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Mixed connective tissue disease

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https://www.readbyqxmd.com/read/29054902/chylous-ascites-in-a-patient-with-an-overlap-syndrome-a-surprising-response-to-rituximab
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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/28839093/imaging-the-emergence-and-natural-progression-of-spontaneous-autoimmune-diabetes
#12
James F Mohan, Rainer H Kohler, Jonathan A Hill, Ralph Weissleder, Diane Mathis, Christophe Benoist
Type 1 diabetes in the nonobese diabetic mouse stems from an infiltration of the pancreatic islets by a mixed population of immunocytes, which results in the impairment and eventual destruction of insulin-producing β-cells. Little is known about the dynamics of lymphocyte movement in the pancreas during disease progression. Using advanced intravital imaging approaches and newly created reporter mice (Flt3-BFP2, Mertk-GFP-DTR, Cd4-tdTomato, Cd8a-tdTomato), we show that the autoimmune process initiates first with a T cell infiltration into the islets, where they have restricted mobility but reside and are activated in apposition to CX3CR1(+) macrophages...
September 12, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28835902/the-microbiome-in-connective-tissue-diseases-and-vasculitides-an-updated-narrative-review
#13
REVIEW
Rossella Talotta, Fabiola Atzeni, Maria Chiara Ditto, Maria Chiara Gerardi, Piercarlo Sarzi-Puttini
OBJECTIVE: To provide a narrative review of the most recent data concerning the involvement of the microbiome in the pathogenesis of connective tissue diseases (CTDs) and vasculitides. METHODS: The PubMed database was searched for articles using combinations of words or terms that included systemic lupus erythematosus, systemic sclerosis, autoimmune myositis, Sjögren's syndrome, undifferentiated and mixed CTD, vasculitis, microbiota, microbiome, and dysbiosis. Papers from the reference lists of the articles and book chapters were reviewed, and relevant publications were identified...
2017: Journal of Immunology Research
https://www.readbyqxmd.com/read/28818881/selexipag-for-the-treatment-of-connective-tissue-disease-associated-pulmonary-arterial-hypertension
#14
Sean Gaine, Kelly Chin, Gerry Coghlan, Richard Channick, Lilla Di Scala, Nazzareno Galiè, Hossein-Ardeschir Ghofrani, Irene M Lang, Vallerie McLaughlin, Ralph Preiss, Lewis J Rubin, Gérald Simonneau, Olivier Sitbon, Victor F Tapson, Marius M Hoeper
Patients with connective tissue disease-associated pulmonary arterial hypertension (PAH-CTD) have a poor prognosis compared with other aetiologies. The underlying CTD can influence treatment response and outcomes. We characterised the GRIPHON study PAH-CTD subgroup and evaluated response to selexipag.Of 334 patients with PAH-CTD, PAH was associated with systemic sclerosis (PAH-SSc) in 170, systemic lupus erythematosus (PAH-SLE) in 82 and mixed CTD/CTD-other in 82. For the primary composite endpoint of morbidity/mortality, hazard ratios (HR) and 95% CI were calculated using Cox proportional hazard models...
August 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/28811075/regulatory-t-cell-deficient-scurfy-mice-exhibit-a-th2-m2-like-inflammatory-response-in-the-skin
#15
Stefanie Haeberle, Verena Raker, Jessica Haub, Yong O Kim, Shih-Yen Weng, Osman K Yilmaz, Alexander Enk, Kerstin Steinbrink, Detlef Schuppan, Eva N Hadaschik
BACKGROUND: Scurfy mice have a functional defect in regulatory T cells (Treg), which leads to lethal multi-organ inflammation. The missing Treg function results in uncontrolled autoimmune cellular and humoral inflammatory responses. We and others have previously shown that during the course of disease scurfy mice develop severe skin inflammation and autoantibodies including anti-nuclear autoantibodies (ANA). OBJECTIVE: Autoimmune skin inflammation and ANA are hallmarks for the diagnosis of autoimmune connective tissue diseases; therefore we analyzed scurfy mice for typical signs of these diseases...
September 2017: Journal of Dermatological Science
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
#16
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
#17
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
#18
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
#19
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/28687351/prevalence-of-anti-nt5c1a-antibodies-in-japanese-patients-with-autoimmune-rheumatic-diseases-in-comparison-with-other-patient-cohorts
#20
Yoshinao Muro, Hirotaka Nakanishi, Masahisa Katsuno, Michihiro Kono, Masashi Akiyama
BACKGROUND: Sporadic inclusion body myositis (sIBM) is usually classified as an idiopathic inflammatory myopathies. Although the diagnosis of sIBM is sometimes challenging, recent studies have shown that the autoantibodies against cytosolic 5'-nucleotidase 1A (NT5C1A) are the possible diagnostic biomarker for sIBM. Few reports have shown the frequencies of anti-NT5C1A antibodies in systemic autoimmune rheumatic diseases (SARDs) using large cohorts of SARDs. METHODS: Serum samples obtained from 314 patients including dermatomyositis (DM) (n=144), systemic lupus erythematosus (SLE) (n=50), systemic sclerosis (SSc) (n=50), Sjögren's syndrome (SS) (n=50), polymyositis (PM) (n=10) and mixed connective tissue disease (n=10), and healthy controls (n=42) in addition to 10 patients with typical sIBM were analysed for the presence of autoantibodies using full-length recombinant NT5C1A ELISA...
July 4, 2017: Clinica Chimica Acta; International Journal of Clinical Chemistry
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