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Eugen Feist, Pierre Quartier, Bruno Fautrel, Rayfel Schneider, Paolo Sfriso, Petros Efthimiou, Luca Cantarini, Karine Lheritier, Karolynn Leon, Chetan S Karyekar, Antonio Speziale
OBJECTIVES: To describe the efficacy, safety, and exposure-response relationship of canakinumab in a subgroup of patients with systemic juvenile idiopathic arthritis (SJIA) aged ≥16 years, representative of adult-onset Still's disease (AOSD) patients, and to compare this subgroup with those of children and young adolescents with SJIA by pooling clinical data collected during the development programme of canakinumab. METHODS: Safety and efficacy data on canakinumab-treated patients were pooled from 4 SJIA studies (NCT00426218, NCT00886769, NCT00889863, and NCT00891046)...
March 2, 2018: Clinical and Experimental Rheumatology
Cem Gabay, Bruno Fautrel, Jürgen Rech, François Spertini, Eugen Feist, Ina Kötter, Eric Hachulla, Jacques Morel, Thierry Schaeverbeke, Mohamed A Hamidou, Thierry Martin, Bernhard Hellmich, Peter Lamprecht, Hendrik Schulze-Koops, Delphine Sophie Courvoisier, Andrew Sleight, Eduardo Jorge Schiffrin
OBJECTIVES: Adult-onset Still's disease (AOSD) is a rare systemic autoinflammatory disease; its management is largely empirical. This is the first clinical study to determine if interleukin (IL)-18 inhibition, using the recombinant human IL-18 binding protein, tadekinig alfa, is a therapeutic option in AOSD. METHODS: In this phase II, open-label study, patients were ≥18 years with active AOSD plus fever or C reactive protein (CRP) levels ≥10 mg/L despite treatment with prednisone and/or conventional synthetic disease-modifying antirheumatic drugs (DMARDs)...
February 22, 2018: Annals of the Rheumatic Diseases
Ankur Sinha, Ravikaran Patti, Paurush Ambesh, Chukwudi Obiagwu, Namrita Malhan, Kabu Chawla
A 29-year-old female with adult-onset Still's disease (AOSD) presented with progressive shortness of breath both on rest and on exertion, increased abdominal girth, and swelling in both legs. She was on oral prednisone and was recently started on canakinumab (interleukin-1 antagonist) for joint pain and rash of AOSD. Echocardiogram showed severely dilated right ventricle, dilated pulmonary artery, moderately reduced right ventricular systolic function, but with normal left ventricular systolic function. Computed tomography with contrast ruled out pulmonary embolism...
January 2018: Journal of Investigative Medicine High Impact Case Reports
Grant S Schulert, Scott W Canna
Hyperferritinemia and pronounced hemophagocytosis help distinguish a subset of patients with a particularly inflammatory and deadly systemic inflammatory response syndrome. Two clinically similar disorders typify these hyperferritinemic syndromes: hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS). HLH is canonically associated with a complete disturbance of perforin/granzyme-mediated cytotoxicity, whereas MAS occurs in the context of the related rheumatic diseases systemic juvenile idiopathic arthritis (SJIA) and adult-onset Still's disease (AOSD), with associated IL-1 family cytokine activation...
February 6, 2018: International Immunology
Chia-Wei Hsieh, Chun-Yu Chang, Yi-Ming Chen, Hsin-Hua Chen, Wei-Ting Hung, Ning-Rong Gung, Shiow-Jiuan Wey, Der-Yuan Chen
The pathogenic role of autophagic immune regulation in adult-onset Still's disease (AOSD) is unclear. We investigated the relative levels of autophagy in AOSD patients and healthy controls, its association with disease activity or course, and the change in autophagy after 6 months of therapy. Autophagosome levels were determined from the mean fluorescence intensity of autophagosomotropic dye incorporated into circulating immune cells. The fluorescent signal from lymphocytes, monocytes, and granulocytes from AOSD patients was greater than from controls...
January 2, 2018: Oncotarget
Sha Zhou, Jianjun Qiao, Juan Bai, Yinhua Wu, Hong Fang
Background: Biotherapy is becoming increasingly important in the treatment of adult-onset Still's disease (AOSD). The aim of our study was to evaluate the efficacy and safety of biological therapy for AOSD resistant to traditional therapy. Patients and methods: Database of Library of Congress, the PubMed, and Web of Science Core Collection were used to retrieve relevant articles published in English language until March 2017. Only studies published in English language were included, and the additional references quoted in these articles were also checked...
2018: Therapeutics and Clinical Risk Management
Chia-Cheng Kuo, Wen-Liang Yu, Chen-Hui Lee, Nan-Chun Wu
RATIONALE: Purulent pericarditis is a rare and usually fatal disease. Immunodeficiency state and preexisting pericardial effusion can predispose patients to infections. However, we are not aware of similar cases in patients with adult-onset Still's disease (AOSD). In addition, it is seldom caused by Salmonella bacteria. PATIENT CONCERNS: We report a 30-year-old woman with dyspnea on exertion and epigastric fullness. She was newly diagnosed with AOSD 4 months previously and medicated with prednisolone...
December 2017: Medicine (Baltimore)
Zheng-Hao Huang, Yu-Chen Chiu, Li-Lu Ho, Hsiu-Lung Fan, Chun-Chi Lu
RATIONALE: Adult-onset Still disease (AOSD) is a rare systemic inflammatory disease of unknown etiology characterized by evanescent salmon-pink rash, spiking fever, arthralgia/ arthritis, and lymphadenopathy. AOSD sometimes was fatal when it is complicated by macrophage activation syndrome (MAS) or hemophagocytic lymphohistiocytosis (HLH). Nonetheless, the literature provides no recommendations for treatment of AOSD patients with severe sepsis. PATIENT CONCERNS: A previously healthy 65-year-old man with history of AOSD was referred to our hospital for persistent right lower quadrant abdominal pain for 2 days...
February 2018: Medicine (Baltimore)
Ji Won Yang, Eunyoung Lee, Ji-Yeon Seo, Ju-Yang Jung, Chang-Hee Suh, Hyoun-Ah Kim
BACKGROUND: Adult-onset Still's disease (AOSD) is an adult form of systemic juvenile idiopathic arthritis (JIA) that differs from the latter in its classification. This study evaluated the concordance between the International League Against Rheumatism (ILAR) criteria for systemic JIA and the Yamaguchi criteria and then compared their possible prognostic value in patients with AOSD. METHODS: In a retrospective review of 169 adults with suspected AOSD, patients were classified according to the Yamaguchi or ILAR criteria...
January 25, 2018: Pediatric Rheumatology Online Journal
Yohei Kirino, Yasushi Kawaguchi, Yoshifumi Tada, Hiroshi Tsukamoto, Toshiyuki Ota, Masahiro Iwamoto, Hiroki Takahashi, Kohei Nagasawa, Shuji Takei, Takahiko Horiuchi, Hisae Ichida, Seiji Minota, Atsuhisa Ueda, Akihide Ohta, Yoshiaki Ishigatsubo
BACKGROUND: Heme oxygenase (HO)-1 is a heme-degrading enzyme highly expressed in monocyte/macrophage, serum levels of which may be promising biomarker for adult-onset Still's disease (AOSD). We here report data on the use of serum ferritin and HO-1 levels in AOSD. METHODS: Under the Hypercytokinemia Study Group collaboration, we collected sera from a total of 145 AOSD patients. Three independent experts judged whether the patients were definite AOSD depending on the clinical information...
January 11, 2018: Modern Rheumatology
Yubo Ma, Meng Wu, Xu Zhang, Qing Xia, Jiajia Yang, Shengqian Xu, Faming Pan
OBJECTIVES: Tocilizumab (TCZ) is the only available biologics inhibiting interleukin-6 presently, and emerging evidences have figured that elevated serum level of interleukin-6 is a crucial link of the pathogenesis of adult-onset Still's disease (AOSD). However, evidence about the efficacy and safety of TCZ in AOSD with strong power is still scarce. Thus, this meta-analysis was conducted to synthetically assess the efficacy and safety of TCZ on AOSD. METHODS: PubMed, Web of Science, Cochrane Library, Chinese National Knowledge Infrastructure, and Chinese Biological Medical Literature database were comprehensively searched up to 31 July 2017 for the pertinent studies...
February 8, 2018: Modern Rheumatology
Silvia Spoto, Sebastiano Costantino, Emanuele Valeriani, Marta Fogolari, Eleonora Cella, Giordano Dicuonzo, Massimo Ciccozzi, Silvia Angeletti
Adult-onset Still's disease (AOSD) due to visceral leishmaniasis (VL) has not been previously reported. This case report analyzes a single episode of AOSD probably due to a visceral leishmaniasis successfully treated with pentamidine isethionate and prednisone.
December 2017: Clinical Case Reports
Shun Yamashita, Naoko E Furukawa, Tomoyo Matsunaga, Yuka Hirakawa, Masaki Tago, Shu-Ichi Yamashita
BACKGROUND Adult-onset Still's disease (AOSD) is a rare multi-systemic inflammatory disorder of unknown etiology characterized by spiking fever, characteristic rash, and arthritis. It often associates with high serum ferritin levels. CASE REPORT An 88-year-old woman had fever of over 39°C without response to extended-spectrum antibiotics for 6 days. She had non-specific erythema with infiltration on her trunk. She had leukocytosis with neutrophilia of 80%, mild hepatic dysfunction, normal level of rheumatoid factor and antinuclear antibody, thrombocytopenia, elevated d-dimer and soluble interleukin2 receptor, extremely high serum ferritin (78 662 ng/mL), and splenomegaly...
December 6, 2017: American Journal of Case Reports
Grant H Lowther, Jason Chertoff, Jessica Cope, Hassan Alnuaimat, Ali Ataya
Adult-onset Still's disease (AOSD) is an inflammatory disorder characterized by recurrent fevers, arthralgia, leukocytosis, and a salmon-colored rash. Diagnosis is made based on the Yamaguchi criteria. Various cardiac and pulmonary manifestations have been described in association with AOSD, including acute respiratory distress syndrome (ARDS) and pulmonary arterial hypertension (PAH). We describe the first case of both PAH and ARDS in a patient with AOSD who, despite aggressive therapy, declined rapidly and ultimately died...
October 2017: Pulmonary Circulation
R Sighart, J Rech, A Hueber, N Blank, S Löhr, A Reis, H Sticht, U Hüffmeier
OBJECTIVE: Adult onset Still's disease (AOSD) is a severe, autoimmune disease that can be challenging to treat with conventional therapeutics and biologicals in a considerable number of cases. Therefore, there is a high need to understand its pathogenesis better. As major clinical symptoms overlap between AOSD and hereditary periodic fever syndromes (HPFS), we analysed four known HPFS genes in AOSD. METHODS: We performed Sanger sequencing and quantitative analysis of all coding regions of MEFV, TNFRSF1A, MVK and NLRP3 in 40 AOSD patients...
November 20, 2017: Rheumatology International
Sakunee Niranvichaiya, Daranporn Triwongwaranat
This study reports two adult-onset Still's disease (AOSD) cases that met both Yamaguchi's and Fautrel's criteria and that presented with notable clinical manifestations. One case presented with atypical dermographism-like rash with an extremely high ferritin level. The other case presented with typical salmon-pink maculopapular rash but had atypical positive rheumatoid factor. This suggests that although negative rheumatoid factor is one of the criteria used for the diagnosis of AOSD, a positive rheumatoid factor result does not exclude AOSD...
2017: Case Reports in Dermatological Medicine
Rainer Hintenberger, Agnes Falkinger, Kathrin Danninger, Herwig Pieringer
Autoinflammatory syndromes (AIS) are characterized by recurring events of inflammation, leading to a variety of organ manifestations and fever attacks. A subgroup of AIS is commonly referred to as hereditary periodic fever syndromes (HPFS). There is substantial evidence that autoimmune diseases such as rheumatoid arthritis and systemic lupus erythematosus are strongly associated with cardiovascular morbidity and mortality. The link between AIS and cardiovascular disease is not that clear, even if the concept of continuous inflammation as a risk factor for cardiovascular disease is widely accepted...
November 1, 2017: Rheumatology International
Ting Li, Liyang Gu, Xiaodong Wang, Li Guo, Hui Shi, Chengde Yang, Sheng Chen
To investigate the efficacy and safety of Tocilizumab (TCZ) in patients with refractory adult-onset Still's disease (AOSD). We enrolled 8 female patients from October 2013 to July 2014. All patients fulfilled Japan's Yamaguch AOSD classification and recognized as refractory AOSD. All Patients received TCZ treatment 4-8 mg/kg every 4 weeks. Evaluation of efficacy was conducted after 3 months and 6 months, including clinical manifestations of AOSD patients, improvement of inflammatory markers as well as glucocorticoids dosage adjustments...
October 18, 2017: Scientific Reports
Tomoyuki Asano, Hiroshi Furukawa, Shuzo Sato, Makiko Yashiro, Hiroko Kobayashi, Hiroshi Watanabe, Eiji Suzuki, Tomoyuki Ito, Yoshifumi Ubara, Daisuke Kobayashi, Nozomi Iwanaga, Yasumori Izumi, Keita Fujikawa, Satoshi Yamasaki, Tadashi Nakamura, Tomohiro Koga, Toshimasa Shimizu, Masataka Umeda, Fumiaki Nonaka, Michio Yasunami, Yukitaka Ueki, Katsumi Eguchi, Naoyuki Tsuchiya, Shigeto Tohma, Koh-Ichiro Yoshiura, Hiromasa Ohira, Atsushi Kawakami, Kiyoshi Migita
BACKGROUND: HLA-DRB1 alleles are major determinants of genetic predisposition to rheumatic diseases. We assessed whether DRB1 alleles are associated with susceptibility to particular clinical features of adult onset Still's disease (AOSD) in a Japanese population by determining the DRB1 allele distributions. METHODS: DRB1 genotyping of 96 patients with AOSD and 1,026 healthy controls was performed. Genomic DNA samples from the AOSD patients were also genotyped for MEFV exons 1, 2, 3, and 10 by direct sequencing...
September 12, 2017: Arthritis Research & Therapy
Piero Ruscitti, Francesco Ursini, Paola Cipriani, Giovambattista De Sarro, Roberto Giacomelli
BACKGROUND: Biological drugs, mainly interleukin (IL)-1 and IL-6 antagonists, but also tumor necrosis factor (TNF) inhibitors, have been used in the treatment of adult onset Still's disease patients (AOSD). METHODS: We summarised the available evidence for the effectiveness of biologic drugs in AOSD. A systematic review of the literature was performed in order to identify all the available data concerning the effectiveness of biologic drugs in AOSD. The proportion of patients achieving complete remission or any clinical response was calculated...
November 2017: Expert Review of Clinical Immunology
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