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https://www.readbyqxmd.com/read/28202869/treatment-of-vasodilator-resistant-mixed-connective-tissue-disease-associated-pulmonary-arterial-hypertension-with-glucocorticoid-and-cyclophosphamide
#1
Eri Sugawara, Masaru Kato, Ryo Hisada, Kenji Oku, Toshiyuki Bohgaki, Tetsuya Horita, Shinsuke Yasuda, Tatsuya Atsumi
Pulmonary arterial hypertension (PAH) associated with systemic lupus erythematosus (SLE) or mixed connective tissue disease (MTCD), in contrast to other types of PAH, may respond to immunosuppressive therapy. Most PAH cases with an immunosuppressant response were in the early stages of the disease (WHO functional class III or less). The present case was a 34-year-old woman with MCTD-associated PAH (WHO functional class IV) who was resistant to a combination of three vasodilators. Afterwards, she was treated with glucocorticoid and cyclophosphamide...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28185705/cardiovascular-magnetic-resonance-imaging-pattern-at-the-time-of-diagnosis-of-treatment-na%C3%A3-ve-patients-with-connective-tissue-diseases
#2
Sophie Mavrogeni, George Markousis-Mavrogenis, Loukia Koutsogeorgopoulou, Theodoros Dimitroulas, Konstantinos Bratis, George D Kitas, Petros Sfikakis, Maria Tektonidou, Georgia Karabela, Efthymios Stavropoulos, Gikas Katsifis, Kyriaki A Boki, Anastasia Kitsiou, Vasiliki Filaditaki, Elias Gialafos, Sotiris Plastiras, Vasiliki Vartela, Genovefa Kolovou
BACKGROUND-AIM: Cardiac involvement at diagnosis of connective tissue disease (CTD) has been described by echocardiography. We hypothesized that cardio-vascular magnetic resonance (CMR) detects occult lesions at CTD diagnosis. PATIENTS-METHODS: CMR was performed early after diagnosis in 78 treatment-naïve CTDs (aged 43±11, 59F/19M) without cardiac involvement [5 Takayasu arteritis (TA), 4 Churg Strauss syndrome (CSS), 5 Wegener granulomatosis (WG), 16 systemic lupus erythematosus (SLE), 12 rheumatoid arthritis (RA), 8 mixed connective tissue diseases (MCTD), 12 ankylosing spondylitis (AS), 3 polymyalgia rheumatica (PMR), 8 systemic sclerosis (SSc) and 5 dermatomyositis (DM)]...
February 1, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28053878/microparticle-subpopulations-are-potential-markers-of-disease-progression-and-vascular-dysfunction-across-a-spectrum-of-connective-tissue-disease
#3
E M McCarthy, D Moreno-Martinez, F L Wilkinson, N J McHugh, I N Bruce, J D Pauling, M Y Alexander, B Parker
OBJECTIVE: Microparticles (MPs) are membrane-bound vesicles derived from vascular and intravascular cells such as endothelial cells (EMPs) and platelets (PMPs). We investigated EMP and PMP numbers across a spectrum of autoimmune rheumatic diseases (AIRDs) with the aim of comparing the levels of, and relationship between, EMPs and PMPs. METHODS: Patients with Systemic Lupus Erythematosus (SLE) (n = 24), Systemic Sclerosis (SSc) (n = 24), Primary Raynauds Phenomenon (RP) (n = 17) and "other CTD" (n = 15) (Primary Sjogrens Syndrome, UCTD or MCTD) as well as 15 healthy controls were recruited...
June 2017: BBA Clinical
https://www.readbyqxmd.com/read/27986694/adhesive-arachnoiditis-in-mixed-connective-tissue-disease-a-rare-neurological-manifestation
#4
Maria Usman Khan, James Anthony Joseph Devlin, Alexander Fraser
The overall incidence of neurological manifestations is relatively low among patients with mixed connective tissue disease (MCTD). We recently encountered a case of autoimmune adhesive arachnoiditis in a young woman with 7 years history of MCTD who presented with severe back pain and myeloradiculopathic symptoms of lower limbs. To the best of our knowledge, adhesive arachnoiditis in an MCTD patient has never been previously reported. We report here this rare case, with the clinical picture and supportive ancillary data, including serology, cerebral spinal fluid analysis, electrophysiological evaluation and spinal neuroimaging, that is, MRI and CT (CT scan) of thoracic and lumbar spine...
December 16, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27900357/datasets-of-yy1-expression-in-rheumatoid-arthritis-patients
#5
Jinpiao Lin, Yujue He, Junmin Chen, Zhiyong Zeng, Bin Yang, Qishui Ou
The data presented in this article are related to the research article entitled "A critical role of transcription factor YY1 in rheumatoid arthritis by regulation of interleukin-6" (J. Lin, Y. He, J. Chen, Z. Zeng, B. Yang, Q. Ou, 2016) [1]. The article describes YY1 overexpression is specific for RA, but not for SLE, SS, DM or MCTD. In early RA, YY1 expression is also increased. In asymptomatic subjects with RF or ACPA positive who have high risk for developing RA, the YY1 expression is not increased obviously...
December 2016: Data in Brief
https://www.readbyqxmd.com/read/27870974/myocardial-perfusion-in-peripheral-raynaud-s-phenomenon-evaluation-using-stress-cardiovascular-magnetic-resonance
#6
Sophie Mavrogeni, Konstantinos Bratis, Loukia Koutsogeorgopoulou, Georgia Karabela, Efthymios Savropoulos, Gikas Katsifis, John Raftakis, George Markousis-Mavrogenis, Genovefa Kolovou
BACKGROUND: Peripheral Raynaud's phenomenon (RP) is either primary (PRP), without any coexisting disease or secondary (SRP), due to connective tissue diseases (CTD). We hypothesized that adenosine stress cardiovascular magnetic resonance (CMR) can assess myocardial perfusion in a population of PRP and SRP. PATIENTS-METHODS: Twenty CTDs, aged 30.6±7.5yrs., 16F/4M, including 9 systemic sclerosis (SSc), 4 systemic lupus erythematosus (SLE), 3 mixed connective tissue disease (MCTD), 2 polymyositis (PM) and 2 rheumatoid arthritis (RA), with SRP, under treatment with calcium blockers, were evaluated by stress CMR and compared with age-sex matched PRP and controls...
February 1, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/27788293/prevalence-of-mixed-connective-tissue-disease-in-a-population-based-registry-of-american-indian-alaska-native-people-in-2007
#7
Elizabeth D Ferucci, Janet M Johnston, Caroline Gordon, Charles G Helmick, S Sam Lim
OBJECTIVE: The objective of this surveillance project was to determine the prevalence of mixed connective tissue disease (MCTD) in 2007 in the Indian Health Service (IHS) active clinical population from 3 regions of the United States. METHODS: The IHS Lupus Registry was designed to identify possible MCTD cases in addition to lupus. The population denominator for this report includes American Indian or Alaska Native adults within the IHS active clinical population in 2007, residing in select communities in 3 regions of the US...
October 27, 2016: Arthritis Care & Research
https://www.readbyqxmd.com/read/27749242/effects-of-rituximab-in-connective-tissue-disorders-related-interstitial-lung-disease
#8
MULTICENTER STUDY
Gemma Lepri, Jerome Avouac, Paolo Airò, Francisco Anguita Santos, Silvia Bellando-Randone, Jelena Blagojevic, Francisco Garcia Hernàndez, Jose Antonio Gonzalez Nieto, Serena Guiducci, Suzana Jordan, Vidya Limaye, Britta Maurer, Albert Selva-O'Callaghan, Valeria Riccieri, Oliver Distler, Marco Matucci-Cerinic, Yannick Allanore
OBJECTIVES: Interstitial lung disease (ILD) is a key prognostic factor in connective tissue disorders (CTDs). The aim of our study was to assess the changes in pulmonary functional tests (PFTs) in various CTDs, including anti-synthetase syndrome (SYN), systemic sclerosis (SSc) and mixed connective tissue disorder (MCTD), following the use of rituximab therapy. METHODS: A multicentre retrospective analysis of patients with ILD secondary to SYN (n=15), MCTD (n=6) and SSc (n=23)...
September 2016: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/27738519/tocilizumab-in-the-treatment-of-mixed-connective-tissue-disease-and-overlap-syndrome-in-children
#9
Natalia Cabrera, Agnes Duquesne, Marine Desjonquères, Jean-Paul Larbre, Jean-Christophe Lega, Nicole Fabien, Alexandre Belot
Arthritis is one of the main manifestations of mixed connective tissue disease (MCTD) and overlap syndrome in children and can be responsible for functional disability. We report on 2 children with arthritis that were dramatically improved by a treatment with interleukin-6 (IL-6) blockers in the context of connective tissue disease. However, in both cases, other systemic autoimmune symptoms were not modified by the treatment and autoantibodies tend to increase, suggesting a differential effect of IL-6 inhibition on articular inflammation and systemic autoimmunity...
2016: RMD Open
https://www.readbyqxmd.com/read/27618831/clinical-association-of-mixed-connective-tissue-disease-and-granulomatosis-with-polyangiitis-a-case-report-and-systematic-screening-of-anti-u1rnp-and-anti-pr3-auto-antibody-double-positivity-in-ten-european-hospitals
#10
Amandine Tubery, Françoise Fortenfant, Bernard Combe, Isabelle Abreu, Xavier Bossuyt, Pascale Chretien, Sophie Desplat-Jégo, Nicole Fabien, Sophie Hue, Catherine Johanet, Daniela Lakomy, Thierry Vincent, Claire I Daïen
We report here the case of a 50-years-old man treated for mixed connective tissue disease (MCTD) positive for anti-U1 ribonucleoprotein (U1RNP) antibodies who secondarily developed a granulomatosis with polyangiitis (GPA) associated with anti-proteinase 3 anti-neutrophil cytoplasmic antibodies (PR3-ANCA). We then evaluated the frequency of the association between anti-U1RNP and anti-PR3-ANCA antibodies by a systematic retrospective study in ten European hospitals. Overall, out of 11,921 samples analyzed for both auto-antibodies, 18 cases of anti-U1RNP and anti-PR3-ANCA double positivity were found and only one patient presented with both MCTD and GPA symptoms...
September 12, 2016: Immunologic Research
https://www.readbyqxmd.com/read/27586323/-intractable-myasthenia-gravis-accompanied-with-thymoma-report-of-a-case
#11
Akira Naomi, Yoshinori Oyamatsu, Kunio Narita, Masato Nakayama, Shoki Maeda
A 46-year-old female visited a hospital due to pelvic inflammatory disease (PID) and then her chest computed tomography revealed an abnormal shadow in the upper mediastinum. Four months later,she complained muscle weakness with her limbs, dysphagia, and ptosis of her eyelids. Total thymectomy was performed through a median sternotomy for mass lesion, which was pathologically proven to be type B1 thymoma. Postoperative myasthenia gravis (MG) crisis, which led to respiratory failure requiring intubation and mechanical ventilation, developed and laboratory tests showed elevated serum anti-AChR Ab(130 nmol/l), antinuclear antibody( ×640 serum dilution, speckled pattern) and anti-RNP Ab(129...
September 2016: Kyobu Geka. the Japanese Journal of Thoracic Surgery
https://www.readbyqxmd.com/read/27567293/pathways-linked-to-internuclear-ophthalmoplegia-on-diffusion-tensor-imaging-in-a-case-with-midbrain-infarction
#12
Haruka Takeshige, Yuji Ueno, Koji Kamagata, Fuyuko Sasaki, Kazuo Yamashiro, Ryota Tanaka, Shigeki Aoki, Nobutaka Hattori
BACKGROUND: Midbrain infarction shows diverse patterns of ophthalmoplegia; however, the association of ophthalmoplegia with a precise microanatomy has not been fully studied. Here, we report a patient with characteristic ophthalmoplegia and explore the associated pathologic fiber tracts using diffusion-tensor imaging (DTI). METHODS: A 21-year-old woman with an 11-year history of mixed connective tissue disease (MCTD) abruptly developed bilateral internuclear ophthalmoplegia (INO) with upward gaze and convergence palsies...
November 2016: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/27504208/pauci-immune-crescentic-glomerulonephritis-in-connective-tissue-disease
#13
Supraja Yeturi, Mary Cronin, Adam Robin, Campbell Lorna, Ann K Rosenthal
Pauci-immune crescentic glomerulonephritis is commonly seen in ANCA-associated vasculitis but it is rarely seen during the course of other connective tissue diseases like lupus or Sjogren's syndrome or MCTD. We report 3 cases of pauci-immune crescentic glomerulonephritis in patients with connective tissue disease other than vasculitis. We reviewed literature and made summary of previously reported cases of this rare entity. Clinical and laboratory features of these patients varied widely, but most of patients have met criteria for lupus...
2016: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/27453743/esophageal-disorders-in-mixed-connective-tissue-diseases
#14
A E Nica, L M Alexa, A O Ionescu, O Andronic, D N Păduraru
Extra Musculoskeletal manifestations are a distinct clinical entity that refers to a combination of clinical features, which are found in multiple rheumatic diseases. Besides the standard manifestations, other organs can be damaged such as the vascular system, skin, gastrointestinal tract, musculoskeletal system, cardiopulmonary system, hematologic system, kidneys, and the central nervous system. Among the gastrointestinal MCTD symptoms, the most frequent are the esophageal ones. Treatment of patients with MCTD must be performed by both medical and surgical multidisciplinary teams in order to provide a management suitable for the patients' needs...
April 2016: Journal of Medicine and Life
https://www.readbyqxmd.com/read/27436003/mixed-connective-tissue-disease-enigma-variations
#15
REVIEW
Natalia C O Ciang, Nídia Pereira, David A Isenberg
In 1972, Sharp et al. described a new autoimmune rheumatic disease that they called MCTD, characterized by overlapping features of SSc, SLE, PM/DM, high levels of anti-U1snRNP and low steroid requirements with good prognosis. MCTD was proposed as a distinct disease. However, soon after the original description, questions about the existence of such a syndrome as well as disputes over the features initially described began to surface. The conundrum of whether MCTD is a distinct disease entity remains controversial...
July 19, 2016: Rheumatology
https://www.readbyqxmd.com/read/27421219/mixed-connective-tissue-disease
#16
REVIEW
Ragnar Gunnarsson, Siri Opsahl Hetlevik, Vibke Lilleby, Øyvind Molberg
The concept of mixed connective tissue disease (MCTD) as a separate connective tissue disease (CTD) has persisted for more than four decades. High titers of antibodies targeting the U1 small nuclear ribonucleoprotein particle (U1 snRNP) in peripheral blood are a sine qua non for the diagnosis of MCTD, in addition to distinct clinical features including Raynaud's phenomenon (RP), "puffy hands," arthritis, myositis, pleuritis, pericarditis, interstitial lung disease (ILD), and pulmonary hypertension (PH). Recently, population-based epidemiology data from Norway estimated the point prevalence of adult-onset MCTD to be 3...
February 2016: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/27353506/can-sle-classification-rules-be-effectively-applied-to-diagnose-unclear-sle-cases
#17
A Mesa, M Fernandez, W Wu, G Narasimhan, E L Greidinger, D K Mills
OBJECTIVE: The objective of this paper is to develop novel classification criteria to distinguish between unclear systemic lupus erythematosus (SLE) and mixed connective tissue disease (MCTD) cases. METHODS: A total of 205 variables from 111 SLE and 55 MCTD patients were evaluated to uncover unique molecular and clinical markers for each disease. Binomial logistic regressions (BLRs) were performed on currently used SLE and MCTD classification criteria sets to obtain six reduced models with power to discriminate between unclear SLE and MCTD patients that were confirmed by receiving operating characteristic (ROC) curve...
June 27, 2016: Lupus
https://www.readbyqxmd.com/read/27328803/prevalence-of-systemic-autoimmune-rheumatic-diseases-and-clinical-significance-of-ana-profile-data-from-a-tertiary-hospital-in-shanghai-china
#18
Zaixing Yang, Yingpeng Ren, Donghong Liu, Feng Lin, Yan Liang
It is necessary and useful to explore prevalence of various systemic autoimmune rheumatic diseases (SARDs) in patients with suspicion of having SARDs and to characterize antinuclear antibodies (ANA) profile for identifying different populations (SARDs and non-SARDs). A total of 5024 consecutive patients with available medical records were investigated, whose sera had been tested for ANA profile, including ANA, anti-dsDNA and anti-extractable nuclear antigen (ENA) antibodies, between 31 January 2012 and 26 March 2014...
September 2016: APMIS: Acta Pathologica, Microbiologica, et Immunologica Scandinavica
https://www.readbyqxmd.com/read/27311277/-evaluation-of-a-computer-aided-microscope-system-and-its-anti-nuclear-antibody-test-kit-for-indirect-immunofluorescence-assay
#19
Nobuhide Hayashi, Jun Saegusa, Kenichi Uto, Chinami Oyabu, Toshiharu Saito, Itsuko Sato, Seiji Kawano, Shunichi Kumagai
Antinuclear antibody (ANA) testing is indispensable for diagnosing and understanding clinical conditions of autoimmune diseases. The indirect immunofluorescence assay (IFA) is the gold standard for ANA screening, and it can detect more than 100 different antibodies, such as anti-PCNA as well as anti-cytoplasmic antibodies. However, complicated procedures of conventional IFA and visual interpretation require highly skilled laboratory staff. This study evaluates the capability, characteristics, and applicability of the recently developed ANA detection system (EUROPattern Cosmic IFA System, EPA) using HEp20-10 cells and the automated pattern recognition microscope...
February 2016: Rinsho Byori. the Japanese Journal of Clinical Pathology
https://www.readbyqxmd.com/read/27252266/clinical-and-serological-associations-of-autoantibodies-to-the-ku70-ku80-heterodimer-determined-by-a-novel-chemiluminescent-immunoassay
#20
M Mahler, A Swart, J Wu, M Szmyrka-Kaczmarek, J-L Senécal, Y Troyanov, J G Hanly, M J Fritzler
BACKGROUND: Autoantibodies targeting Ku, an abundant nuclear protein with DNA helicase activity, have been reported in patients with systemic autoimmune rheumatic diseases. Little is known about the clinical associations of anti-Ku antibodies, especially when novel diagnostic technologies are used. The objective of the present study was to analyse the prevalence of anti-Ku antibodies in different medical conditions using a novel chemiluminescent immunoassay. PATIENTS AND METHODS: Serum samples from adult patients with systemic lupus erythematosus (SLE, n=305), systemic sclerosis (SSc, n=70) and autoimmune myositis patients (AIM, n=109) were the primary focus of the study...
July 2016: Lupus
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