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https://www.readbyqxmd.com/read/27926812/the-role-of-18f-fluorodeoxyglucose-positron-emission-tomography-in-the-assessment-of-disease-activity-of-adult-onset-still-s-disease
#1
Young-Sil An, Chang-Hee Suh, Ju-Yang Jung, Hundo Cho, Hyoun-Ah Kim
Background/Aims: 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) has been suggested as a reliable imaging technique for monitoring of disease activity in patients with adult-onset Still's disease (AOSD). Therefore, we investigated the clinical significance of 18F-FDG PET/CT in Korean AOSD patients. Methods: Thirteen AOSD patients were included in the study. The PET/CT images were evaluated with visual and semiquantitative method using standardized uptake values (SUVs)...
December 7, 2016: Korean Journal of Internal Medicine
https://www.readbyqxmd.com/read/27903264/adult-onset-still-s-disease-evaluation-of-prognostic-tools-and-validation-of-the-systemic-score-by-analysis-of-100-cases-from-three-centers
#2
Piero Ruscitti, Paola Cipriani, Francesco Masedu, Daniela Iacono, Francesco Ciccia, Vasiliki Liakouli, Giuliana Guggino, Francesco Carubbi, Onorina Berardicurti, Paola Di Benedetto, Marco Valenti, Giovanni Triolo, Gabriele Valentini, Roberto Giacomelli
BACKGROUND: Adult-onset Still's disease (AOSD) is rare inflammatory disease of unknown etiology that usually affects young adults. The more common clinical manifestations are spiking fevers, arthritis, evanescent rash, elevated liver enzymes, lymphadenopathy, hepatosplenomegaly, and serositis. The multi-visceral involvement of the disease and the different complications, such as macrophage activation syndrome, may strongly decrease the life expectancy of AOSD patients. METHODS: This study aimed to identify the positive and negative features correlated with the outcome of patients...
December 1, 2016: BMC Medicine
https://www.readbyqxmd.com/read/27888598/adult-onset-still-s-disease-with-different-antibodies-a-case-report-and-review-of-literature
#3
Maryam Mobini, Roya Ghasemian, Fatemeh Zameni
 Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology. There is not currently any specific serological markers for AOSD , and  diagnosis still relying on the exclusion of other likely diagnoses. Yamaguchi's criteria are used as a diagnostic criterion which contains negative serologic markers for other collagen vascular diseases including systemic lupus erythematosus and rheumatoid arthritis. Here we report a 28-year-old woman with arthralgia, fever, rash, leukocytosis, lymphadenopathy, sore throat, abnormal liver function and negative rheumatoid factor and ANA but  seropositive for anti-CCP, anti-dsDNA, and C-ANCA...
October 2016: Acta Medica Iranica
https://www.readbyqxmd.com/read/27886796/adult-onset-still-s-disease-advances-in-the-treatment
#4
REVIEW
Santos Castañeda, Ricardo Blanco, Miguel A González-Gay
Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder mainly characterized by persistent high spiking fevers, evanescent rash, and joint involvement. The pathogenesis of AOSD is only partially known, but pro-inflammatory cytokines such as tumor necrosis factor (TNF)-α, interleukin (IL)-1, IL-6, IL-18, and IFN-γ seem to play a major role in this disorder. AOSD is at the crossroad of auto-inflammatory syndromes and autoimmune diseases. It is diagnosed by exclusion to determine the presence of high serum ferritin levels, which is usually >1000 μg/L...
April 2016: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/27847518/adult-onset-still-s-disease-associated-with-mycoplasma-pneumoniae-infection-and-hemophagocytic-lymphohistiocytosis
#5
Abhishek Agnihotri, Allison Ruff, Lauren Gotterer, Addie Walker, Amy H McKenney, Andrei Brateanu
Adult Onset Still's Disease (AOSD) is a systemic inflammatory disorder that can be associated with hemophagocytic lymphohistiocytosis (HLH), a rare but potentially fatal disease of overactive histiocytes and lymphocytes. We present a unique case of AOSD complicated by Mycoplasma pneumonia infection and HLH. A 28-year-old female developed joint pains followed by a diffuse, erythematous, pruritic skin rash that quickly spread throughout the body. The patient deteriorated and developed fever, chills, cough, and dyspnea and had to be intubated...
2016: Case Reports in Medicine
https://www.readbyqxmd.com/read/27846765/adult-onset-still-s-disease-like-manifestation-accompanied-by-the-cancer-recurrence-after-long-term-resting-state
#6
Kazuhito Fukuoka, Ayako Miyamoto, Yuko Ozawa, Noriko Ikegaya, Tomohiro Maesono, Yoshinori Komagata, Shinya Kaname, Yoshihiro Arimura
A 72-year-old woman presented nine months ago with skin rash on her bilateral forearms, which was followed by intermittent high fever, and stiffness and swelling of her bilateral fingers. She was diagnosed with seronegative rheumatoid arthritis (RA). She had a past history of breast cancer and had undergone breast preservation surgery 13 years previously. During admission in our hospital, she developed high fever and leukocytosis with a relapsing skin rash, sore throat, polyarthralgia and increased levels of serum ALT/AST and ferritin, all of which fulfilled Yamaguchi's criteria for adult-onset Still's disease (AOSD)...
November 16, 2016: Modern Rheumatology
https://www.readbyqxmd.com/read/27843366/adult-onset-still-s-disease-current-challenges-and-future-prospects
#7
REVIEW
Mariam Siddiqui, Michael S Putman, Anisha B Dua
Adult-onset Still's disease (AOSD) - a multi-systemic inflammatory condition characterized by high fevers, polyarthritis, an evanescent rash, and pharyngitis - has been a challenging condition to diagnose expediently and treat effectively. Questions remain regarding the underlying pathophysiology and etiology of AOSD. Pathognomonic diagnostic tests and reliable biomarkers remain undiscovered. Over the past decade, important progress has been made. Diagnostic criteria employing glycosylated ferritin have improved specificity...
2016: Open Access Rheumatology: Research and Reviews
https://www.readbyqxmd.com/read/27761568/adult-onset-still-s-disease-with-dermatopathic-lymphadenopathy
#8
Ahmad Z Qureshi, Mohammad AlSheef, Waqas T Qureshi, Waseem Amjad
Adult onset Still's disease (AOSD) is a chronic inflammatory disorder involving multiple systems. The symptoms mimic those of lymphomas, therefore, the diagnosis of lymphoma needs to be excluded prior to establishing the diagnosis of AOSD. Another similar condition is dermatopathic lymphadenopathy (DL). In DL, the histopathological appearance of lymph node biopsy may also mimic AOSD. The DL is associated with several systemic pathologies, such as malignant lymphomas, and rarely AOSD. We present a case of a 43-year-old male presented with 3 months history of fatigue, fever, and lymphadenopathy...
November 2016: Saudi Medical Journal
https://www.readbyqxmd.com/read/27699261/anakinra-as-a-diagnostic-challenge-and-treatment-option-for-systemic-autoinflammatory-disorders-of-undefined-etiology
#9
Stephanie R Harrison, Dennis McGonagle, Sharmin Nizam, Stephen Jarrett, Jeroen van der Hilst, Michael F McDermott, Sinisa Savic
BACKGROUND: Some adult patients presenting with unexplained pyrexia, serositis, skin rashes, arthralgia, myalgia, and other symptoms commonly found in autoinflammatory disorders may not fit a specific diagnosis, either because their clinical phenotype is nondiagnostic or genetic tests are negative. We used the term undifferentiated systemic autoinflammatory disorder (uSAID) to describe such cases. Given that well-defined autoinflammatory diseases show responses to IL-1 blockade, we evaluated whether anakinra was useful for both diagnosing and treating uSAID patients...
May 5, 2016: JCI Insight
https://www.readbyqxmd.com/read/27688774/successful-tocilizumab-therapy-for-macrophage-activation-syndrome-associated-with-adult-onset-still-s-disease-a-case-based-review
#10
Eri Watanabe, Hitoshi Sugawara, Takeshi Yamashita, Akira Ishii, Aya Oda, Chihiro Terai
We report the case of a 71-year-old Japanese woman with adult-onset Still's disease (AOSD) in whom macrophage activation syndrome (MAS) developed despite therapy with oral high-dose prednisolone and intravenous methylprednisolone pulse therapy twice. She was successfully treated with tocilizumab (TCZ). Soon afterward, her fever ceased and high levels of both ferritin and C-reactive protein levels decreased. Her course was complicated by disseminated intravascular coagulation, cytomegalovirus infection, and Pneumocystis jirovecii pneumonia...
2016: Case Reports in Medicine
https://www.readbyqxmd.com/read/27616144/elevated-serum-levels-of-free-interleukin-18-in-adult-onset-still-s-disease
#11
Charlotte Girard, Jürgen Rech, Michael Brown, Danièle Allali, Pascale Roux-Lombard, François Spertini, Eduardo J Schiffrin, Georg Schett, Bernhard Manger, Sylvette Bas, Greg Del Val, Cem Gabay
OBJECTIVE: IL-18 is a pro-inflammatory cytokine of the IL-1 family that is naturally inhibited by IL-18 binding protein (IL-18BP). High levels of IL-18 have been described in the serum of adult-onset Still's disease (AOSD) patients, but only total IL-18 levels (including inactive IL-18 bound to IL-18BP) have been measured. With a specific immunoassay, we aimed to measure free IL-18 serum levels in AOSD patients and other rheumatic diseases. METHODS: An ELISA was developed to measure free IL-18...
September 10, 2016: Rheumatology
https://www.readbyqxmd.com/read/27603335/occasional-detection-of-thymic-epithelial-tumor-4-years-after-diagnosis-of-adult-onset-still-disease-a-challenging-case-report-and-immuno-oncological-considerations-coming-from-pertinent-literature-review
#12
Filippo Lococo, Gianluigi Bajocchi, Andrea Caruso, Riccardo Valli, Tommaso Ricchetti, Giorgio Sgarbi, Carlo Salvarani
BACKGROUND: Thymoma is a T cell neoplasm arising from the thymic epithelium that due to its immunological role, frequently undercover derangements of immunity such a tumors and autoimmune diseases. METHODS: Herein, we report, to the best of our knowledge, the first description of an association between thymoma and adult onset Still disease (AOSD) in a 47-year-old man. The first one was occasionally detected 4 years later the diagnosis of AOSD, and surgically removed via right lateral thoracotomy...
September 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27599266/a-rare-ocular-manifestation-of-adult-onset-still-s-disease-purtscher-s-like-retinopathy
#13
Cihan Buyukavsar, Ergenekon Karagoz, Murat Sonmez, Taner Kar, Abdullah Kaya, Eyup Düzgun, Yildiray Yildirim
Adult-onset Still's disease (AOSD) is a rare multisystemic immune-mediated disease of unknown etiology with quotidian spiking fever, evanescent rash, arthralgia, and multiple organ involvement. The few AOSD cases that have been reported developed Purtscher's-like retinopathy associated with thrombotic microangiopathy (TMA). Here, we report Purtscher's-like retinopathy without TMA in a patient with AOSD. A 29-year-old-man who presented for evaluation of blurred vision was diagnosed with AOSD based on Yamaguchi criteria...
September 6, 2016: Ocular Immunology and Inflammation
https://www.readbyqxmd.com/read/27588099/adult-onset-still-s-disease-accompanied-by-acute-respiratory-distress-syndrome-a-case-report
#14
Xiao-Tu Xi, Mao-Jie Wang, Run-Yue Huang, Bang-Han Ding
Adult onset Still's disease (AOSD) is a systemic inflammatory disorder characterized by rash, leukocytosis, fever and arthralgia/arthritis. The most common pulmonary manifestations associated with AOSD are pulmonary infiltrates and pleural effusion. The present study describes a 40-year-old male with AOSD who developed fever, sore throat and shortness of breath. Difficulty breathing promptly developed, and the patient was diagnosed with acute respiratory distress syndrome (ARDS). The patient did not respond to antibiotics, including imipenem, vancomycin, fluconazole, moxifloxacin, penicillin, doxycycline and meropenem, but was sensitive to glucocorticoid treatment, including methylprednisolone sodium succinate...
September 2016: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/27537874/tlr4-endogenous-ligand-s100a8-a9-levels-in-adult-onset-still-s-disease-and-their-association-with-disease-activity-and-clinical-manifestations
#15
Hyoun-Ah Kim, Jae Ho Han, Woo-Jung Kim, Hyun Jin Noh, Jeong-Mi An, Hyunee Yim, Ju-Yang Jung, You-Sun Kim, Chang-Hee Suh
S100A8/A9 has been suggested as a marker of disease activity in patients with adult-onset Still's disease (AOSD). We evaluated the clinical significance of S100A8/A9 as a biomarker and its pathogenic role in AOSD. Blood samples were collected prospectively from 20 AOSD patients and 20 healthy controls (HCs). Furthermore, skin and lymph node biopsy specimens of AOSD patients were investigated for S100A8/A9 expression levels via immunohistochemistry. Peripheral blood mononuclear cells (PBMCs) of active AOSD patients and HCs were investigated for S100A8/A9 cell signals...
2016: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/27502500/epidemiological-study-of-adult-onset-still-s-disease-using-a-japanese-administrative-database
#16
Nobuo Sakata, Sayuri Shimizu, Fumio Hirano, Kiyohide Fushimi
Adult-onset Still's disease (AOSD) is a rare disease, and large epidemiological studies of this disease are limited. Furthermore, it has been difficult to show the incidence and characteristics of severe AOSD complications due to the rarity of this disease. The aim of our study was to describe the demographics of AOSD and the incidence and characteristics of severe complications. Using a large Japanese administrative database, we identified hospitalized patients with AOSD and described the demographics. We also calculated the incidence of severe complications (i...
October 2016: Rheumatology International
https://www.readbyqxmd.com/read/27472698/parenchymal-lung-involvement-in-adult-onset-still-disease-a-strobe-compliant-case-series-and-literature-review
#17
Mathieu Gerfaud-Valentin, Vincent Cottin, Yvan Jamilloux, Arnaud Hot, Agathe Gaillard-Coadon, Isabelle Durieu, Christiane Broussolle, Jean Iwaz, Pascal Sève
Parenchymal lung involvement (PLI) in adult-onset Still's disease (AOSD) has seldom, if ever, been studied. We examine here retrospective cohort AOSD cases and present a review of the literature (1971-2014) on AOSD-related PLI cases.Patients with PLI were identified in 57 AOSD cases. For inclusion, the patients had to fulfill Yamaguchi or Fautrel classification criteria, show respiratory symptoms, and have imaging evidence of pulmonary involvement, and data allowing exclusion of infectious, cardiogenic, toxic, or iatrogenic cause of PLI should be available...
July 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27462914/efficacy-of-tocilizumab-therapy-in-korean-patients-with-adult-onset-still-s-disease-a-multicentre-retrospective-study-of-22-cases
#18
Seung Taek Song, Jin Ju Kim, Seung Lee, Hyoun-Ah Kim, Eun Young Lee, Ki Chul Shin, Joo Hyun Lee, Kwang-Hoon Lee, Sang Tae Choi, Hoon-Suk Cha, Dae-Hyun Yoo
OBJECTIVES: To evaluate the efficacy of tocilizumab (TCZ), a monoclonal antibody against the interleukin (IL)-6 receptor, for refractory adult-onset Still's disease (AOSD) in the Korean population. METHODS: This retrospective study included 22 Korean patients with refractory AOSD who were given TCZ at one of seven university hospital-based clinics for rheumatic disease. Patients were subdivided into groups according to disease course: monocyclic, systemic polycyclic, and chronic articular...
September 2016: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/27408785/pulmonary-arterial-hypertension-secondary-to-adult-onset-still-s-disease-response-to-cyclosporine-and-sildenafil-over-15-years-of-follow-up
#19
Jason Weatherald, Johan Lategan, Doug Helmersen
Adult onset Still's disease (AOSD) is an autoimmune disease characterized by systemic inflammation and is a rarely reported cause of pulmonary arterial hypertension (PAH). We describe the clinical course of a 40-year-old woman who presented with PAH 19 months after a diagnosis of AOSD. Sildenafil and immunosuppressive therapy with cyclosporine resulted in clinical and hemodynamic improvement with long-term survival 15 years after her initial presentation of AOSD. We review the literature for published cases of PAH due to AOSD and discuss the potential mechanisms relating inflammatory diseases and PAH...
2016: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/27359073/assessment-of-the-hscore-for-reactive-haemophagocytic-syndrome-in-patients-with-rheumatic-diseases
#20
E D Batu, A Erden, E Seyhoğlu, L Kilic, Y Büyükasık, O Karadag, Y Bilginer, S A Bilgen, A Akdogan, S Kiraz, A I Ertenli, S Özen, U Kalyoncu
OBJECTIVES: Reactive haemophagocytic syndrome (RHS) is a hyperinflammatory disorder often occurring in the background of several disorders such as infections, malignancies, and rheumatic diseases. Recently, a score known as the HScore was developed for the diagnosis of RHS. In the original study, most of the patients had underlying haematological malignancy or infection and the best cut-off value for the HScore was 169 (sensitivity 93%; specificity 86%). In this study we aimed to analyse the performance of the HScore in rheumatic disease-related RHS...
June 30, 2016: Scandinavian Journal of Rheumatology
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