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https://www.readbyqxmd.com/read/29151514/a-case-of-dermatomyositis-complicated-by-digital-ischemia-and-lung-adenocarcinoma-in-a-patient-with-positive-anti-signal-recognition-particle-antibodies
#1
Takashi Nawata, Makoto Kubo, Hitomi Mitsui, Keiji Oishi, Masatoshi Omoto, Takashi Kanda, Masafumi Yano
A 58-year-old Japanese woman was diagnosed with anti-signal recognition particle (SRP)-positive dermatomyositis associated with Sjögren's syndrome, rheumatoid arthritis and lung adenocarcinoma. She presented with cutaneous lesions, including ulceration of her right middle finger. Tissue specimens obtained from her right deltoid muscle were positive for CD4(+) T-cell infiltration and the sarcolemma showed the upregulation of major histocompatibility complex (MHC) class I antigens. The present case suggests that overlapping autoimmune diseases or complications of malignancy may result in an atypical clinical presentations and histological findings in patients with anti-SRP antibody-positive dermatomyositis...
November 20, 2017: Internal Medicine
https://www.readbyqxmd.com/read/28994016/cardiopulmonary-manifestations-of-collagen-vascular-diseases
#2
REVIEW
Hamza Jawad, Sebastian R McWilliams, Sanjeev Bhalla
PURPOSE OF REVIEW: The study aimed to illustrate the cardiopulmonary findings of the following collagen vascular diseases on cross-sectional imaging: rheumatoid arthritis, scleroderma (progressive systemic sclerosis), systemic lupus erythematosus, the inflammatory myopathies (polymyositis/dermatomyositis), and Sjögren's syndrome. RECENT FINDINGS: Although collagen vascular diseases can affect any part of the body, interstitial lung disease and pulmonary hypertension are the two most important cardiopulmonary complications and are responsible for the majority of morbidity and mortality in this patient population...
October 9, 2017: Current Rheumatology Reports
https://www.readbyqxmd.com/read/28913747/primary-sj%C3%A3-gren-s-syndrome-extraglandular-manifestations-and-hydroxychloroquine-therapy
#3
J Demarchi, S Papasidero, M A Medina, D Klajn, R Chaparro Del Moral, O Rillo, V Martiré, G Crespo, A Secco, A Catalan Pellet, C Amitrano, C Crow, C Asnal, P Pucci, F Caeiro, N Benzanquen, J P Pirola, M Mayer, F Zazzetti, S Velez, J Barreira, N Tamborenea, L Santiago, L Raiti
The use of hydroxychloroquine (HCQ) in Primary Sjögren's Syndrome (pSS) has been assessed in different studies over the last years, with conflicting results regarding its efficacy in sicca syndrome and extraglandular manifestations (EGM). The goal of this study was to compare the incidence rate of EGM in pSS patients with and without HCQ therapy.We performed a multicenter retrospective study, including patients with pSS (European classification criteria) with at least 1 year of follow-up. Subjects with concomitant fibromyalgia, autoimmune hepatitis, primary biliary cirrhosis, and primary sclerosing cholangitis were excluded...
November 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28814662/irgm1-coordinately-regulates-autoimmunity-and-host-defense-at-select-mucosal-surfaces
#4
Kathleen M Azzam, Jennifer H Madenspacher, Derek W Cain, Lihua Lai, Kymberly M Gowdy, Prashant Rai, Kyathanahalli Janardhan, Natasha Clayton, Willie Cunningham, Heather Jensen, Preeyam S Patel, John F Kearney, Gregory A Taylor, Michael B Fessler
The pathogenesis of primary Sjogren's syndrome (SS), an autoimmune disease that targets the mucosa of exocrine tissues, is poorly understood. Although several mouse models have been developed that display features of SS, most of these are within the larger context of a lupus-like presentation. Immunity-related GTPase family M protein 1 (Irgm1) is an interferon-inducible cytoplasmic GTPase that is reported to regulate autophagy and mitochondrial homeostasis. Here, we report that naive Irgm1-/- mice display lymphocytic infiltration of multiple mucosal tissues including the lung in a manner reminiscent of SS, together with IgA class-predominant autoantibodies including anti-Ro and anti-La...
August 17, 2017: JCI Insight
https://www.readbyqxmd.com/read/28789696/long-term-follow-up-in-primary-sj%C3%A3-gren-s-syndrome-reveals-differences-in-clinical-presentation-between-female-and-male-patients
#5
Jorge I Ramírez Sepúlveda, Marika Kvarnström, Per Eriksson, Thomas Mandl, Katrine Brække Norheim, Svein Joar Johnsen, Daniel Hammenfors, Malin V Jonsson, Kathrine Skarstein, Johan G Brun, Lars Rönnblom, Helena Forsblad-d'Elia, Sara Magnusson Bucher, Eva Baecklund, Elke Theander, Roald Omdal, Roland Jonsson, Gunnel Nordmark, Marie Wahren-Herlenius
BACKGROUND: Despite men being less prone to develop autoimmune diseases, male sex has been associated with a more severe disease course in several systemic autoimmune diseases. In the present study, we aimed to investigate differences in the clinical presentation of primary Sjögren's syndrome (pSS) between the sexes and establish whether male sex is associated with a more severe form of long-term pSS. METHODS: Our study population included 967 patients with pSS (899 females and 68 males) from Scandinavian clinical centers...
August 8, 2017: Biology of Sex Differences
https://www.readbyqxmd.com/read/28748510/clinical-characteristics-of-autoimmune-rheumatic-disease-related-organizing-pneumonia
#6
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/28439062/a-rare-case-of-necrotizing-myopathy-and-fibrinous-and-organizing-pneumonia-with-anti-ej-antisynthetase-syndrome-and-ssa-antibodies
#7
Muhammad Kashif, Divya Arya, Masooma Niazi, Misbahuddin Khaja
BACKGROUND Idiopathic inflammatory myopathies are autoimmune disorders that can involve the skin, joints, muscles, and lungs. The most common of these disorders are dermatomyositis, polymyositis, overlap syndrome, and inclusion body myositis. Necrotizing autoimmune myopathy is an idiopathic inflammatory myopathy that is rarely associated with Sjögren's syndrome. The most common lung findings associated with anti-EJ antisynthetase syndrome are nonspecific interstitial pneumonia and usual interstitial pneumonia; this condition is rarely associated with fibrinous and organizing pneumonia...
April 25, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28417151/a-review-of-the-role-and%C3%A2-clinical-utility-of-anti-ro52-trim21-in-systemic-autoimmunity
#8
REVIEW
Adrian Y S Lee
Anti-Ro52/tripartite motif-containing 21 (TRIM21) is a ubiquitous antibody found in a number of systemic autoimmune conditions including Sjögren's syndrome, systemic lupus erythematosus and systemic sclerosis, appearing in about half of these patients. Once coupled with its closely related antibody, anti-Ro60 as the anti-SSA antibody, anti-Ro52 is emerging as a unique antibody with direct pathogenic disease involvement and distinct clinical properties. As a result, recent attention has turned to this antibody and its clinical associations and utility...
August 2017: Rheumatology International
https://www.readbyqxmd.com/read/28415674/primary-sj%C3%A3-gren-s-syndrome-with-diffuse-cystic-lung-changes-developed-systemic-lupus-erythematosus-a-case-report-and-literature-review
#9
Xiao Liu, Hao Li, Yunhong Yin, Dedong Ma, Yiqing Qu
Sjögren's syndrome (SS) is a chronic inflammatory autoimmune disease that can occur as a unique existence (primary Sjögren's syndrome) or merge with other systemic diseases like systemic lupus erythematosus (SLE), rheumatoid arthritis or systemic sclerosis (secondary Sjögren's syndrome). Data on the two diseases occurrence order are inadequate. Primary Sjögren's syndrome (pSS) may relatively uncommonly lead to diffuse cystic lung changes. We represent a female who was diagnosed pSS with diffuse cystic lung alterations developed SLE two years later...
May 23, 2017: Oncotarget
https://www.readbyqxmd.com/read/28288723/clinical-features-and-management-of-non-gastrointestinal-non-ocular-extranodal-mucosa-associated-lymphoid-tissue-enmalt-marginal-zone-lymphomas
#10
REVIEW
Estella Matutes, Carlos Montalban
Extranodal mucosa associated lymphoid tissue (ENMALT) marginal zone lymphomas may arise at any site of the body. The most frequent localizations other than gastrointestinal and eye are salivary gland, skin, lung and thyroid. These lymphomas usually arise in a setting of inflammation due to a persistent infection or autoimmune diseases such as Sjogren syndrome in salivary MALT lymphomas and Hashimoto's thryroiditis in thyroid lymphomas. They affect middle-aged patients with a female predominance when lymphoma arises in certain locations...
March 2017: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/28166540/effect-of-tobacco-smoking-on-the-clinical-histopathological-and-serological-manifestations-of-sj%C3%A3-gren-s-syndrome
#11
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/27719973/imaging-of-pulmonary-manifestations-of-connective-tissue-diseases
#12
REVIEW
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
https://www.readbyqxmd.com/read/27492512/nonspecific-interstitial-pneumonia-preceding-diagnosis-of-collagen-vascular-disease
#13
Masato Kono, Yutaro Nakamura, Katsuhiro Yoshimura, Yasunori Enomoto, Yoshiyuki Oyama, Hironao Hozumi, Noriyuki Enomoto, Tomoyuki Fujisawa, Naoki Inui, Etsuko Hamada, Thomas V Colby, Masato Maekawa, Takafumi Suda
BACKGROUND: The aim of this study was to evaluate the incidence and clinical features of patients who developed collagen vascular disease (CVD) after an initial diagnosis of idiopathic nonspecific interstitial pneumonia (NSIP). METHODS: We conducted a retrospective review of 72 consecutive patients with NSIP who were diagnosed by surgical lung biopsy in our institution (idiopathic NSIP, n = 35; CVD-NSIP, n = 37 at initial diagnosis). No patients fulfilled the American College of Rheumatology criteria for a diagnosis with CVD within six months after the diagnosis of idiopathic NSIP...
August 2016: Respiratory Medicine
https://www.readbyqxmd.com/read/27421224/primary-sj%C3%A3-gren-s-syndrome
#14
REVIEW
Maureen Rischmueller, Joanna Tieu, Susan Lester
Primary Sjögren's syndrome (pSS) is a relatively common autoimmune systemic rheumatic disease. In addition to sicca syndrome and swollen salivary glands, systemic features manifest in the majority of patients, and are severe in 15%, particularly affecting the joints, skin, lungs, and peripheral nervous system. A recent meta-analysis estimated a pooled relative risk of 13.76 for the development of non-Hodgkin lymphoma, particularly in pSS patients who have parotid enlargement, vasculitis, cryoglobulinemia, and antibodies to Ro and La...
February 2016: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/27421127/-pulmonary-involvement-in-connective-tissue-disease
#15
Małgorzata Bartosiewicz
The connective tissue diseases are a variable group of autoimmune mediated disorders characterized by multiorgan damage. Pulmonary complications are common, usually occur after the onset of joint symptoms, but can also be initially presenting complaint. The respiratory system may be involved in all its component: airways, vessels, parenchyma, pleura and respiratory muscles. Lung involvement is an increasing cause of morbidity and mortality in the connective tissue diseases. Clinical course is highly variable - can range from mild to rapidly progressive, some processes are reversible, while others are irreversible...
2016: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/27275245/sj%C3%A3-gren-s-syndrome-and-silicosis-a-case-report
#16
Aleksandra Plavsic, Rada Miskovic, Jasna Bolpacic, Branka Šuštran, Aleksandra Peric-Popadic, Mirjana Bogic
Sjögren's syndrome is an autoimmune disease of unknown etiology where immune response to self-antigens is believed to result from interactions between genetic and environmental factors. We describe the case of a patient who has been diagnosed with Sjögren's syndrome based on typical clinical and immunological parameters. The clinical picture was dominated by the respiratory symptoms, and radiographic and multislice computed tomography examination of the chest showed certain changes characteristic of pneumoconiosis...
June 15, 2015: Open Access Macedonian Journal of Medical Sciences
https://www.readbyqxmd.com/read/27197325/-sj%C3%A3-gren-s-syndrome-when-to-suspect-and-how-to-confirm
#17
Aikaterini Liapi, Alice Horisberger, Spertini François, Camillo Ribi
Sjögren's syndrome (SS) is an autoimmune disease leading to mucosal dryness. It may also involve joints, nerves, kidneys and lungs. Patients with SS are also at increased risk for lymphoma. Diagnosis of SS relies on clinical, biological, histological and radiological criteria, after exclusion of other causes. Initial work-up may be performed in general practice, by serology (antinuclear and anti-SSA/SSB antibodies, rheumatoid factor) and by measuring lacrimal and salivary flow. Antibodies may be within normal range in up to one third of patients and when present are not specific for SS...
April 6, 2016: Revue Médicale Suisse
https://www.readbyqxmd.com/read/27130621/risk-of-autoimmune-disease-in-adults-with-chronic-insomnia-requiring-sleep-inducing-pills-a-population-based-longitudinal-study
#18
Victor C Kok, Jorng-Tzong Horng, Guo-Dung Hung, Jia-Li Xu, Tzu-Wei Hung, Yu-Ching Chen, Chien-Lung Chen
BACKGROUND: Recent studies indicate that chronic insomnia is associated with the development of certain somatic diseases. Whether it would be associated with the development of an autoimmune disease (AID) was unknown. OBJECTIVE: We aimed to examine the association and quantify the magnitude of risk for AID in individuals suffering from chronic insomnia requiring sleep-inducing pills. DESIGN: This was a population-based, nationwide longitudinal study...
September 2016: Journal of General Internal Medicine
https://www.readbyqxmd.com/read/26807990/rett-syndrome-an-autoimmune-disease
#19
REVIEW
Claudio De Felice, Silvia Leoncini, Cinzia Signorini, Alessio Cortelazzo, Paolo Rovero, Thierry Durand, Lucia Ciccoli, Anna Maria Papini, Joussef Hayek
Rett syndrome (RTT) is a devastating neurodevelopmental disease, previously included into the autistic spectrum disorders, affecting almost exclusively females (frequency 1:10,000). RTT leads to intellective deficit, purposeful hands use loss and late major motor impairment besides featuring breathing disorders, epilepsy and increased risk of sudden death. The condition is caused in up to 95% of the cases by mutations in the X-linked methyl-CpG binding protein 2 (MECP2) gene. Our group has shown a number of previously unrecognized features, such as systemic redox imbalance, chronic inflammatory status, respiratory bronchiolitis-associated interstitial lung disease-like lung disease, and erythrocyte morphology changes...
April 2016: Autoimmunity Reviews
https://www.readbyqxmd.com/read/26765478/increased-risks-of-spontaneous-bacterial-peritonitis-and-interstitial-lung-disease-in-primary-biliary-cirrhosis-patients-with-concomitant-sj%C3%A3-gren-syndrome
#20
Chun-Ting Chen, Yu-Chen Tseng, Chih-Wei Yang, Hsuan-Hwai Lin, Peng-Jen Chen, Tien-Yu Huang, Yu-Lueng Shih, Wei-Kuo Chang, Tsai-Yuan Hsieh, Heng-Cheng Chu
The incidence of Sjögren syndrome (SS) in primary biliary cirrhosis (PBC) patients is high. The influence of SS on the clinical outcomes of PBC patients, however, remains unclear. Our study retrospectively collected data on PBC-only patients and PBC patients with concomitant SS (PBC-SS) to compare the clinical differences of long-term outcomes between them.A total of 183 patients were diagnosed with PBC from January 1999 to December 2014 at our hospital. Of these, the authors excluded patients with diabetes, hypertension, advanced liver cirrhosis at initial diagnosis of PBC (Child-Turcotte-Pugh classification score of ≥7) and other liver diseases (ie, alcoholic liver disease, alpha-antitrypsin deficiency, viral hepatitis, and primary sclerosing cholangitis), and autoimmune diseases such as systemic lupus erythematosus and rheumatoid arthritis...
January 2016: Medicine (Baltimore)
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