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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
#1
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/28904690/-mixed-connective-tissue-disease-prevalence-and-clinical-characteristics-in-african-black-study-of-7-cases-in-gabon-and-review-of-the-literature
#2
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
#3
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/28864939/intravenous-immunoglobulins-for-treatment-of-connective-tissue-diseases-in-dermatology
#4
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
https://www.readbyqxmd.com/read/28864092/facts-and-controversies-in-mixed-connective-tissue-disease
#5
REVIEW
Julia Martínez-Barrio, Lara Valor, F Javier López-Longo
Mixed connective tissue disease (MCTD) is a systemic autoimmune rheumatic disease (SARD) characterised by the combination of clinical manifestations of systemic lupus erythematosus (SLE), cutaneous systemic sclerosis (SSc) and polymyositis-dermatomyositis, in the presence of elevated titers of anti-U1-RNP antibodies. Main symptoms of the disease are polyarthritis, hand oedema, Raynaud's phenomenon, sclerodactyly, myositis and oesophageal hypomobility. Although widely discussed, most authors today accept MCTD as an independent entity...
August 29, 2017: Medicina Clínica
https://www.readbyqxmd.com/read/28768981/protein-losing-gastroenteropathy-related-to-mixed-connective-tissue-disease-a-case-report-of-a-successful-outcome-and-literature-review
#6
Yuya Kobayashi, Yasuhiro Shimojima, Yasufumi Kondo, Ryota Takamatsu, Daigo Miyazaki, Dai Kishida, Yoshiki Sekijima, Shu-Ichi Ikeda
We herein report the case of a 44-year-old woman who developed protein-losing gastroenteropathy (PLGE) with hypoalbuminemia as the first manifestation of mixed connective tissue disease (MCTD). Albumin leakage from the stomach and intestinal tract was demonstrated by (99m)Tc-labeled human serum albumin scintigraphy. The patient's response to prednisolone therapy was insufficient; therefore, additional cyclosporin A (CsA) treatment was administered, and clinical remission was achieved. We concluded that although PLGE is a rare complication of MCTD, it may manifest as an initial clinical episode of MCTD...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28766758/comparisons-of-neutrophil-monocyte-eosinophil-and-basophil-lymphocyte-ratios-among-various-systemic-autoimmune-rheumatic-diseases
#7
COMPARATIVE STUDY
Zaixing Yang, Zhiyu Zhang, Feng Lin, Yingpeng Ren, Donghong Liu, Renqian Zhong, Yan Liang
This study was aimed to evaluate levels of neutrophil- (NLR), monocyte- (MLR), eosinophil- (ELR), and basophil-lymphocyte ratio (BLR) and their association with inflammatory markers in systemic autoimmune rheumatic diseases (SARDs). A total of 1139 SARD patients and 170 healthy individuals were enrolled. Clinical and laboratory data were extracted. NLR and MLR were significantly increased, but BLR decreased in most SARD patients (p < 0.05). ELR were significantly decreased in systemic lupus erythematosus (SLE) patients, but increased in those with other SARDs (p < 0...
October 2017: APMIS: Acta Pathologica, Microbiologica, et Immunologica Scandinavica
https://www.readbyqxmd.com/read/28700492/an-unusual-association-between-hemophagocytic-lymphohistiocytosis-mixed-connective-tissue-disease-and-autoimmune-hemolytic-anemia-a-case-report
#8
Amar H Kelkar, Anushi A Shah, Sherri L Yong, Zohair Ahmed
RATIONALE: In the adult patient, hemophagocytic lymphohistiocytosis (HLH) is uncommon and frequently difficult to diagnose due to its nonspecific presentation and numerous complications. PATIENT CONCERNS: Herein, we present the case of a 25-year-old female who initially presented for evaluation of persistent fevers and fatigue. She was found to have splenomegaly, generalized lymphadenopathy, pancytopenia, and acute hepatic failure. DIAGNOSES, INTERVENTIONS, AND OUTCOMES: Her course was further complicated by the development of nephrotic syndrome and autoimmune hemolytic anemia (AIHA)...
July 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28690947/autoamputation-and-polyneuropathy-in-mixed-connective-tissue-disorder-a-case-report
#9
Syeda Naqvi, Vikash Talib, Razia Aijaz, Zeeshan Ali, Shehroz Bashir, Syed Masroor Ahmad, Shabnam Naveed
Mixed connective tissue disorder (MCTD) is a multisystem disease with overlapping features of other autoimmune diseases, such as systemic lupus erythematosus (SLE), myositis, rheumatoid arthritis, and scleroderma. MCTD presents with a distinctive antibody in serum known as U1-ribonucleoprotein (RNP). MCTD is quite rare as compared to other connective tissue disorders like SLE, systemic sclerosis, dermatomyositis, and polymyositis. We describe a case of MCTD in a young Asian female of 30 year old. This case highlights rare co-existence of polyneuropathy and autoamputation in MCTD disorder...
June 5, 2017: Curēus
https://www.readbyqxmd.com/read/28592713/pulmonary-limited-granulomatosis-with-polyangiitis-coexisting-with-mixed-connective-tissue-disease
#10
Masaru Ando, Akihiko Goto, Mari Yamasue, Yuko Usagawa, Hiroaki Oka, Takehiko Shigenaga, Jun-Ichi Kadota
Granulomatosis with polyangiitis (GPA) is a systemic disease characterized by necrotizing, granulomatous vasculitis of the upper and lower respiratory tracts and glomerulonephritis, and is classified as a classical or limited form. The classical form of GPA demonstrates the involvement of the upper respiratory tract, sinuses, lungs and kidneys, whereas the limited form is characterized by the lack of the renal involvement with female predominance. On the other hand, mixed connective tissue disease (MCTD) shows the clinical and laboratorial features of systemic lupus erythematosus, systemic sclerosis and polymyositis, along with high titers of anti-ribonucleoprotein antibodies and is characterized by good response to corticosteroid therapy and favorable prognosis...
June 2017: Tohoku Journal of Experimental Medicine
https://www.readbyqxmd.com/read/28400867/evaluation-of-autoantibodies-in-patients-with-primary-and-secondary-sjogren-s-syndrome
#11
Ellen De Langhe, Xavier Bossuyt, Long Shen, Kishore Malyavantham, Julian L Ambrus, Lakshmanan Suresh
BACKGROUND: Antibodies to salivary gland protein 1 (SP1), carbonic anhydrase 6 (CA6) and parotid secretory protein (PSP) were discovered in an animal model of Sjogren's syndrome (SS). Their expression was noted in patients with SS, especially those with lower focus scores on lip biopsies and those with early disease lacking antibodies to Ro and La. OBJECTIVE: The current studies evaluated these autoantibodies in patients with long-standing SS expressing high levels of anti-Ro antibodies and in patients with Sjogren's syndrome secondary to systemic lupus erythematosus (SLE), systemic sclerosis (SSc) and mixed connective tissue disease (MCTD)...
2017: Open Rheumatology Journal
https://www.readbyqxmd.com/read/28379478/progression-and-mortality-of-interstitial-lung-disease-in-mixed-connective-tissue-disease-a-long-term-observational-nationwide-cohort-study
#12
Silje Reiseter, Ragnar Gunnarsson, Trond Mogens Aaløkken, May Brit Lund, Georg Mynarek, Jukka Corander, Joanna Haydon, Øyvind Molberg
Objectives.: To assess the prevalence, extent, progression, functional impact and mortality of interstitial lung disease (ILD) in a nationwide unselected MCTD cohort. Methods.: The study cohort included patients with high-resolution CT lung scans available at baseline (n = 135) and at follow-up (n = 119). The extent of disease was expressed as percentage of total lung volume (TLV). Results.: ILD was present in 41% of MCTD patients at follow-up...
March 29, 2017: Rheumatology
https://www.readbyqxmd.com/read/28275485/serum-kl-6-and-surfactant-protein-d-as-monitoring-and-predictive-markers-of-interstitial-lung-disease-in-patients-with-systemic-sclerosis-and-mixed-connective-tissue-disease
#13
Hideaki Yamakawa, Eri Hagiwara, Hideya Kitamura, Yumie Yamanaka, Satoshi Ikeda, Akimasa Sekine, Tomohisa Baba, Koji Okudela, Tae Iwasawa, Tamiko Takemura, Kazuyoshi Kuwano, Takashi Ogura
BACKGROUND: Interstitial lung disease (ILD) is frequent complication of systemic sclerosis (SSc) and mixed connective tissue disease (MCTD). The disease is heterogeneous, and its outcome is unpredictable. Some patients have severe and progressive deterioration of ILD, which is the leading cause of mortality. We aimed to determine whether serum levels of Krebs von den Lungen-6 (KL-6) and surfactant protein-D (SP-D) correlate with SSc/MCTD-associated ILD activity. METHODS: We retrospectively analyzed the medical records of 40 patients with SSc/MCTD-associated ILD: 29 patients with SSc and 11 patients with MCTD...
February 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28255922/the-clinical-and-pathological-characteristics-of-nephropathies-in-connective-tissue-diseases-in-the-japan-renal-biopsy-registry-j-rbr
#14
Kazunobu Ichikawa, Tsuneo Konta, Hiroshi Sato, Yoshihiko Ueda, Hitoshi Yokoyama
BACKGROUND: In connective tissue diseases, a wide variety of glomerular, tubulointerstitial, and vascular lesions of the kidney are observed. Nonetheless, recent information is limited regarding renal lesions in connective tissue diseases, except in systemic lupus erythematosus (SLE). METHODS: In this study, we used a nationwide database of biopsy-confirmed renal diseases in Japan (J-RBR) (UMIN000000618). In total, 20,523 registered patients underwent biopsy between 2007 and 2013; from 110 patients with connective tissue diseases except SLE, we extracted data regarding the clinico-pathological characteristics of the renal biopsy...
March 2, 2017: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/28248800/mixed-connective-tissue-disease-and-epitope-spreading-an-historical-cohort-study
#15
Laura Escolà-Vergé, Iago Pinal-Fernandez, Andreu Fernandez-Codina, Eduardo L Callejas-Moraga, Juan Espinosa, Ana Marin, Moises Labrador-Horrillo, Albert Selva-O'Callaghan
OBJECTIVES: Mixed connective tissue disease (MCTD) is characterized by the presence of anti-U1-snRNP autoantibodies and a variable set of associated clinical features. Some MCTD patients test positive over time to autoantibodies against Sm, proteins spatially related with U1-snRNP. This situation has been attributed to expanding of the autoimmune response by a phenomenon known as epitope spreading. Our aim was to study the frequency of this phenomenon in MCTD patients and the specific clinical features of those with epitope spreading...
April 2017: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/28202869/treatment-of-vasodilator-resistant-mixed-connective-tissue-disease-associated-pulmonary-arterial-hypertension-with-glucocorticoid-and-cyclophosphamide
#16
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
#17
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)]...
June 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
#18
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
#19
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
#20
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
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