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https://www.readbyqxmd.com/read/28436448/elevated-circulating-levels-of-the-interferon-%C3%AE-induced-chemokines-are-associated-with-disease-activity-and-cutaneous-manifestations-in-adult-onset-still-s-disease
#1
Jae Ho Han, Chang-Hee Suh, Ju-Yang Jung, Mi-Hyun Ahn, Mi Hwa Han, Ji Eun Kwon, Hyunee Yim, Hyoun-Ah Kim
C-X-C motif chemokine 9 (CXCL9), CXCL10, and CXCL11 are produced in response to interferon-γ (IFN-γ) and trigger inflammation with the accumulation of activated lymphocytes. It appears that these chemokines could play a role in the pathogenesis of adult-onset Still's disease (AOSD). Therefore, we investigated the associations between the levels of these chemokine and clinical manifestations in patients with active AOSD. Serum levels of IFN-γ, CXCL9, CXCL10 and CXCL11 were determined using enzyme-linked immunosorbent assays...
April 24, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28412707/application-of-the-2016-eular-acr-printo-classification-criteria-for-macrophage-activation-syndrome-in-patients-with-adult-onset-still-disease
#2
Sung Soo Ahn, Byung-Woo Yoo, Seung Min Jung, Sang-Won Lee, Yong-Beom Park, Jason Jungsik Song
OBJECTIVE: To evaluate the clinical significance of the 2016 European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR)/Pediatric Rheumatology International Trials Organization (PRINTO) classification criteria for macrophage activation syndrome (MAS) in patients with adult-onset Still disease (AOSD). METHODS: We performed a retrospective analysis of patients with AOSD with fever who were admitted to Severance Hospital between 2005 and 2016...
April 15, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28365573/serum-levels-of-interleukin-33-and-soluble-st2-are-associated-with-the-extent-of-disease-activity-and-cutaneous-manifestations-in-patients-with-active-adult-onset-still-s-disease
#3
Jae Ho Han, Chang-Hee Suh, Ju-Yang Jung, Mi-Hyun Ahn, Ji Eun Kwon, Hyunee Yim, Hyoun-Ah Kim
OBJECTIVE: Interleukin 33 (IL-33), a member of the IL-1 family and a ligand of the orphan receptor ST2, plays key roles in innate and adaptive immunity. We examined the associations between IL-33/ST2 levels and clinical manifestations of patients with active adult-onset Still's disease (AOSD). METHODS: Blood samples were collected from 40 patients with active AOSD, 28 patients with rheumatoid arthritis (RA), and 27 healthy controls (HC). The serum levels of IL-33 and soluble ST2 were determined using ELISA...
April 1, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28342280/clinical-and-histopathological-features-of-cutaneous-manifestations-of-adult-onset-still-disease
#4
Erin Santa, Jeanne M McFalls, Joya Sahu, Jason B Lee
Adult-onset Still disease (AOSD) is a rare autoinflammatory syndrome characterized by recurring fevers, arthralgia, and consistent laboratory abnormalities that include leukocytosis and hyperferritinemia. Skin findings accompany the disease in nearly 90% of the cases. Early reports described evanescent, pruritic, salmon-pink or urticarial lesions, referred to as the typical eruption of AOSD. Histopathologic findings consist of superficial perivascular dermatitis with varying number of interstitial neutrophils...
March 25, 2017: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/28296747/adult-onset-still-s-disease-with-atypical-cutaneous-manifestations
#5
Francisco Javier Narváez Garcia, María Pascual, Mercè López de Recalde, Pablo Juarez, Isabel Morales-Ivorra, Jaime Notario, Anna Jucglà, Joan M Nolla
The diagnosis of adult-onset Still's disease (AOSD) can be very difficult. There are no specific tests available, and diagnosis is usually based on a symptom complex and the well-described typical evanescent rash seen in the majority of patients. However, in recent years, other atypical cutaneous manifestations of AOSD have been reported. These atypical skin eruptions often present in addition to the typical evanescent rash but may also be the only skin manifestation, resulting in delayed diagnosis because of under-recognition...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28204878/unusual-onset-of-adult-still-s-disease-due-to-a%C3%A2-systemic-reaction-to-artificial-breast-implants
#6
A Wehr, G Grieb, C Trautwein, K Streetz
In this manuscript, we report an exceptional observation study of a young woman suffering from an autoimmune syndrome induced by adjuvants (ASIA). Until today only seven cases of adult-onset Still's disease (AOSD) induced by breast implants have been published. This may be due to the fact that this illness itself is very rare; however, the reason might also be that the community is not sensitized to the case-specific symptoms. Within this article, we show for the first time highly detailed diagnostic test procedures such as PET-CT scans and specific histological staining of the breast tissue, displaying proinflammatory macrophages that are a well-known activator and booster of autoimmune diseases...
February 15, 2017: Zeitschrift Für Rheumatologie
https://www.readbyqxmd.com/read/28154368/adult-onset-still-s-disease-still-a-serious-health-problem-a-case-report-and-literature-review
#7
REVIEW
Mojgan Agha-Abbaslou, Ana Maria Bensaci, Oluchi Dike, Mark C Poznansky, Arooj Hyat
BACKGROUND Adult-onset Still's Disease (AOSD) is a rare systemic inflammatory disease accompanied by a triad of spiking fever, maculopapular exanthema, and arthralgia. To date, there is no definite laboratory or imaging test available for diagnosing AOSD, and the diagnosis is one of exclusion, which can be very challenging. CASE REPORT We report on the case of a 53-year-old female who presented with fever, arthralgia, and abdominal pain. Her initial laboratory tests showed elevated AST and ALT, and normal leukocytes with bandemia...
February 3, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28149024/extremely-high-ferritinemia-associated-with-haemophagocytic-lympho-histiocytosis-hlh
#8
Neelima Verma, Jyoti Chakraverty, Pankaj Baweja, Alka Girotra, Leena Chatterjee, Manish Chugh
Hyperferritinemia (>10,000 ng/ml) is an important hallmark used as an indicator of infection triggered macrophage activation syndrome leading to hemophagocytic lympho histiocytosis (HLH). Measurement of serum ferritin can be used in diagnosis as well as disease monitoring indicator and prognosis related to HLH, cAPS, sepsis, neoplasm and inflammatory conditions. It is a major contributor to manage critically ill patients as predicting and monitoring indicator. It can be used as acute phase response in conditions of MAS, AOSD, cAPS etc...
March 2017: Indian Journal of Clinical Biochemistry: IJCB
https://www.readbyqxmd.com/read/28130282/rumpel-leede-phenomenon-in-a-patient-with-adult-onset-still-s-disease
#9
Jacob Trygve Lambdin, Catherine Mackenzie
Rumpel-Leede phenomenon (RLP), also known as acute capillary rupture syndrome (ACRS), is a rare occurrence where distal dermal capillaries rupture in response to a proximal compressive force, such as a blood pressure cuff or tourniquet. This phenomenon has been reported to occur in states of vascular fragility such as long-term steroid use, hypertension or diabetes mellitus. Here, we provide a report of RLP occurring secondary to tourniquet application in a 26-year-old woman with adult-onset Still's disease (AOSD) and a recent drug rash...
January 27, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28106835/highly-expression-of-cd11b-and-cd32-on-peripheral-blood-mononuclear-cells-from-patients-with-adult-onset-still-s-disease
#10
Hyoun-Ah Kim, Bunsoon Choi, Chang-Hee Suh, Mi Hwa Han, Ju-Yang Jung, Hasan M Sayeed, Ye Won Kim, Seonghyang Sohn
BACKGROUND: We investigated the potential role of several pattern-recognition receptors (PRRs; CD11b, CD11c, CD32, CD206, CD209, and dectin-1) in adult-onset Still's disease (AOSD). METHODS: The study included 13 untreated AOSD patients, 19 rheumatoid arthritis (RA) patients (as a disease control), and 19 healthy controls (HCs). The PRRs were quantified in peripheral blood using flow cytometry. The serum levels of interleukin-17 (IL-17), IL-18, and IL-23 were measured by enzyme-linked immunosorbent assay...
January 19, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28056958/about-the-complexity-of-adult-onset-still-s-disease%C3%A2-and-advances-still-required-for-its-management
#11
EDITORIAL
Philippe Guilpain, Alain Le Quellec
Adult onset Still's disease (AOSD) is a rare inflammatory disorder that remains poorly understood. Its pathophysiology is yet to be completely elucidated, but is known to consist mainly on a cytokine cascade, responsible for the systemic manifestations. AOSD diagnosis is usually difficult and delayed, with physicians having to rule out several other conditions, including cancer or infectious diseases. Prognosis is heterogeneous and difficult to establish, ranging from benign outcome to chronic destructive polyarthritis and/or life-threatening events...
January 6, 2017: BMC Medicine
https://www.readbyqxmd.com/read/28018698/combination-immunosuppressive-therapy-including-rituximab-for-epstein-barr-virus-associated-hemophagocytic-lymphohistiocytosis-in-adult-onset-still-s-disease
#12
Eva Johanna Schäfer, Wolfram Jung, Peter Korsten
Hemophagocytic lymphopcytosis (HLH) is a life-threatening condition. It can occur either as primary form with genetic defects or secondary to other conditions, such as hematological or autoimmune diseases. Certain triggering factors can predispose individuals to the development of HLH. We report the case of a 25-year-old male patient who was diagnosed with HLH in the context of adult-onset Still's disease (AOSD) during a primary infection with Epstein-Barr virus (EBV). During therapy with anakinra and dexamethasone, he was still symptomatic with high-spiking fevers, arthralgia, and sore throat...
2016: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/28005947/metabolic-disturbances-in-adult-onset-still-s-disease-evaluated-using-liquid-chromatography-mass-spectrometry-based-metabolomic-analysis
#13
Der-Yuan Chen, Yi-Ming Chen, Han-Ju Chien, Chi-Chen Lin, Chia-Wei Hsieh, Hsin-Hua Chen, Wei-Ting Hung, Chien-Chen Lai
OBJECTIVE: Liquid chromatography/mass spectrometry (LC/MS)-based comprehensive analysis of metabolic profiles with metabolomics approach has potential diagnostic and predictive implications. However, no metabolomics data have been reported in adult-onset Still's disease (AOSD). This study investigated the metabolomic profiles in AOSD patients and examined their association with clinical characteristics and disease outcome. METHODS: Serum metabolite profiles were determined on 32 AOSD patients and 30 healthy controls (HC) using ultra-performance liquid chromatography (UPLC)/MS analysis, and the differentially expressed metabolites were quantified using multiple reactions monitoring (MRM)/MS analysis in 44 patients and 42 HC...
2016: PloS One
https://www.readbyqxmd.com/read/27981819/successful-treatment-of-refractory-adult-onset-still-s-disease-with-rituximab
#14
N Belfeki, M Smiti Khanfir, F Said, A Hamzaoui, T Ben Salem, I Ben Ghorbel, M Lamloum, M H Houman
Adult-onset Still's disease (AOSD) is an uncommon inflammatory condition of unknown origin. In chronic disease, joint involvement is often predominant and erosions are noted in one third of patients. Therapeutic strategies derive from observational data. Corticosteroids are usually the first-line treatment. With inadequate response to corticosteroids, methotrexate appears the best choice to control disease activity and allow for tapering of steroid use. For refractory disease, biological therapy seems the most promising...
December 16, 2016: Reumatismo
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
#15
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
#16
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
#17
REVIEW
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
#18
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
#19
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
#20
Kazuhito Fukuoka, Ayako Miyamoto, Yuko Ozawa, Noriko Ikegaya, Tomohiro Maesono, Yoshinori Komagata, Shinya Kaname, Yoshihiro Arimura
A 72-year-old woman presented 9 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)...
December 9, 2016: Modern Rheumatology
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