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Mixed connective tissue disease

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https://www.readbyqxmd.com/read/29413506/pulmonary-arterial-hypertension-in-the-setting-of-scleroderma-is-different-than-in-the-setting-of-lupus-a-review
#1
REVIEW
Isabel S Bazan, Kofi A Mensah, Anastasiia A Rudkovskaia, Percy K Adonteng-Boateng, Erica L Herzog, Lenore Buckley, Wassim H Fares
Pulmonary hypertension (PH) is a clinical syndrome that is subdivided into five groups per the World Health Organization (WHO) classification, based largely on hemodynamic and pathophysiologic criteria. WHO Group 1 PH, termed pulmonary arterial hypertension (PAH), is a clinically progressive disease that can eventually lead to right heart failure and death, and it is hemodynamically characterized by pre-capillary PH and increased pulmonary vascular resistance in the absence of elevated left ventricular filling pressures...
January 2018: Respiratory Medicine
https://www.readbyqxmd.com/read/29369534/autoimmunity-in-uveitis
#2
Josianne C Ten Berge, Marco W J Schreurs, Paul L A van Daele, Aniki Rothova
PURPOSE: Recent insights into the pathogenesis of immune-mediated diseases proposed a new classification, which includes autoimmune and auto-inflammatory diseases. The prevalence of specific autoimmune and auto-inflammatory diseases in uveitis and/or scleritis is not yet known. In this study, we examine the presence of systemic immune-mediated diseases in patients with uveitis and/or scleritis and put a special emphasis on autoimmune disorders by reporting on their clinical manifestations and visual prognosis...
January 25, 2018: Acta Ophthalmologica
https://www.readbyqxmd.com/read/29364249/simultaneous-distinction-of-monospecific-and-mixed-dfs70-patterns-during-ana-screening-with-a-novel-hep-2-elite-dfs70-knockout-substrate
#3
Kishore S Malyavantham, Lakshmanan Suresh
Systemic autoimmune connective tissue disorders are characterized by circulating antinuclear antibodies (ANA). Although there are several technologies available for ANA screening, indirect immunofluorescence (IIF) using Human epithelial cells-2 (HEp-2) substrate remains the primary and recommended method because of its superior sensitivity. HEp-2 substrates can detect a multitude of patterns resulting from autoantibody binding to various protein and nucleic acid autoantigens distributed throughout the nucleus and cytoplasm of the cells...
January 17, 2018: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/29356883/evaluation-of-itgb2-cd18-and-sell-cd62l-genes-expression-and-methylation-of-itgb2-promoter-region-in-patients-with-systemic-sclerosis
#4
Navid Dashti, Mahdi Mahmoudi, Farhad Gharibdoost, Hoda Kavosi, Ramazan Rezaei, Vahideh Imeni, Ahmadreza Jamshidi, Saeed Aslani, Shayan Mostafaei, Mohammad Vodjgani
Systemic sclerosis (SSc), an autoimmune disease of connective tissue, is characterized by inflammation, fibrosis, and vessel endothelial damage. Products of Integrin subunit beta 2 (ITGB2) and selectin L (SELL) genes participate in several functional pathways of immune system. The aim of this investigation was to survey the transcript level of ITGB2 and SELL genes as well as methylation status of CpG sites in promoter region of differently expressed gene in PBMCs of SSc patients. PBMCs were isolated from whole blood of 50 SSc patients and 30 healthy controls...
January 22, 2018: Rheumatology International
https://www.readbyqxmd.com/read/29353024/outcomes-and-mortality-prediction-model-of-critically-ill-adults-with-acute-respiratory-failure-and-interstitial-lung-disease
#5
Whitney Gannon, David J Lederer, Mauer Biscotti, Azka Javaid, Nina Patel, Daniel Brodie, Matthew Bacchetta, Matthew Baldwin
BACKGROUND: We aimed to examine short- and long-term mortality in a mixed population of interstitial lung disease (ILD) patients with acute respiratory failure, and to identify those at lower versus higher risk of in-hospital death. METHODS: We conducted a single-center retrospective cohort study of 126 consecutive adults with ILD admitted to an intensive care unit (ICU) for respiratory failure at a tertiary care hospital between 2010 and 2014 who did not undergo lung transplantation during their hospitalization...
January 15, 2018: Chest
https://www.readbyqxmd.com/read/29306915/screening-for-connective-tissue-disease-associated-antibodies-by-automated-immunoassay
#6
Philippe Willems, Ellen De Langhe, Jolien Claessens, René Westhovens, Erna Van Hoeyveld, Koen Poesen, Steven Vanderschueren, Daniel Blockmans, Xavier Bossuyt
BACKGROUND: Antinuclear antibodies (ANAs) are useful for the diagnosis of ANA-associated systemic rheumatic disease (AASRD). The objective of this study was the evaluation of an immunoassay that detects antibodies to a mixture of 17 antigens as an alternative to indirect immunofluorescence (IIF). METHODS: Nine thousand eight hundred and fifty-six consecutive patients tested for ANAs were tested by IIF and EliA connective tissue disease screen (Thermo-Fisher). Medical records were reviewed for 2475 patients, including all patients that tested positive/equivocal by either test and a selection of 500 patients that tested negative...
January 8, 2018: Clinical Chemistry and Laboratory Medicine: CCLM
https://www.readbyqxmd.com/read/29275576/-clinical-features-of-acute-diffuse-pulmonary-exudative-disorders
#7
L Y Mi, C J Ban, Y Liu, N Bao, B Sun, M Zhu, Y H Ren, H P Dai
Objective: To analyze the clinical features of acute diffuse pulmonary exudative disorders. Methods: The data were collected from patients who were hospitalized in respiratory intensive care unit (RICU) of Beijing Chaoyang Hospital affiliated to the Capital Medical University during January 2009 to December 2011, and had acute clinical course with imaging findings of diffuse pulmonary infiltrated shadows (similar to acute respiratory distress syndrome (ARDS)). The causes of disease and clinical features were analyzed...
November 28, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/29237099/calcium-channel-blockers-for-primary-and-secondary-raynaud-s-phenomenon
#8
REVIEW
Fadumo Rirash, Paul C Tingey, Sarah E Harding, Lara J Maxwell, Elizabeth Tanjong Ghogomu, George A Wells, Peter Tugwell, Janet Pope
BACKGROUND: Raynaud's phenomenon is a vasospastic disease characterized by digital pallor, cyanosis, and extremity pain. Primary Raynaud's phenomenon is not associated with underlying disease, but secondary Raynaud's phenomenon is associated with connective tissue disorders such as systemic sclerosis, systemic lupus erythematosus, and mixed connective tissue disease. Calcium channel blockers promote vasodilation and are commonly used when drug treatment for Raynaud's phenomenon is required...
December 13, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/29199167/which-diseases-are-risk-factors-for-developing-gastroesophageal-reflux-disease
#9
Filiz Akyüz, Özlem Mutluay Soyer
Although the pathophysiology of gastroesophageal reflux disease (GERD) remains unclear, it is accepted as a multifactorial disease. It is thought that some of the interventions that might cause alterations in the normal gastrointestinal tract anatomy and diseases that affect the lower esophageal sphincter, esophageal clearance, and stomach motility (gastric emptying) might lay a foundation for GERD development. Moreover, it is common knowledge that GERD might cause various extraesophageal symptoms and complications...
December 2017: Turkish Journal of Gastroenterology: the Official Journal of Turkish Society of Gastroenterology
https://www.readbyqxmd.com/read/29072657/-the-influence-of-biological-compatibility-of-the-cyanoacrylate-glue-on-regeneration-of-the-cartilaginous-tissue
#10
F V Semenov, N F Skibitskaya
The objective of the present study was to evaluate the possibility of the application of the cyanoacrylate-based glue for the strengthening of the reconstructed elements of the middle ear and its influence on the regeneration of the cartilaginous tissue. We used the cartilaginous tissue from the auricles of the male California rabbits as a model. The cartilage was destroyed in a standard press. Half of the cartilage thus fragmented was implanted into the left auricle. The remaining part was mixed up with the cyanoacrylate glue and implanted into the right auricle of the same animal...
2017: Vestnik Otorinolaringologii
https://www.readbyqxmd.com/read/29054902/chylous-ascites-in-a-patient-with-an-overlap-syndrome-a-surprising-response-to-rituximab
#11
Alexandra Daniel, Gianluca Bagnato, Edward Vital, Francesco Del Galdo
The authors present a case of a 51-year-old woman with clinical diagnosis of mixed connective tissue disease and overlap systemic lupus erythematosus features, with a 6-month history of progressive painless abdominal distension. On examination, evident signs of ascites were present. Both the abdominal-pelvic ultrasound and CT scan confirmed a large amount of ascites. A diagnostic paracentesis was performed, which revealed typical features of chylous ascites (CA). An extensive diagnostic work-up led by a multidisciplinary team was performed, excluding malignancy, cirrhosis, infectious, as well as cardiac and primary lymphatic causes...
October 20, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/29051891/scleroderma-renal-crisis-in-mixed-connective-tissue-disease-with-full-renal-recovery-within-3-months-a-case-report-with-expanding-treatment-modalities-to-treat-each-clinical-sign-as-an-independent-entity
#12
Jordana Cheta, Suresh Rijhwani, Harlan Rust
Mixed connective tissue disease (MCTD) is a rheumatologic overlap syndrome that can present with symptoms of systemic lupus erythematous, scleroderma, and polymyositis. A severe but rare complication that can occur in MCTD is scleroderma renal crisis. With multiple poor prognostic indicators, the renal outcome is usually poor. The clinical and histological picture is one of a thrombotic microangiopathy. Clinical suspicion has to be high for additional thrombotic or autoimmune processes coexisting due to associated morbidity...
October 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/28977485/advocating-for-early-interstitial-lung-disease-detection-in-mixed-connective-tissue-disease-ild-detection-in-mixed-connective-tissue-disease
#13
Bridget A Graney, Aryeh Fischer
No abstract text is available yet for this article.
July 18, 2017: Rheumatology
https://www.readbyqxmd.com/read/28952230/how-can-we-effectively-address-the-paraneoplastic-dermatomyositis-diagnosis-risk-factors-and-treatment-options
#14
Ioannis Zerdes, Maria Tolia, Michail Nikolaou, Nikolaos Tsoukalas, Louloudenia Velentza, Jiannis Hajiioannou, Michail Mitsis, George Kyrgias
PURPOSE: Dermatomyositis (DM) represents an auto-immune inflammatory myopathy. In this review, we analyzed the incidence of DM as a clinical manifestation highlighting the peculiar clinical and treatment characteristics of this disease when occurring in the context of different malignancies. METHODS: A systematic literature review was performed based on database search in PubMed/Medline and included English articles until December 2016. RESULTS: In up to 20% of cases DM appears as a paraneoplastic syndrome associated with multiple malignancies such as ovarian, breast, prostate, lung, nasopharyngeal and colorectal cancer, and non-Hodgkin lymphomas...
July 2017: Journal of B.U.ON.: Official Journal of the Balkan Union of Oncology
https://www.readbyqxmd.com/read/28937479/retinal-vasculitis-and-choroidopathy-in-pediatric-onset-mixed-connective-tissue-disease
#15
Bradley Postlethwaite, Henry G Wynn, Debendra Pattanaik, Shelley Ost, Charles B MacDonald, R Christopher Walton, Seunghyun Kim, Linda K Myers, Monica Brown Lobbins
No abstract text is available yet for this article.
October 2017: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/28931060/isolated-positive-anti-ss-b-autoantibodies-are-not-related-to-clinical-features-of-systemic-autoimmune-diseases-results-from-a-routine-population-survey
#16
Sabine Jardel, Nicole Fabien, Arnaud Hot, Sandra Vukusic, Jacques Tebib, Vincent Cottin, Pascal Sève, Maurice Laville, Alexandre Belot, Isabelle Durieu, Lorna Garnier, Frédéric Coutant, Quitterie Reynaud, Jean Christophe Lega
OBJECTIVE: To assess in clinical practice the frequency and diagnosis associated with the SS-B-positive/SS-A negative autoantibody profile. METHODS: We analyzed a one-year consecutive population of 624 patients referred by clinicians to the immunology laboratory to investigate anti-SS-A and/or anti-SS-B autoantibodies, who were detected using luminex technology. Data were analyzed for patients with isolated anti-SS-B autoantibodies. The clinical characteristics and diagnosis of connective tissue diseases (CTD) were retrieved according to the international criteria...
2017: PloS One
https://www.readbyqxmd.com/read/28904690/-mixed-connective-tissue-disease-prevalence-and-clinical-characteristics-in-african-black-study-of-7-cases-in-gabon-and-review-of-the-literature
#17
REVIEW
Landry Missounga, Josaphat Iba Ba, Ingrid Rosalie Nseng Nseng Ondo, Maria Ines Carine Nziengui Madjinou, Doris Malekou, Emeline Gracia Mouendou Mouloungui, Emmanuel Ecke Nzengue, Jean Bruno Boguikouma, Moussavou Kombila
The literature reports that mixed connective tissue disease seems more frequent in the black population and among Asians. This study aims to determine the prevalence of mixed connective tissue disease (MCTD) among connective tissue disorders and all rheumatologic pathologies in a hospital population in Gabon as well as to describe the clinical features of this disease. We conducted a retrospective study by reviewing the medical records of patients treated for mixed connective tissue disease (Kasukawa criteria) and other entities of connective tissue disorders (ACR criteria) in the Division of Rheumatology at the University Hospital in Libreville between January 2010 and December 2015...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28904255/predictors-of-favorable-responses-to-immunosuppressive-treatment-in-pulmonary-arterial-hypertension-associated-with-connective-tissue-disease
#18
Hidekata Yasuoka, Yuichiro Shirai, Yuichi Tamura, Tsutomu Takeuchi, Masataka Kuwana
BACKGROUND: The potential efficacy of immunosuppressive (IS) treatment has been reported in patients with pulmonary arterial hypertension (PAH) associated with connective tissue disease (CTD), but its positioning in the treatment algorithm remains uncertain. The aim of this study was to identify predictors of favorable responses to first-line IS treatment.Methods and Results:This single-center retrospective study included 30 patients with PAH accompanied by systemic lupus erythematosus (SLE), mixed CTD (MCTD), or primary Sjögren's syndrome (SS) who received first-line IS treatment alone or in combination with pulmonary vasodilators...
September 12, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/28872085/-manifestations-of-the-connective-tissue-associated-interstitial-lung-disease-under-high-resolution-computed-tomography
#19
Hailan Li, Zeng Xiong, Jinkang Liu, Yisha Li, Bin Zhou
To analyze the features of the connective tissue associated interstitial lung disease (CTD-ILD) by high resolution computed tomography (HRCT).
 Methods: A total of 127 patients with CTD-ILD, who were diagnosed by clinic laboratory examination and pathology in Xiangya Hospital of Central South University form September 2013 to September 2015, were enrolled for this study. Their lung features of HRCT imaging were retrospectively analyzed.
 Results: The classifications for 127 patients were as follows: 36 cases of rheumatoid arthritis (28...
August 28, 2017: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
https://www.readbyqxmd.com/read/28864939/intravenous-immunoglobulins-for-treatment-of-connective-tissue-diseases-in-dermatology
#20
REVIEW
Lyubomir A Dourmishev, Dimitrina V Guleva, Ljubka G Miteva
BACKGROUND: Connective tissue diseases are a heterogeneous group of autoimmune disorders affecting not only skin, but also various organs and systems. First-line treatment of connective tissue diseases is systemic steroids as monotherapy or combined with immunosuppressive drugs. Since intravenous immunoglobulins (IVIGs) have been found to be effective for various autoimmune dermatoses, their indications have expanded tremendously. OBJECTIVE: The aim this review article is to highlight the indications, effectiveness, and side effects of high doses immunoglobulins for treatment of patients with connective tissue diseases...
September 1, 2017: Wiener Medizinische Wochenschrift
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