane Nocturne, Scott Haskett, Saida Boudaoud, Thierry Lazure, Christine Le Pajolec, Xavier Mariette, Michael Mingueneau, Daliya Banerjee
OBJECTIVE: The relevance of the Th17 pathway in primary SS (pSS) is unclear. Published studies have relied on restimulating circulating CD161(+) T cells in vitro for quantitation of IL-17-producing cells. While CD161 marks all IL-17(+) T cells, it is also expressed by other Th subsets. The aim of this study was to directly analyse retinoic acid receptor-related orphan nuclear receptor (ROR)-γ expressing and non-expressing subsets of CD161(+) T cells to determine the relevance of the Th17 pathway in pSS...
November 1, 2016: Rheumatology
https://www.readbyqxmd.com/read/27782867/predictive-significance-of-ccl21-and-cxcl13-levels-in-the-minor-salivary-glands-of-patients-with-sj%C3%A3-gren-s-syndrome
#9
Kyung-Eun Lee, Ji-Hyoun Kang, Yi-Rang Yim, Ji-Eun Kim, Jeong-Won Lee, Lihui Wen, Dong-Jin Park, Tae-Jong Kim, Yong-Wook Park, Kyung Chul Yoon, Ji Shin Lee, Shin-Seok Lee
OBJECTIVES: To investigate whether CCL21 and CXCL13 expression levels in the minor salivary gland are associated with the laboratory and clinical manifestations of Sjögren's syndrome (SS). METHODS: Sociodemographic data on 106 SS patients were obtained and the glandular and extraglandular manifestations of the disease were documented. In addition, minor salivary gland biopsies were performed and the patients' laboratory findings were analysed. European League Against Rheumatism SS disease activity index (ESSDAI) values of SS disease activity at the time of biopsy and the SS disease damage index (SSDDI) values were also recorded...
October 21, 2016: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/27777501/human-postmortem-lacrimal-and-submandibular-glands-stored-in-rnalater-are-suitable-for-molecular-biochemical-and-cell-biological-studies
#10
Dillon Hawley, Hema Aluri, Helene Armaos, Gina Kim, Claire Kublin, Driss Zoukhri
PURPOSE: Gene expression and protein analysis studies require high-quality human tissue which is a challenge and difficult to obtain through live human biopsies. Human postmortem lacrimal gland (LG) and submandibular gland (SMG) tissues have the potential to provide an invaluable source for studying the mechanisms involved in LG and SMG dysfunction. Therefore, we aimed to test the suitability of post-mortem LG and SMG for molecular, biochemical, and cell biological studies. METHODS: LG and SMG tissue from healthy donors was collected and immediately placed in RNAlater solution and then shipped overnight at 4 °C...
2016: Molecular Vision
https://www.readbyqxmd.com/read/27753592/a-case-of-idiopathic-retroperitoneal-fibrosis-associated-with-sj%C3%A3-gren-s-syndrome
#11
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
https://www.readbyqxmd.com/read/27738539/bilateral-lacrimal-gland-lymphoma-in-sj%C3%A3-gren-syndrome
#12
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
https://www.readbyqxmd.com/read/27738403/sj%C3%A3-gren-s-syndrome-associated-with-systemic-lupus-erythematosus
#13
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
https://www.readbyqxmd.com/read/27672471/skin-findings-in-a-patient-with-sjogren-s-syndrome
#14
Prajwal Boddu, Abdul S Mohammed, Sonali Khandelwal
Hypergammaglobulinemic purpura (HGP) is a syndrome constellating recurrent purpura, hypergammaglobulinemia, positive rheumatoid factor (RF), anti-Ro/La antibodies, and elevated erythrocyte sedimentation rate (ESR). We present a case of a 29-year-old female who was diagnosed with Sjogren's syndrome four years prior to presenting with rash on her lower extremities for a period of 6 months. Skin biopsy at the initial visit was consistent with leukocytoclastic vasculitis and was initiated on treatment for it. Her rash evolved into 2-5 mm scattered purpurae while she was on the treatment and a repeat biopsy showed extravasation of RBCs, a sparse mononuclear infiltrate with deposition of plasma cells, and no evidence of leukocytoclastic vasculitis, thus showing a transition from neutrophilic to mononuclear inflammatory vascular disease which is a rare occurrence...
2016: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/27662653/high-grade-salivary-gland-involvement-assessed-by-histology-or-ultrasonography-is-associated-with-a-poor-response-to-a-single-rituximab-course-in-primary-sj%C3%A3-gren-s-syndrome-data-from-the-tears-randomized-trial
#15
Divi Cornec, Sandrine Jousse-Joulin, Sebastian Costa, Thierry Marhadour, Pascale Marcorelles, Jean-Marie Berthelot, Eric Hachulla, Pierre-Yves Hatron, Vincent Goeb, Olivier Vittecoq, Emmanuel Nowak, Jacques-Olivier Pers, Valérie Devauchelle-Pensec, Alain Saraux
PURPOSE: To determine whether the severity of salivary-gland involvement, assessed using salivary gland ultrasonography [SGUS], histological focus score, or the unstimulated whole salivary flow [UWSF], was associated with the response to rituximab in patients with primary Sjögren's syndrome [pSS]. MATERIALS AND METHODS: Among the 120 patients with pSS enrolled in the randomised TEARS trial of rituximab versus placebo, 35 underwent either centralised minor salivary-gland biopsy or SGUS at inclusion...
2016: PloS One
https://www.readbyqxmd.com/read/27644591/association-of-dry-eye-tests-with-extraocular-signs-among-3514-participants-in-the-sj%C3%A3-gren-s-syndrome-international-registry
#16
Vatinee Y Bunya, Satasuk Joy Bhosai, Ana Maria Heidenreich, Kazuko Kitagawa, Genevieve B Larkin, Thomas M Lietman, Bruce D Gaynor, Esen K Akpek, Mina Massaro-Giordano, M Srinivasan, Travis C Porco, John P Whitcher, Stephen C Shiboski, Lindsey A Criswell, Caroline H Shiboski
PURPOSE: To identify a screening strategy for dry eye patients with a high likelihood of having Sjogren syndrome (SS) through the evaluation of the association of ocular surface tests with the extraocular signs used for the diagnosis of SS. DESIGN: Multicenter cross-sectional study. METHODS: The Sjogren's International Clinical Collaborative Alliance (SICCA) registry enrolled 3514 participants with SS or possible SS from 9 international academic sites...
September 16, 2016: American Journal of Ophthalmology
https://www.readbyqxmd.com/read/27629974/acute-motor-dominant-polyneuropathy-as-guillain-barr%C3%A3-syndrome-and-multiple-mononeuropathies-in-a-patient-with-sj%C3%A3-gren-s-syndrome
#17
Kenichiro Tanaka, Hiroyuki Nakayasu, Yutaka Suto, Shotaro Takahashi, Yoshihiro Konishi, Hirotake Nishimura, Rino Ueno, Susumu Kusunoki, Kenji Nakashima
A patient with xerostomia and xerophthalmia due to Sjögren's syndrome presented with acute motor-dominant polyneuropathy and multiple mononeuropathy with antiganglioside antibodies. Nerve conduction studies and a sural nerve biopsy revealed the neuropathy as a mixture of segmental demyelination and axonal degeneration. Positive results were obtained for several antiganglioside antibodies. Corticosteroid treatment proved effective. The neuropathy was considered to represent a mixture of polyneuropathy as Guillain-Barré syndrome and multiple mononeuropathy via Sjögren's syndrome...
2016: Internal Medicine
https://www.readbyqxmd.com/read/27585081/autoimmune-swallowing-disorders
#18
Janine Amos, Anthony Baron, Adam D Rubin
PURPOSE OF REVIEW: Dysphagia can be the presenting symptom of autoimmune disease. The otolaryngologist should be familiar with and consider these disorders when assessing the patient with swallowing difficulties. RECENT FINDINGS: The present review gives a brief overview of Sjogren's syndrome, granulomatosis with polyangiitis, pemphigus and pemphigoid, rheumatoid arthritis, systemic lupus erythematosus, scleroderma and inflammatory myopathies, and how they affect swallowing...
August 31, 2016: Current Opinion in Otolaryngology & Head and Neck Surgery
https://www.readbyqxmd.com/read/27567226/interleukin-15-as-a-potential-new-target-in-sj%C3%A3-gren-s-syndrome-associated-inflammation
#19
Margherita Sisto, Loredana Lorusso, Sabrina Lisi
IL-15 is a key regulatory cytokine that shares many biological properties with IL-2. Recently, it has been shown that IL-15 could be up-regulated in T cell-mediated inflammatory disorders, such as rheumatoid arthritis and inflammatory bowel diseases. However, the role and expression of IL-15 in the inflammatory autoimmune disease Sjögren's syndrome (SS) has not been investigated. In the present study we evaluated the expression of IL-15 mRNA and protein in minor salivary gland (MSG) biopsy specimens and in human salivary gland epithelial cell (SGEC) cultures obtained from patients with primary SS (pSS) and compared their expression with that seen in normal healthy control subjects...
October 2016: Pathology
https://www.readbyqxmd.com/read/27562035/serum-cxcl4-increase-in-primary-sj%C3%A3-gren-s-syndrome-characterizes-patients-with-microvascular-involvement-and-reduced-salivary-gland-infiltration-and-lymph-node-involvement
#20
Serena Vettori, Rosaria Irace, Antonella Riccardi, Daniela Iacono, Luciana Pellecchia, Lucia Vicedomini, Gabriele Valentini
CXCL4 is an antiangiogenic and immunomodulatory chemokine. We aimed to investigate serum levels of CXCL4 in primary Sjögren's syndrome (pSS), looking for associations with disease features. Thirty-nine consecutive pSS patients underwent clinical-serological assessment and nailfold videocapillaroscopy (NVC). Thirty-six patients and 30 controls affected by osteoarthritis were also investigated for serum levels of CXCL4 and soluble E-selectin (sE-selectin). CXCL4 was higher in pSS patients than in controls (1...
October 2016: Clinical Rheumatology
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