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adult onset still's disease

Ahmad Z Qureshi, Mohammad AlSheef, Waqas T Qureshi, Waseem Amjad
Adult onset Still's disease (AOSD) is a chronic inflammatory disorder involving multiple systems. The symptoms mimic those of lymphomas, therefore, the diagnosis of lymphoma needs to be excluded prior to establishing the diagnosis of AOSD. Another similar condition is dermatopathic lymphadenopathy (DL). In DL, the histopathological appearance of lymph node biopsy may also mimic AOSD. The DL is associated with several systemic pathologies, such as malignant lymphomas, and rarely AOSD. We present a case of a 43-year-old male presented with 3 months history of fatigue, fever, and lymphadenopathy...
November 2016: Saudi Medical Journal
Filipa Oliveira-Ramos, Mónica Eusébio, Fernando M Martins, Ana Filipa Mourão, Carolina Furtado, Raquel Campanilho-Marques, Inês Cordeiro, Joana Ferreira, Marcos Cerqueira, Ricardo Figueira, Iva Brito, Helena Canhão, Maria José Santos, José A Melo-Gomes, João Eurico Fonseca
OBJECTIVES: To determine how adult juvenile idiopathic arthritis (JIA) patients fulfil classification criteria for adult rheumatic diseases, evaluate their outcomes and determine clinical predictors of inactive disease, functional status and damage. METHODS: Patients with JIA registered on the Rheumatic Diseases Portuguese Register ( older than 18 years and with more than 5 years of disease duration were included. Data regarding sociodemographic features, fulfilment of adult classification criteria, Health Assessment Questionnaire, Juvenile Arthritis Damage Index-articular (JADI-A) and Juvenile Arthritis Damage Index-extra-articular (JADI-E) damage index and disease activity were analysed...
2016: RMD Open
Erica Lin, Isabel A Hujoel, Thomas G Mason
This manuscript describes an original case report with key words adult-onset Still's Disease and fever curve. It is not under consideration for submission or publication by any other journal. All authors contributed to the preparation of this manuscript. All authors approved the manuscript and this submission and can be reached through the email addresses, listed below. All authors declare that they have no conflicts of interest. The authors report no proprietary or commercial interest in any product mentioned or concept discussed in this article...
October 14, 2016: American Journal of Medicine
Bikkina Ghana Syam, K Jayachandran, Tolstoy
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
Christeena J, Vineeta Shobha, Jyothi Idiculla, Soumya Umesh
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
Stephanie R Harrison, Dennis McGonagle, Sharmin Nizam, Stephen Jarrett, Jeroen van der Hilst, Michael F McDermott, Sinisa Savic
BACKGROUND: Some adult patients presenting with unexplained pyrexia, serositis, skin rashes, arthralgia, myalgia, and other symptoms commonly found in autoinflammatory disorders may not fit a specific diagnosis, either because their clinical phenotype is nondiagnostic or genetic tests are negative. We used the term undifferentiated systemic autoinflammatory disorder (uSAID) to describe such cases. Given that well-defined autoinflammatory diseases show responses to IL-1 blockade, we evaluated whether anakinra was useful for both diagnosing and treating uSAID patients...
May 5, 2016: JCI Insight
K Dahan
Membranous nephropathy (MN) accounts for about 20% of cases of nephrotic syndrome in the adult. Thickening of glomerular capillary walls results from subepithelial formation of immune deposits containing IgG and the membrane attack complex of complement, which is the major mediator of proteinuria, and antigens. Idiopathic forms of MN (IMN) represent 70 to 80% of all cases. A major breakthrough was the identification of the podocyte antigen PLA2R as the target of circulating antibodies in about 70% of IMN, which confirmed that the disease was auto-immune in nature...
May 25, 2016: La Revue de Médecine Interne
Eri Watanabe, Hitoshi Sugawara, Takeshi Yamashita, Akira Ishii, Aya Oda, Chihiro Terai
We report the case of a 71-year-old Japanese woman with adult-onset Still's disease (AOSD) in whom macrophage activation syndrome (MAS) developed despite therapy with oral high-dose prednisolone and intravenous methylprednisolone pulse therapy twice. She was successfully treated with tocilizumab (TCZ). Soon afterward, her fever ceased and high levels of both ferritin and C-reactive protein levels decreased. Her course was complicated by disseminated intravascular coagulation, cytomegalovirus infection, and Pneumocystis jirovecii pneumonia...
2016: Case Reports in Medicine
Joshua J Minori, Edward H Wieseltier, Cheryl Benjamin, Mary H Lawler
No abstract text is available yet for this article.
September 2016: PM & R: the Journal of Injury, Function, and Rehabilitation
M De Antonio, C Dogan, D Hamroun, M Mati, S Zerrouki, B Eymard, S Katsahian, G Bassez
The broad clinical spectrum of myotonic dystrophy type 1 (DM1) creates particular challenges for both medical care and design of clinical trials. Clinical onset spans a continuum from birth to late adulthood, with symptoms that are highly variable in both severity and nature of the affected organ systems. In the literature, this complex phenotype is divided into three grades (mild, classic, and severe) and four or five main clinical categories (congenital, infantile/juvenile, adult-onset and late-onset forms), according to symptom severity and age of onset, respectively...
October 2016: Revue Neurologique
Charlotte Girard, Jürgen Rech, Michael Brown, Danièle Allali, Pascale Roux-Lombard, François Spertini, Eduardo J Schiffrin, Georg Schett, Bernhard Manger, Sylvette Bas, Greg Del Val, Cem Gabay
OBJECTIVE: IL-18 is a pro-inflammatory cytokine of the IL-1 family that is naturally inhibited by IL-18 binding protein (IL-18BP). High levels of IL-18 have been described in the serum of adult-onset Still's disease (AOSD) patients, but only total IL-18 levels (including inactive IL-18 bound to IL-18BP) have been measured. With a specific immunoassay, we aimed to measure free IL-18 serum levels in AOSD patients and other rheumatic diseases. METHODS: An ELISA was developed to measure free IL-18...
September 10, 2016: Rheumatology
Christian Vogelberg
Asthma is a chronic inflammatory airway disease, and its treatment is frequently challenging despite detailed national and international guidelines. While basic anti-inflammatory therapy usually consists of inhaled corticosteroids in doses adapted to the asthma severity, add-on treatment with bronchodilators is essential in more severe asthma. Only recently, the long-acting anticholinergic tiotropium was introduced into the GINA guidelines. This review reports on the studies that have been performed with tiotropium in adult asthmatic patients...
September 9, 2016: Therapeutic Advances in Respiratory Disease
Jelena Arsenijevic, Wim Groot, Marzena Tambor, Stanislawa Golinowska, Christoph Sowada, Milena Pavlova
BACKGROUND: Health promotion interventions for older adults are important as they can decrease the onset and evolution of diseases and thus can reduce the medical costs related to those diseases. However, there is no comparative evidence on how those interventions are funded in European countries. The aim of this study is to explore the funding of health promotion interventions in general and health promotion interventions for older adults in particular in European countries. METHOD: We use desk research to identify relevant sources of information such as official national documents, international databases and scientific articles...
2016: BMC Health Services Research
Filippo Lococo, Gianluigi Bajocchi, Andrea Caruso, Riccardo Valli, Tommaso Ricchetti, Giorgio Sgarbi, Carlo Salvarani
BACKGROUND: Thymoma is a T cell neoplasm arising from the thymic epithelium that due to its immunological role, frequently undercover derangements of immunity such a tumors and autoimmune diseases. METHODS: Herein, we report, to the best of our knowledge, the first description of an association between thymoma and adult onset Still disease (AOSD) in a 47-year-old man. The first one was occasionally detected 4 years later the diagnosis of AOSD, and surgically removed via right lateral thoracotomy...
September 2016: Medicine (Baltimore)
Pedro Luiz Toledo de Arruda Lourenção, Simone Antunes Terra, Erika Veruska Paiva Ortolan, Maria Aparecida Marchesan Rodrigues
Intestinal neuronal dysplasia type B (IND-B) is a controversial entity among the gastrointestinal neuromuscular disorders. It may occur alone or associated with other neuropathies, such as Hirschsprung's disease (HD). Chronic constipation is the most common clinical manifestation of patients. IND-B primarily affects young children and mimics HD, but has its own histopathologic features characterized mainly by hyperplasia of the submucosal nerve plexus. Thus, IND-B should be included in the differential diagnoses of organic causes of constipation...
August 6, 2016: World Journal of Gastrointestinal Pharmacology and Therapeutics
Cihan Buyukavsar, Ergenekon Karagoz, Murat Sonmez, Taner Kar, Abdullah Kaya, Eyup Düzgun, Yildiray Yildirim
Adult-onset Still's disease (AOSD) is a rare multisystemic immune-mediated disease of unknown etiology with quotidian spiking fever, evanescent rash, arthralgia, and multiple organ involvement. The few AOSD cases that have been reported developed Purtscher's-like retinopathy associated with thrombotic microangiopathy (TMA). Here, we report Purtscher's-like retinopathy without TMA in a patient with AOSD. A 29-year-old-man who presented for evaluation of blurred vision was diagnosed with AOSD based on Yamaguchi criteria...
September 6, 2016: Ocular Immunology and Inflammation
Xiao-Tu Xi, Mao-Jie Wang, Run-Yue Huang, Bang-Han Ding
Adult onset Still's disease (AOSD) is a systemic inflammatory disorder characterized by rash, leukocytosis, fever and arthralgia/arthritis. The most common pulmonary manifestations associated with AOSD are pulmonary infiltrates and pleural effusion. The present study describes a 40-year-old male with AOSD who developed fever, sore throat and shortness of breath. Difficulty breathing promptly developed, and the patient was diagnosed with acute respiratory distress syndrome (ARDS). The patient did not respond to antibiotics, including imipenem, vancomycin, fluconazole, moxifloxacin, penicillin, doxycycline and meropenem, but was sensitive to glucocorticoid treatment, including methylprednisolone sodium succinate...
September 2016: Experimental and Therapeutic Medicine
L Gusdorf, B Asli, S Barbarot, A Néel, A Masseau, X Puéchal, J-E Gottenberg, G Grateau, C Blanchard-Delaunay, R Rizzi, F Lifermann, X Kyndt, F Aubin, D Bessis, T Boye, S Gayet, F Rongioletti, E Sauleau, J-P Fermand, D Lipsker
BACKGROUND: Schnitzler syndrome is characterized by an urticarial rash, a monoclonal gammopathy and clinical, histological and biological signs of neutrophil-mediated inflammation. The aim of this study was to assess the applicability and validity of the existing diagnostic criteria in real life patients. METHODS: This multicentric study was conducted between 2009 and 2014 in 14 hospitals in which patients with Schnitzler syndrome or controls with related disorders were followed-up...
August 26, 2016: Allergy
Savina Apolloni, Paola Fabbrizio, Susanna Amadio, Cinzia Volonté
BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a disease with a strong neuroinflammatory component sustained by activated microglia contributing to motoneuron death. However, how to successfully balance neuroprotective versus neurotoxic actions by the use of antinflammatory agents is still under scrutiny. We have recently shown that the antihistamine clemastine, an FDA-approved drug, can influence the M1/M2 switch occurring in SOD1-G93A ALS microglia. METHODS: Here, we have chronically treated female SOD1-G93A mice with clemastine, evaluated disease progression and performed mice lumbar spinal cord analysis at symptomatic and end stage of the disease...
2016: Journal of Neuroinflammation
Kazeem Olalekan, Andy Fox, Rodney Gilbert
BACKGROUND: Unlicensed medications are used all the time in the management of diseases in childhood. Tolvaptan (Jinarc®) is a vasopressin V2-receptor antagonist licensed for use to slow the progression of cyst development and renal insufficiency of ADPKD in adults with CKD stage 1 to 3 with evidence of rapidly progressing disease. Studies of animal models implicate the antidiuretic hormone arginine vasopressin and its messenger cyclic adenosine monophosphate (cAMP) as promoters of kidney-cyst cell proliferation and luminal fluid secretion...
September 2016: Archives of Disease in Childhood
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