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https://www.readbyqxmd.com/read/28781105/adult-s-onset-still-disease-occurring-during-pregnancy-case-report-and-literature-review
#1
Léo Plaçais, Arsène Mekinian, Marie Bornes, Armelle Poujol-Robert, Naiké Bigé, Eric Maury, Olivier Fain
INTRODUCTION: Adult onset Still's disease is a rare affection classified among non-hereditary autoinflammatory diseases. We here report a case of AOSD revealed during pregnancy with a life-threatening presentation along with a review of 19 cases from literature. CASE: A 38-years old woman was treated in our department for diffuse systemic sclerosis and associated Sjögren syndrome. She was pregnant and presented with acute fever and arthralgias. Laboratory data revealed mild liver cytolysis but a large screening for infectious and auto-immune diseases was negative and hepato-biliar imaging was normal...
July 10, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/28765926/imaging-characteristics-of-adult-onset-still-s-disease-demonstrated-with-18f-fdg-pet-ct
#2
Lei Jiang, Yan Xiu, Taoying Gu, Caihong Dong, Bing Wu, Hongcheng Shi
The diagnosis of adult onset Still's disease (AOSD) is non‑specific, and requires the exclusion of other diseases including infectious, inflammatory and malignant diseases. The current study aimed to summarize the imaging characteristics of fluorodeoxyglucose (18F‑FDG) positron emission tomography (PET)/computerized tomography (CT) in patients with AOSD. The 18F‑FDG PET/CT characteristic observations of 32 patients with definite AOSD were retrospectively reviewed based on visual interpretation and the semi‑quantitative index of standard uptake value of maximum (SUVmax)...
September 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28760536/validation-of-the-fautrel-classification-criteria-for-adult-onset-still-s-disease
#3
Delphine Lebrun, Stéphanie Mestrallet, Monique Dehoux, Jean Louis Golmard, Benjamin Granger, Sophie Georgin-Lavialle, Laurent Arnaud, Gilles Grateau, Jacques Pouchot, Bruno Fautrel
OBJECTIVES: To validate the Fautrel classification criteria for adult-onset Still's disease (AOSD) and to compare the discriminative performance to that of the Yamaguchi criteria. METHODS: We retrospectively reviewed the medical charts of 426 patients who had serum ferritin level and percentage glycosylated ferritin assayed at the biochemistry laboratory of Bichat Hospital. Medical data were extracted by use of a standardized form. All clinical, biological, and imaging features were collected, as well, evidence favoring an alternative diagnosis, specifically symptoms suggestive of other immune-mediated inflammatory diseases (IMID) or active infections...
July 12, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/28757235/adult-onset-still-s-disease-the-evidence-that-anti-interleukin-1-treatment-is-effective-and-well-tolerated-a-comprehensive-literature-review
#4
REVIEW
Guido Junge, June Mason, Eugen Feist
The literature contains many reports of the use of commercially available anti-IL-1 agents (anakinra/Kineret(®), canakinumab/Ilaris(®), or rilonacept/Arcalyst(®)) in treatment-resistant adult-onset Still's disease (AOSD). These have been widely summarized in many review articles, but a full account of all reports with each of the agents used is not available. This literature review includes all reports of treatment outcomes in patients treated for AOSD with any commercially available anti-IL-1 agent (excluding cases of unconfirmed or atypical AOSD or treatments only for rare AOSD complications)...
June 23, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/28755070/-still-s-disease-in-children-and-adults
#5
C M Hedrich, C Günther, M Aringer
Systemic juvenile idiopathic arthritis (sJIA) is characterized by fever, arthritis, and other signs of systemic inflammation. Historically, sJIA was named Still's disease after George Frederic Still, who first reported patients. Individuals who manifest after the 16(th) birthday are diagnosed with adult onset Still's disease (AOSD). The pathophysiology of sJIA and AOSD are incompletely understood. Increased activation of inflammasomes and the expression of proinflammatory cytokines play a central role. S100 proteins, which can activate Toll-like receptors, thus, maintaining positive feedback loops, have also been detected at increased levels in sera from sJIA patients...
July 28, 2017: Zeitschrift Für Rheumatologie
https://www.readbyqxmd.com/read/28725546/pulmonary-involvement-in-adult-still-s-disease-case-report-and-brief-review-of-literature
#6
Aldo Guerrieri, Giulia Angeletti, Massimiliano Mazzolini, Ilaria Bassi, Stefano Nava
BACKGROUND: Adult onset Still disease (AOSD) is a rare condition characterized by elevated fever along with arthritic symptoms, elevated polymorphonuclear neutrophil count, evanescent rash, and hyperferritinemia. Diagnosis can be made only after have ruled out more frequent conditions, and Yamagouchi or Fautrel criteria should be applied. Parenchimal lung involvement (PLI) is present in less than 5% of AOSD cases and ranges from aspecific reticular interstitial opacities to life threatening conditions, such as acute respiratory distress syndrome (ARDS)...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28723802/tocilizumab-for-uncontrollable-systemic-inflammatory-response-syndrome-complicating-adult-onset-still-disease-case-report-and-review-of-literature
#7
REVIEW
Asami Masui-Ito, Ryuji Okamoto, Kaoru Ikejiri, Mika Fujimoto, Muneyoshi Tanimura, Shiro Nakamori, Tomohiro Murata, Eiji Ishikawa, Norikazu Yamada, Hiroshi Imai, Masaaki Ito
RATIONALE: Adult-onset Still disease (AOSD) is a rare systemic inflammatory disease of unknown etiology characterized by evanescent salmon-pink rash, fever spikes, arthralgia, and lymphadenopathy. AOSD usually has a good prognosis, but it can sometimes be fatal, especially when it is complicated by systemic inflammatory response syndrome (SIRS) and multiple organ failure. PATIENT CONCERNS: A previously healthy 26-year-old woman was referred to our hospital for persistent high fever and mild systemic edema...
July 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28723775/the-neutrophil-to-lymphocyte-ratio-could-be-a-good-diagnostic-marker-and-predictor-of-relapse-in-patients-with-adult-onset-still-s-disease-a-strobe-compliant-retrospective-observational-analysis
#8
Ji-Yeoun Seo, Chang-Hee Suh, Ju-Yang Jung, Ar-Reum Kim, Ji Won Yang, Hyoun-Ah Kim
The neutrophil-to-lymphocyte ratio (NLR) is the proportion of absolute neutrophil count to lymphocytes on routine complete blood count (CBC) tests, and has been studied as a simple marker of the systemic inflammatory response. This study was performed to investigate whether the NLR could be used as a tool to diagnose and predict prognosis in cases of adult-onset Still's disease (AOSD).We retrospectively reviewed 164 patients with suspected AOSD. Among 164 patients with suspected AOSD, 37 patients received another diagnosis (such as viral infection) and were compared with the 127 patients who received a diagnosis of AOSD...
July 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28720215/autoimmune-meningitis-and-encephalitis-in-adult-onset-still-disease-case-report
#9
Bożek Milena, Konopko Magdalena, Wierzba-Bobrowicz Teresa, Witkowski Grzegorz, Makowicz Grzegorz, Sienkiewicz-Jarosz Halina
INTRODUCTION: Adult-onset Still disease (AOSD) is a rare systemic inflammatory disease of unknown cause. Its symptoms usually include persistent fever, fugitive salmon-colored rash, arthritis, sore throat (not specific), but it may also lead to internal organs' involvement, which presents with enlargement of the liver and spleen, swollen lymph nodes, carditis or pleuritis - potentially life-threatening complications. In rare cases, AOSD can cause aseptic meningitis or/and encephalitis...
July 8, 2017: Neurologia i Neurochirurgia Polska
https://www.readbyqxmd.com/read/28690954/delay-in-the-diagnosis-of-adult-onset-still-s-disease
#10
Stella Pak, Cindy Pham
Adult-onset Still's disease (AOSD) is a systemic autoinflammatory disease characterized by symptoms including spiking fever, arthralgia, myalgia, maculopapular rash, and pharyngitis. The lack of diagnostic biomarker, non-specific clinical presentation, and the rarity of AOSD often result in a significant delay in diagnosis and treatment. While the average time of initial presentation to diagnosis is four months, we present a case of AOSD diagnosis three years after initial onset of classical symptoms. By reporting the case of delayed diagnosis for AOSD, we hope to raise awareness in our medical community about the diagnostic difficulty in AOSD...
June 7, 2017: Curēus
https://www.readbyqxmd.com/read/28690235/-diagnosis-and-treatment-for-one-case-of-elderly-diabetes-complicated-with-still-s-disease
#11
Tuanyu Fang, Kaining Chen, Eryuan Liao
Adult onset Still's disease (AOSD) is a clinical syndrome with multiple organ failure. The patients normally show intermittent high fever for a long time, a transient rash, arthritis or joint pain as the main performance, accompanied by an increase in granulocytes and enlargement in liver, spleen and lymph node. A 71-years-old female patient with type 2 diabetes admitted hospital because of high fever, skin rash, joint pain and increased granulocyte. After review of the iron protein, she was diagnosed as AOSD...
June 28, 2017: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
https://www.readbyqxmd.com/read/28659802/response-to-interleukin-1-inhibitors-in-140-italian-patients-with-adult-onset-still-s-disease-a-multicentre-retrospective-observational-study
#12
Serena Colafrancesco, Roberta Priori, Guido Valesini, Lorenza Argolini, Elena Baldissera, Elena Bartoloni, Daniele Cammelli, Giovanni Canestrari, Luca Cantarini, Elena Cavallaro, Giulio Cavalli, Lucia Cerrito, Paola Cipriani, Lorenzo Dagna, Ginevra De Marchi, Salvatore De Vita, Giacomo Emmi, Gianfranco Ferraccioli, Micol Frassi, Mauro Galeazzi, Roberto Gerli, Roberto Giacomelli, Elisa Gremese, Florenzo Iannone, Giovanni Lapadula, Giuseppe Lopalco, Raffaele Manna, Alessandro Mathieu, Carlomaurizio Montecucco, Marta Mosca, Ilaria Piazza, Matteo Piga, Irene Pontikaki, Micol Romano, Silvia Rossi, Maurizio Rossini, Piero Ruscitti, Elena Silvestri, Chiara Stagnaro, Rosaria Talarico, Angela Tincani, Ombretta Viapiana, Gianfranco Vitiello, Francesca Fabris, Sara Bindoli, Leonardo Punzi, Paola Galozzi, Paolo Sfriso
Background: Interleukin (IL)-1 plays a crucial role in the pathogenesis of Adult onset Still's disease (AOSD). Objectives: To evaluate the efficacy and safety of anakinra (ANA) and canakinumab (CAN) in a large group of AOSD patients. Methods: Data on clinical, serological features, and concomitant treatments were retrospectively collected at baseline and after 3, 6, and 12 months from AOSD patients (Yamaguchi criteria) referred by 18 Italian centers. Pouchot's score was used to evaluate disease severity. Results: One hundred forty patients were treated with ANA; 4 were subsequently switched to CAN after ANA failure...
2017: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/28646209/upregulation-of-circulating-microrna-134-in-adult-onset-still-s-disease-and-its-use-as-potential-biomarker
#13
Tsai-Ling Liao, Yi-Ming Chen, Chia-Wei Hsieh, Hsin-Hua Chen, Hsiu-Chin Lee, Wei-Ting Hung, Kuo-Tung Tang, Der-Yuan Chen
Adult-onset Still's disease (AOSD) is a multi-systemic inflammatory disorder of unknown etiology. To date, no single diagnostic test is available for AOSD. Herein, we investigated the pathogenic role of microRNAs in AOSD. MicroRNA profiles in plasma from AOSD patients and healthy controls were analyzed by microarray analysis, followed by quantitative reverse transcription PCR validation. The biological functions of microRNAs were evaluated using in vitro cell-based assay. Among the differentially expressed microRNAs, microRNA-134 (miR-134) expression was positively correlated with AOSD activity scores and significantly decreased after effective treatment...
June 23, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28614216/refractory-adult-onset-still-disease-complicated-by-macrophage-activation-syndrome-and-acute-myocarditis-a-case-report-treated-with-high-doses-8%C3%A2-mg-kg-d-of-anakinra
#14
Federico Parisi, Annamaria Paglionico, Valentina Varriano, Gianfranco Ferraccioli, Elisa Gremese
RATIONALE: Myocarditis is a rare but potentially fatal complication of Still's disease (about 7% of total cases). PATIENT CONCERNS: A 42-year-old woman was admitted to our ward with high-grade fever, rash and polyarthralgia, lasting since 4 weeks and rapidly complicated by MAS and acute heart failure. DIAGNOSES: Adult Onset Still's Disease rapidly developping macrophage activation syndrome and disseminated intravascular coagulopathy, further complicated by iperacute myocarditis with cardiac arrest...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28589388/-efficacy-and-safety-analysis-of-off-label-treatment-with-biologics-in-autoinflammatory-diseases-experiences-from-a%C3%A2-german-registry%C3%A2-graid2
#15
F Proft, M Fleck, C Fiehn, H Schulze-Koops, M Witt, T Dörner, J C Henes
OBJECTIVE: To evaluate the safety and efficacy of therapy with biologics in patients with autoinflammatory diseases (AIF) or macrophage activating syndrome (MAS) in a real-life setting in Germany. METHODS: The German Register of Autoimmune Diseases 2 (GRAID2) is a retrospective, non-interventional, multicenter registry collecting data from all patients with inflammatory rheumatic diseases refractory to conventional therapy and treated with initial off-label biologics between August 2006 and December 2013...
June 6, 2017: Zeitschrift Für Rheumatologie
https://www.readbyqxmd.com/read/28540751/treatment-of-adult-onset-still-s-disease-up-to-date
#16
Dae Hyun Yoo
Adult onset Still's disease (AOSD) is a systemic inflammatory disorder of unknown etiology, and approximately 60-70% of patients may develop a chronic polyphasic form of the disease or a chronic polyarthritis. Due to rarity of disease, treatment of AOSD is not based on controlled study, but on case based experiences. Areas covered: Recently, the application of anti-cytokine therapy based on pathophysiology has resulted in significant progress in the treatment of AOSD. Here, we review current knowledge of the pathogenesis, disease progression, currently available biomarkers of disease activity, standard therapeutic agents, utility of biologic agents, future perspectives for treatment and treatment of macrophage activation syndrome...
September 2017: Expert Review of Clinical Immunology
https://www.readbyqxmd.com/read/28532741/coexistence-of-sarcoidosis-and-adult-onset-still-disease
#17
Huseyin Semiz, Senol Kobak
Sarcoidosis is a chronic, inflammatory disease with unknown cause characterized by non-caseating granuloma formations. It can be presented with bilateral hilar lymphadenopathy, skin lesions, eye involvement and locomotor system findings. Adult onset Still disease (AOSD) is a chronic inflammatory disease which presents with fever, arthritis and typical skin rashes. The disease is rare and can be misdiagnosed due to the absence of typical clinical and laboratory findings. The association of sarcoidosis and AOSD has not been previously reported in the literature...
May 19, 2017: Reumatología Clinica
https://www.readbyqxmd.com/read/28529117/new-markers-for-adult-onset-still-s-disease
#18
Stéphane Mitrovic, Bruno Fautrel
Adult-onset Still's disease (AOSD) is a rare systemic auto-inflammatory disorder (SAID). Although the pathogenesis of the disease is complex and far from being fully understood, recent progresses in pathophysiological knowledge have paved the way to new diagnostic approaches. Indeed, AOSD diagnosis can be a real challenge, owing to its infrequency, and to the lack of specificity of the principal clinical features (high fever, arthralgia or arthritis, skin rash) and laboratory findings (elevated acute phase reactants, hyperleukocytosis ≥ 10,000 cells/mm(3) with neutrophils ≥80%)...
May 18, 2017: Joint, Bone, Spine: Revue du Rhumatisme
https://www.readbyqxmd.com/read/28507179/elevated-expression-of-the-nlrp3-inflammasome-and-its-correlation-with-disease-activity-in-adult-onset-still-disease
#19
Chia-Wei Hsieh, Yi-Ming Chen, Chi-Chen Lin, Kuo-Tung Tang, Hsin-Hua Chen, Wei-Ting Hung, Kuo-Lung Lai, Der-Yuan Chen
OBJECTIVE: The dysregulation of the NLRP3 (NLR containing a pyrin domain) inflammasome is involved in autoinflammatory diseases. Adult-onset Still disease (AOSD) is regarded as an autoinflammatory disease. However, the pathogenic involvement of NLRP3 inflammasome in AOSD remains unclear and NLRP3 activators in AOSD are currently unknown. METHODS: The mRNA expression of NLRP3 inflammasome signaling in peripheral blood mononuclear cells (PBMC) from 34 patients with AOSD and 14 healthy subjects was determined using quantitative-PCR (qPCR)...
May 15, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28470759/phosphorylated-signal-transducer-and-activator-of-transcription-3-in-the-epidermis-in-adult-onset-still-s-disease
#20
Takuya Takeichi, Naoki Watanabe, Yoshinao Muro, Shiho Teshigawara, Motoki Sato, Nobutaro Ban, Masashi Akiyama
Adult-onset Still's disease (AOSD) is characterized by multiple systemic inflammation of unknown etiology. Although the typical eruption of AOSD is salmon-pink rheumatoid rash on the trunk and extremities, persistent pruritic papules and plaques have also been reported. Correlations between serum cytokines, including interleukin-6 and -18, and disease activity in AOSD have been reported. Activated signal transducer and activator of transcription 3 (STAT3) is transported into the nucleus, where it functions as a transcription factor that regulates genes involved in cell survival and inflammation...
May 4, 2017: Journal of Dermatology
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