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mixed connective tissue guidelines

Andy Goldberg, Katrina Mitchell, Julian Soans, Louise Kim, Razi Zaidi
BACKGROUND: The management of articular cartilage defects presents many clinical challenges due to its avascular, aneural and alymphatic nature. Bone marrow stimulation techniques, such as microfracture, are the most frequently used method in clinical practice however the resulting mixed fibrocartilage tissue which is inferior to native hyaline cartilage. Other methods have shown promise but are far from perfect. There is an unmet need and growing interest in regenerative medicine and tissue engineering to improve the outcome for patients requiring cartilage repair...
March 9, 2017: Journal of Orthopaedic Surgery and Research
Ramya Aithala, Anoop G Alex, Debashish Danda
Pulmonary hypertension (PH) is a relatively commoner complication of systemic sclerosis (SSc) with estimated prevalence ranging between 8% and 12% as compared to much lower figures in other connective tissue diseases (CTD). It is a major cause of morbidity and mortality in CTDs. PH is classified into five major groups. CTD-associated PH belongs to group 1 PH, also known as pulmonary arterial hypertension (PAH). Around 30% of scleroderma-related deaths are due to PAH. Underlying pathogenesis is related to pulmonary vasculopathy involving small vessels...
January 2017: International Journal of Rheumatic Diseases
Yasushi Kawaguchi
No abstract text is available yet for this article.
October 10, 2014: Nihon Naika Gakkai Zasshi. the Journal of the Japanese Society of Internal Medicine
Atsushi Sugiura, Nobusada Funabashi, Koya Ozawa, Yoshio Kobayashi
PURPOSE: We investigated the relationship of left ventricular (LV) diastolic dysfunction and LV mass index (LVMI) against pulmonary hypertension (PH) in systemic autoimmune disease (SAD). METHODS: A total of 84 SAD patients (68 females; 53±17years; systemic lupus erythematosus, 27%; scleroderma, 17%; vasculitis, 16%; mixed connective tissue disease, 13% and polymyositis/dermatomyositis complex, 10%) without significant pericardial effusion (PE) on TTE (Vivid E9, GE) were analyzed...
October 1, 2016: International Journal of Cardiology
Margaret von Mehren, R Lor Randall, Robert S Benjamin, Sarah Boles, Marilyn M Bui, Ernest U Conrad, Kristen N Ganjoo, Suzanne George, Ricardo J Gonzalez, Martin J Heslin, John M Kane, Henry Koon, Joel Mayerson, Martin McCarter, Sean V McGarry, Christian Meyer, Richard J O'Donnell, Alberto S Pappo, I Benjamin Paz, Ivy A Petersen, John D Pfeifer, Richard F Riedel, Scott Schuetze, Karen D Schupak, Herbert S Schwartz, William D Tap, Jeffrey D Wayne, Mary Anne Bergman, Jillian Scavone
Soft tissue sarcomas (STS) are rare solid tumors of mesenchymal cell origin that display a heterogenous mix of clinical and pathologic characteristics. STS can develop from fat, muscle, nerves, blood vessels, and other connective tissues. The evaluation and treatment of patients with STS requires a multidisciplinary team with demonstrated expertise in the management of these tumors. The complete NCCN Guidelines for Soft Tissue Sarcoma (available at provide recommendations for the diagnosis, evaluation, and treatment of extremity/superficial trunk/head and neck STS, as well as intra-abdominal/retroperitoneal STS, gastrointestinal stromal tumor, desmoid tumors, and rhabdomyosarcoma...
June 2016: Journal of the National Comprehensive Cancer Network: JNCCN
Elisabeth Bendstrup, Toby M Maher, Effrosyni D Manali, Marlies Wijsenbeek
According to current international guidelines the idiopathic interstitial pneumonias (IIPs) are grouped into three categories; major, rare, and unclassifiable. Idiopathic pulmonary fibrosis (IPF) is one of the major IIPs and has been recognised as a distinct clinical entity since 2001. This has led to significant advances in our understanding and treatment of the disease and to the identification of new therapeutic targets. While multidisciplinary team assessment yields a definite diagnosis in many cases of interstitial lung disease (ILD), 15-25% of patients remain unclassifiable...
August 3, 2015: Sarcoidosis, Vasculitis, and Diffuse Lung Diseases: Official Journal of WASOG
Ganary Dabiri, Vincent Falanga
Connective tissue disorders (CTD), which are often also termed collagen vascular diseases, include a number of related inflammatory conditions. Some of these diseases include rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis (scleroderma), localized scleroderma (morphea variants localized to the skin), Sjogren's syndrome, dermatomyositis, polymyositis, and mixed connective tissue disease. In addition to the systemic manifestations of these diseases, there are a number of cutaneous features that make these conditions recognizable on physical exam...
November 2013: Journal of Tissue Viability
Ragnar Gunnarsson, Arne K Andreassen, Øyvind Molberg, Åse Stavland Lexberg, Kari Time, Alvilde Sofie Strand Dhainaut, Liv-Turid Bertelsen, Øyvind Palm, Karen Irgens, Andrea Becker-Merok, Jan Leidulf Nordeide, Villy Johnsen, Sonja Pedersen, Anne Prøven, Lamya Samir Noori Garabet, Torhild Garen, Trond Mogens Aaløkken, Inge-Margrethe Gilboe, Jan Tore Gran
OBJECTIVES: The aim of this study was to assess the overall prevalence of pulmonary hypertension (PH) in an unselected MCTD cohort and review the current knowledge with a systematic database search. METHODS: A nationwide multicentre cohort of 147 adult MCTD patients were initially screened for PH by echocardiography, high-resolution computed tomography (HRCT), pulmonary function tests and N-terminal pro-brain natriuretic peptide (NT-proBNP) and then followed up for a mean of 5...
July 2013: Rheumatology
Sevdalina Nikolova Lambova, Walter Hermann, Ulf Müller-Ladner
No guidelines for the application of qualitative and quantitative analysis of the capillaroscopic examination in the rheumatologic practice exist. The aims of the study were to compare qualitative and quantitative analysis of key capillaroscopic parameters in patients with common rheumatic diseases and to assess the reproducibility of the qualitative evaluation of the capillaroscopic parameters, performed by two different investigators. Two hundred capillaroscopic images from 93 patients with different rheumatic diseases were analysed quantitatively and qualitatively by two different investigators...
December 2012: Rheumatology International
Shunji Yoshida
Pulmonary hypertension (PH) was found to be the primary cause of death in mixed connective tissue disease (MCTD). This led to investigation of the prevalence of PH in other connective tissue diseases (CTD). In 1998, the Ministry of Health and Welfare's MCTD Research Committee revealed complication of PH diagnosed by physicians in 5.02% MCTD patients, 0.90% systemic lupus erythematosus patients, 2.64% systemic sclerosis patients, and 0.56% polymyositis/dermatomyositis patients. These results have been supported by a similar survey performed in North America...
December 2011: Allergology International: Official Journal of the Japanese Society of Allergology
Jadranka Sertić, Bosko Skorić, Jasna Lovrić, Tamara Bozina, Zeljko Reiner
Thorough control of risk factors is pivotal for cardiocerebrovascular diseases. As classic risk assessment accounts for only 50% of risk variability and due to the role of inflammatory processes in endothelial dysfunction and atherosclerotic plaque rupture, it is necessary to identify new biomarkers for risk prediction. In addition to the inflammatory marker high sensibility C-reactive protein (hs-CRP), lipoprotein associated phospholipase A2 (Lp-PLA2) is gaining increasing significance, since it is directly involved in the pathogenesis of atherosclerotic plaque progression...
October 2010: Acta Medica Croatica: C̆asopis Hravatske Akademije Medicinskih Znanosti
Annette F Jansson, Claudia Sengler, Jasmin Kuemmerle-Deschner, Bernd Gruhn, A Birgitta Kranz, Hartwig Lehmann, Daniela Kleinert, Lars Pape, Hermann J Girschick, Ivan Foeldvari, Dieter Haffner, Johannes P Haas, Dagmar Moebius, Dirk Foell, Joachim Peitz, Veit Grote
Data on B cell depletion therapy in severe autoimmune diseases in paediatric patients are very limited. We conducted a retrospective cohort study and recruited patients who were treated with rituximab (RTX) and followed up for at least 6 months through the German societies of paediatric rheumatology and nephrology. The aim was to describe the spectrum of autoimmune disorders for which RTX was used and to describe the applied therapeutic regimens, the observed efficacy, as well as potential immunological side effects...
January 2011: Clinical Rheumatology
Martin H Gastens, Kristin Goltry, Wolfgang Prohaska, Diethelm Tschöpe, Bernd Stratmann, Dirk Lammers, Stanley Kirana, Christian Götting, Knut Kleesiek
Ex vivo expansion is being used to increase the number of stem and progenitor cells for autologous cell therapy. Initiation of pivotal clinical trials testing the efficacy of these cells for tissue repair has been hampered by the challenge of assuring safe and high-quality cell production. A strategy is described here for clinical-scale expansion of bone marrow (BM)-derived stem cells within a mixed cell population in a completely closed process from cell collection through postculture processing using sterile connectable devices...
2007: Cell Transplantation
H-P Hauber, P Zabel
Pulmonary involvement is commonly seen in autoimmune disease. In this review the clinical presentation of pulmonary involvement in vasculitis, rheumatoid arthritis and connective tissue disease is presented. Clinical symptoms and diagnostic approach will be discussed.
August 2007: Deutsche Medizinische Wochenschrift
Piotr Krawiec, Bogdan Batko, Antoni Skura, Teresa Adamek-Guzik, Marta Cześnikiewicz-Guzik, Marcin Krzanowski, Iwona Rydz-Stryszowska, Natalia Ryszawa, Tomasz J Guzik
Sjögren's Syndrome (SS) is the second most common autoimmune disorder after rheumatoid arthritis (RA). It can be found as a lone condition (primary Sjögren's Syndrome) or may accompany other autoimmune rheumatic diseases (secondary Sjögren's Syndrome). Despite such frequent occurrence, accurate diagnosis of Sjögren Syndrome is difficult. These difficulties result from highly variable symptoms of SS as well as from common presence of other autoimmune disorders. In the following article authors present current knowledge concerning clinical symptoms, diagnostic methods and latest clinical guidelines on the diagnosis of SS...
2006: Przegla̧d Lekarski
B Ostendorf, M Cohnen, A Scherer
Guidelines and diagnostic algorithms for the introduction of diagnostic procedures for connective tissue diseases have not yet been established and standardized. Diagnosis is often based on the patients's typical history, clinical symptoms and specific laboratory profiles. The use of diagnostic imaging procedures is relevant for differential diagnosis, for the assessment of disease activity and organ involvement, for defining the prognosis and monitoring responses to therapy and side effects. Experience with these techniques and diagnostic procedures, their application, indications and analysis represent the platform for rational and optimal diagnosis as well of defined therapy stratification...
October 2006: Zeitschrift Für Rheumatologie
Uwe-Frithjof Haustein
Mixed connective tissue disease is a disease entity characterized by overlapping symptoms of lupus erythematosus (LE), systemic sclerosis (SSc), polymyositis/dermatomyositis (PM/DM) and rheumatoid arthritis (RA). Diagnostic criteria include high titers of antibodies against U1RNP as well as the presence of at least 3 of 5 of the following clinical features: edema of hands, synovitis, myositis, Raynaud phenomenon and acroscierosis. In terms of the pathogenesis, genetic as well as infectious (viral) factors appear to play a role...
February 2005: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
K Gensch, S Gudowius, T Niehues, A Kuhn
Connective tissue diseases are a heterogeneous group of chronic multisystem inflammatory disorders including systemic lupus erythematosus (SLE), progressive systemic sclerosis (PSS), dermato- (DM) and polymyositis (PM), mixed connective tissue disease (MCTD), and Sjögren's syndrome (SS). Patients can present with similar clinical features, particularly during the first onset of symptoms, which frequently makes the diagnosis of a specific disease difficult. The incidence of connective tissue diseases is much lower in children than adults; however, the clinical picture is more variable...
October 2005: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
T Schlossbauer, C Becker-Gaab, R Eibel
Collagen based vascular diseases with pulmonary involvement comprise rheumatoid arthritis, systemic lupus erythematosus, progressive systemic sclerosis, dermatomyositis and polymyositis, ankylosing spondylitis, Sjögren's syndrome, and mixed connective tissue diseases. The different characteristics of pulmonary involvement are described. In such circumstances, early recognition of lung involvement is of considerable significance and the relationship to the corresponding disease has to be made. Frequently unrecognised pulmonary involvement ends up as fibrosis with irreversible deficits in respiratory function...
July 2005: Der Radiologe
Ingrid Strusberg, Ana M Bertoli, Ruth C de Pizzolato, Gonzalo Fierro, Alberto M Strusberg
The objective of this study was to obtain post-marketing information about the use of infliximab in an ambulatory setting. We studied--retrospectively and prospectively--the case records of patients with rheumatoid arthritis (n=37), psoriatic arthritis (n=5), mixed connective tissue disease (n=1), and ankylosing spondylitis (n=2) who received infliximab (3 mg/kg) from August 2000 to January 2003. Descriptive values were given as percentage, mean or median, and standard deviation or interquartile range. Wilcoxon test was used for paired analysis of pre/post doses of corticosteroids, non-steroidal anti-inflammatory drugs, and methotrexate therapy...
2005: Medicina
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