The Experts below are selected from a list of 66 Experts worldwide ranked by ideXlab platform
Pavel Horak - One of the best experts on this subject based on the ideXlab platform.
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adult onset Stills Disease a difficult path to diagnosis through fever and effusions of unknown origin
Vnitr̆ní lékar̆ství, 2014Co-Authors: Jana Malegova, Lukas Koten, Pavel HorakAbstract:Fever of unknown origin, pleural and pericardial effusions can be caused by a variety of independent agents. On the other hand, we can identify a common causative condition in other cases. Infectious Diseases, malignancies and autoimmune Diseases are the most common etiological factors. Considering the pleural and pericardial effusion, we also have to think of cardiovascular and pulmonary Diseases. The basis of every diagnostic process is thorough medical history and detailed clinical examination followed by laboratory and imaging methods. In spite of that, the right diagnosis sometimes stays long time hidden. One of such conditions is Adult-onset Stills Disease (AOSD). It is a rare inflammatory, potentially life-threatening Disease with unclear pathogenesis and heterogeneous symptoms. It has some features similar to systemic form of juvenile idiopathic arthritis. Diagnosis is established so called per exclusionem by fulfilling a set of clinical criteria and ruling out other Diseases with similar symptomatology. In our article, we present an example of such an arduous diagnostic journey to final diagnosis.
Sakin Abdullah - One of the best experts on this subject based on the ideXlab platform.
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A rare cause of pleural effusion: adult onset Stills Disease
2015Co-Authors: Demirbas Soner, Kutlu Orkide, Bahar Kandemir, Sakin AbdullahAbstract:Adult onset Stills Disease is a rare systemic inflammatory disorder. At the onset of the Disease sore throat, pharyngitis; which does not respond to antibiotics, one or two times peaking febrile episodes, marked salmoncolored rash on the trunk and extremities, arthralgia, arthritis, myalgia, fatigue, loss of appetite with nausea and weight loss; hepatosplenomegaly and lymphadenopathy can be seen. Among laboratory examinations levels of ferritin and other acute phase reactants distinctly rise, and neutrophilic leukocytosis; ANA and RF negativity are detected. Pleural and pericardial effusions, transient pulmonary infiltration, and rarely myocarditis can be seen during the course of the Disease. Here we report a patient who was examined for fever of unknown origin and diagnosed with adult onset Stills Disease which is a rare etiology of pleural effusion
Rodica Mihaescu - One of the best experts on this subject based on the ideXlab platform.
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adultonset Stills Disease presenting as persisten t fever of unknown origin case report and review of literature
2013Co-Authors: Ioana Mozos, Elena Sirbu, Rodica MihaescuAbstract:Adult onset Still’s Disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology that can raise serious positive and differential diagnosis problems due to the particular complexity of clinical and biological data. One of the most common presentations of the Disease is fever of unknown origin. In Romania is also very rare, therefore we present a case report of AOSD who became the first case to be notified in our hospital until now. A 24-year-old woman with persistent fever of unknown etiology, rash, arthralgia and myalgia of three weeks duration presented to our hospital. The purpose of this presentation is to describe a case which put uncertainty on differential diagnosis and to review the literature about AOSD from a primary care perspective.
Jana Malegova - One of the best experts on this subject based on the ideXlab platform.
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adult onset Stills Disease a difficult path to diagnosis through fever and effusions of unknown origin
Vnitr̆ní lékar̆ství, 2014Co-Authors: Jana Malegova, Lukas Koten, Pavel HorakAbstract:Fever of unknown origin, pleural and pericardial effusions can be caused by a variety of independent agents. On the other hand, we can identify a common causative condition in other cases. Infectious Diseases, malignancies and autoimmune Diseases are the most common etiological factors. Considering the pleural and pericardial effusion, we also have to think of cardiovascular and pulmonary Diseases. The basis of every diagnostic process is thorough medical history and detailed clinical examination followed by laboratory and imaging methods. In spite of that, the right diagnosis sometimes stays long time hidden. One of such conditions is Adult-onset Stills Disease (AOSD). It is a rare inflammatory, potentially life-threatening Disease with unclear pathogenesis and heterogeneous symptoms. It has some features similar to systemic form of juvenile idiopathic arthritis. Diagnosis is established so called per exclusionem by fulfilling a set of clinical criteria and ruling out other Diseases with similar symptomatology. In our article, we present an example of such an arduous diagnostic journey to final diagnosis.
Bella Mehta - One of the best experts on this subject based on the ideXlab platform.
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op0083 temporal trends and mortality of hospitalisedpatients with adult onset Stills Disease a nationwide estimate
Annals of the Rheumatic Diseases, 2018Co-Authors: Bella Mehta, W Briggs, Petros EfthimiouAbstract:Background There is a dearth of epidemiological studies on Adult Onset Still’s Disease (AOSD). Majority of studies are from single centres or are regional. The largest AOSD epidemiological study till date describes 512 patients.1 Objectives To describe the demographics, complications, mortality and trends of hospitalised patients with AOSD in United States. Also, to understand the factors associated with mortality in these patients. Methods All adult (>18 years) hospitalised patients between 2009 and 2013 from a nationwide inpatient sample (NIS) database were captured. AOSD patients were identified using the ICD-9 code 714.2. Patients also coded for Rheumatoid Arthritis, Lupus, Myositis, Polymyalgia Rheumatica, Ankylosing Spondylosis and Psoriatic Arthritis were excluded. This was done in order to capture patients with strictly AOSD. NIS is the largest all-payer inpatient care database in the United States with approximately 8 million hospitalizations each year. Discharge weights were used to enable nationwide estimates. Descriptive statistics were represented as means/medians for continuous and as frequencies and percentages for categorical variables. A survey weighted logistic regression model was used to describe the associations with in-hospital death. Results Between 2009 and 2013, 5,820 AOSD patients were hospitalised (table 1). AOSD patients had a mean age of 53.6 (SE – 0.61) years, 3817 (65.6%) were females. The racial/ethnic distribution showed that 56% white, 15% African American, 11.7% Hispanic and 3% Asian patients were affected. Over the years, the number of white patients that are hospitalised seems to have increased. 37.6% were hospitalised in urban teaching hospitals. 100 (1.7%) of patients developed Macrophage Activating Syndrome (MAS). 66 (1.1%) patients had disseminated intravascular coagulation (DIC) and 25 (0.4%) had thrombotic thrombocytopenic purpura (TTP). Mean length of stay was 6.9 days. There were 154 inpatient deaths in 5 years (mortality 2.6%). The patients who died in the hospital had a higher mean age of 62.4 (SE- 3.1) years, had a higher proportion of Asians (13.9%) and had increased number of comorbid conditions. Asians had 6.4 times the risk of in-hospital death compared to Whites. The risk for in-hospital death is 30 times higher if a AOSD patient had concurrent DIC. Conclusions In hospitalised American AOSD patients, the average age was higher than previously described in cross sectional studies. This may indicate an ageing population with a higher number of comorbidities that justify hospitalisation. More patients were in large or urban teaching hospitals compared to small or rural hospitals. In-hospital death was associated with increased comorbid conditions and was significantly higher among Asians and patients with DIC. To our knowledge, this is the largest epidemiological study of AOSD. Reference [1] Sakata N, et al. Rheumatol Int2016;36(10):1399–405. doi:10.1007/s00296-016-3546-8 Disclosure of Interest None declared