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adult onset stills disease

Lijun Liu, Shengyun Liu, Jingbo Su
No abstract text is available yet for this article.
March 13, 2018: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
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
Ali Sibtain Farooq Sheikh, Jonathan Marks, Neil Hopkinson
Adult-onset Still's disease is a systemic autoinflammatory disease the presentation of which can often mimic infection. As a consequence, there is often a delay in diagnosis. Serositis is a recognised but less common clinical feature that can result in complications including cardiac tamponade and constrictive pericarditis. We describe a case of adult-onset Still's disease without the hallmark rash or significant arthritis, presenting with polyserositis that showed a good response to initial steroid treatment and sustained remission with anakinra...
January 1, 2018: Scottish Medical Journal
Ankur Kumar Jindal, Ashish Agarwal, Sandesh Guleria, Deepti Suri, Mini P Singh, Saniya Sharma, Shano Naseem, Radha Kant Ratho
No abstract text is available yet for this article.
February 27, 2018: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
N Veronese, B Stubbs, M Solmi, T O Smith, J-Y Reginster, S Maggi
OBJECTIVES: Although osteoarthritis (OA) is a common condition in older adults, the role of OA in increasing cardiovascular disease (CVD) incidence is still debated. The aim of this study was to investigate the association between OA and the onset of CVD in a large database of American adults. DESIGN: Longitudinal. SETTING: Community-dwelling. PARTICIPANTS: People with OA or at high risk of OA. MEASUREMENTS: Osteoarthritis was defined as the presence of OA of the hand, knee, hip, back/neck or of other sites...
2018: Journal of Nutrition, Health & Aging
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
Katerina Theodoropoulou, Sophie Georgin-Lavialle
Still's disease is a rare multifactorial disease associated with systemic inflammation. Systemic-onset juvenile idiopathic arthritis and adult-onset Still's disease are both pediatric and respectively adult forms of the disease with a cut-off age of 16 years. The disease is characterized by the following features : hectic fever > 39° C, arthralgia or arthritis, rash, neutrophilia and systemic inflammation. The prognosis of the disease is functional and vital. The evolution over time is variable : regression, evolution by relapses with regression at term and chronic joint evolution...
February 14, 2018: Revue Médicale Suisse
Carla Bizzarri, Tiziana Antonia Timpanaro, Maria Cristina Matteoli, Ippolita Patrizia Patera, Marco Cappa, Stefano Cianfarani
BACKGROUND: Linear growth was reported to be negatively affected by type 1 diabetes mellitus (T1DM), in relation to disease duration and poor metabolic control. It is unclear whether a subtle growth failure still persists despite the optimization of therapy. Our aim was to analyse pubertal growth, adult height, and metabolic profile in a cohort of children with T1DM undergoing intensive insulin treatment by multiple daily injections or continuous subcutaneous insulin infusion (CSII). METHODS: One-hundred and four children (51 males) with prepubertal onset of T1DM were prospectively followed up to final height attainment...
February 16, 2018: Hormone Research in Pædiatrics
Zhe Hou, Brian R Hinds, Philip R Cohen
Neutrophilic urticarial dermatosis (NUD) is a useful diagnostic term for urticarial lesions that are less pruritic and more painful than conventional urticaria. The histopathologic features include neutrophilic infiltrates in the interstitial dermis with a higher density than idiopathic urticaria. NUD has been associated with several systemic conditions, which are predominantly autoimmune and autoinflammatory in nature. A woman with Crohn disease who developed NUD is described. Literature reports of other conditions in which neutrophilic urticarial dermatosis have been observed are also reviewed and summarized...
November 15, 2017: Dermatology Online Journal
Izet Masic, Zijo Begic, Nabil Naser, Edin Begic
Pediatrics is defined as the science of a healthy and sick child from birth to end of adolescence. Diseases of the cardiovascular system are the leading causes of mortality in adults, with frequent onset in childhood. The cardiologic examination starts with anamnesis in a pleasant atmosphere, refined space, enough time and patience, detailed measurements, and preferably a noncrying child. Anamnesis, regardless of the development of diagnostic procedures, still constitutes the basis of every clinical examination...
2018: International Journal of Preventive Medicine
Yakeel T Quiroz, Reisa A Sperling, Daniel J Norton, Ana Baena, Joseph F Arboleda-Velasquez, Danielle Cosio, Aaron Schultz, Molly Lapoint, Edmarie Guzman-Velez, John B Miller, Leo A Kim, Kewei Chen, Pierre N Tariot, Francisco Lopera, Eric M Reiman, Keith A Johnson
Importance: It is critically important to improve our ability to diagnose and track Alzheimer disease (AD) as early as possible. Individuals with autosomal dominant forms of AD can provide clues as to which and when biological changes are reliably present prior to the onset of clinical symptoms. Objective: To characterize the associations between amyloid and tau deposits in the brains of cognitively unimpaired and impaired carriers of presenilin 1 (PSEN1) E280A mutation...
February 12, 2018: JAMA Neurology
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)
Arun Agarwal, Mudit Agarwal, Vipul Khandelwal, Priya Mathur
No abstract text is available yet for this article.
February 1, 2018: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
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)
Roni S Moran, Daniel S Moran, Gil Fire
The World Health Organization (WHO) has declared obesity a global epidemic. WHO sheds much light on this matter in its publications on health promotion and preventative medicine. Lack of physical activity, an unbalanced diet and an unhealthy lifestyle are the leading causes of developing obesity and chronic diseases. In Israel, the growing rate of obesity is a reason for concern. About 500,000 diabetics, mainly as a result of obesity, live in Israel today and by 2030 the number is expected to rise to 2,000,000...
January 2018: Harefuah
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
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