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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
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
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
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
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)
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
Hyoun-Ah Kim, Jae Ho Han, Woo-Jung Kim, Hyun Jin Noh, Jeong-Mi An, Hyunee Yim, Ju-Yang Jung, You-Sun Kim, Chang-Hee Suh
S100A8/A9 has been suggested as a marker of disease activity in patients with adult-onset Still's disease (AOSD). We evaluated the clinical significance of S100A8/A9 as a biomarker and its pathogenic role in AOSD. Blood samples were collected prospectively from 20 AOSD patients and 20 healthy controls (HCs). Furthermore, skin and lymph node biopsy specimens of AOSD patients were investigated for S100A8/A9 expression levels via immunohistochemistry. Peripheral blood mononuclear cells (PBMCs) of active AOSD patients and HCs were investigated for S100A8/A9 cell signals...
2016: International Journal of Molecular Sciences
Nobuo Sakata, Sayuri Shimizu, Fumio Hirano, Kiyohide Fushimi
Adult-onset Still's disease (AOSD) is a rare disease, and large epidemiological studies of this disease are limited. Furthermore, it has been difficult to show the incidence and characteristics of severe AOSD complications due to the rarity of this disease. The aim of our study was to describe the demographics of AOSD and the incidence and characteristics of severe complications. Using a large Japanese administrative database, we identified hospitalized patients with AOSD and described the demographics. We also calculated the incidence of severe complications (i...
October 2016: Rheumatology International
Mathieu Gerfaud-Valentin, Vincent Cottin, Yvan Jamilloux, Arnaud Hot, Agathe Gaillard-Coadon, Isabelle Durieu, Christiane Broussolle, Jean Iwaz, Pascal Sève
Parenchymal lung involvement (PLI) in adult-onset Still's disease (AOSD) has seldom, if ever, been studied. We examine here retrospective cohort AOSD cases and present a review of the literature (1971-2014) on AOSD-related PLI cases.Patients with PLI were identified in 57 AOSD cases. For inclusion, the patients had to fulfill Yamaguchi or Fautrel classification criteria, show respiratory symptoms, and have imaging evidence of pulmonary involvement, and data allowing exclusion of infectious, cardiogenic, toxic, or iatrogenic cause of PLI should be available...
July 2016: Medicine (Baltimore)
Seung Taek Song, Jin Ju Kim, Seung Lee, Hyoun-Ah Kim, Eun Young Lee, Ki Chul Shin, Joo Hyun Lee, Kwang-Hoon Lee, Sang Tae Choi, Hoon-Suk Cha, Dae-Hyun Yoo
OBJECTIVES: To evaluate the efficacy of tocilizumab (TCZ), a monoclonal antibody against the interleukin (IL)-6 receptor, for refractory adult-onset Still's disease (AOSD) in the Korean population. METHODS: This retrospective study included 22 Korean patients with refractory AOSD who were given TCZ at one of seven university hospital-based clinics for rheumatic disease. Patients were subdivided into groups according to disease course: monocyclic, systemic polycyclic, and chronic articular...
July 26, 2016: Clinical and Experimental Rheumatology
Jason Weatherald, Johan Lategan, Doug Helmersen
Adult onset Still's disease (AOSD) is an autoimmune disease characterized by systemic inflammation and is a rarely reported cause of pulmonary arterial hypertension (PAH). We describe the clinical course of a 40-year-old woman who presented with PAH 19 months after a diagnosis of AOSD. Sildenafil and immunosuppressive therapy with cyclosporine resulted in clinical and hemodynamic improvement with long-term survival 15 years after her initial presentation of AOSD. We review the literature for published cases of PAH due to AOSD and discuss the potential mechanisms relating inflammatory diseases and PAH...
2016: Respiratory Medicine Case Reports
E D Batu, A Erden, E Seyhoğlu, L Kilic, Y Büyükasık, O Karadag, Y Bilginer, S A Bilgen, A Akdogan, S Kiraz, A I Ertenli, S Özen, U Kalyoncu
OBJECTIVES: Reactive haemophagocytic syndrome (RHS) is a hyperinflammatory disorder often occurring in the background of several disorders such as infections, malignancies, and rheumatic diseases. Recently, a score known as the HScore was developed for the diagnosis of RHS. In the original study, most of the patients had underlying haematological malignancy or infection and the best cut-off value for the HScore was 169 (sensitivity 93%; specificity 86%). In this study we aimed to analyse the performance of the HScore in rheumatic disease-related RHS...
June 30, 2016: Scandinavian Journal of Rheumatology
P Ruscitti, P Cipriani, F Ciccia, P Di Benedetto, V Liakouli, O Berardicurti, F Carubbi, G Guggino, S Di Bartolomeo, G Triolo, R Giacomelli
Adult-onset Still's disease (AOSD) patients may show an evanescent salmon-pink erythema appearing during febrile attacks and reducing without fever. Some patients may experience this eruption for many weeks. During AOSD, exceptionally high serum levels of ferritin may be observed; it is an iron storage protein composed of 24 subunits, heavy (H) subunits and light (L) subunits. The ferritin enriched in L subunits (L-ferritin) and the ferritin enriched in H subunits (H-ferritin) may be observed in different tissues...
October 2016: Clinical and Experimental Immunology
Natsumi Inoue, Masaki Shimizu, Shinichiro Tsunoda, Mitsuhiro Kawano, Masami Matsumura, Akihiro Yachie
To compare pro-inflammatory cytokine profiles and kinetics in patients with adult-onset Still's disease (AOSD) to those in patients with systemic juvenile idiopathic arthritis (s-JIA), we analyzed serum cytokine concentrations in 33 patients with AOSD and 77 patients with s-JIA and compared them with clinical features. Patients with AOSD and s-JIA shared a common cytokine profile pattern of a significant increase in IL-18. Patients with AOSD were classified into two subgroups based on serum IL-6 and IL-18 levels...
August 2016: Clinical Immunology: the Official Journal of the Clinical Immunology Society
Ju-Yang Jung, Chang-Hee Suh, Seonghyang Sohn, Jin-Young Nam, Hyoun-Ah Kim
High-mobility group box-1 (HMGB1) is a nuclear protein, and such prototypical damage-associated molecular patterns mediate the immune response in the noninfectious inflammatory response. Adult-onset Still's disease (AOSD) is a systemic inflammatory disorder involved in the dysregulation of innate immunity. We investigated the serum HMGB1 level in patients with AOSD and evaluated its clinical significance. Blood samples were collected from 40 patients with active AOSD and 40 healthy controls (HC). Of the patients with AOSD, follow-up samples were collected from 16 patients after a resolution of AOSD disease activity...
August 2016: Clinical Rheumatology
Paolo Sfriso, Roberta Priori, Guido Valesini, Silvia Rossi, Carlo Maurizio Montecucco, Anna D'Ascanio, Linda Carli, Stefano Bombardieri, Gaetana LaSelva, Florenzo Iannone, Giovanni Lapadula, Stefano Alivernini, Gianfranco Ferraccioli, Michele Colaci, Clodoveo Ferri, Daniela Iacono, Gabriele Valentini, Luisa Costa, Raffaele Scarpa, Andrea LoMonaco, Valentina Bagnari, Marcello Govoni, Ilaria Piazza, Silvano Adami, Francesco Ciccia, Giovanni Triolo, Elisa Alessandri, Maurizio Cutolo, Luca Cantarini, Mauro Galeazzi, Piero Ruscitti, Roberto Giacomelli, Francesco Caso, Paola Galozzi, Leonardo Punzi
Adult-onset Still's disease (AOSD) is a systemic inflammatory condition of unknown aetiology characterized by typical episodes of spiking fever, evanescent rash, arthralgia, leukocytosis and hyperferritinemia. Given the lack of data in Italian series, we promote a multicentric data collection to characterize the clinical phenotype of Italian patients with AOSD. Data from 245 subjects diagnosed with AOSD were collected by 15 centres between March and May 2013. The diagnosis was made following Yamaguchi's criteria...
July 2016: Clinical Rheumatology
Mili V Mehta, Daniel K Manson, Evelyn M Horn, Jennifer Haythe
Pulmonary arterial hypertension (PAH) is a known complication of rheumatologic diseases, but it is only rarely associated with adult-onset Still's disease (AOSD). We describe the case of a 30-year-old woman who presented in a pulmonary hypertension crisis and was found to have underlying AOSD with PAH and nonspecific interstitial pneumonia (NSIP) with a course complicated by macrophage activation syndrome (MAS). She dramatically improved with steroids, cyclosporine A, and anakinra, with total resolution of the MAS and significant improvement of her pulmonary arterial pressures...
March 2016: Pulmonary Circulation
L Guilleminault, S Laurent, A Foucher, P Poubeau, F Paganin
BACKGROUND: Adult onset of Still's disease (AOSD) is a rare systemic inflammatory disease. Cardiorespiratory complications are mainly represented by pleural and pericardial disorders and are less frequent than cutaneous and articular complaints. Pulmonary arterial hypertension (PAH) occurring in AOSD is rarely described in literature. CASE PRESENTATION: We present the case of a young patient who developed severe PAH 2 years after diagnosis of AOSD. This is a rare and severe complication which is probably underestimated...
2016: BMC Pulmonary Medicine
Xin Yu, Heyi Zheng
Adult-onset Still disease (AOSD) is a systemic autoimmune disease (AIID) that can develop after exposure to infectious agents. Genital human papillomavirus (HPV) infection has been reported to induce or exacerbate AIIDs, such as systemic lupus erythematosus (SLE). No guidelines are available for the management of genital warts in AOSD. Case report and literature review. We report a patient who was diagnosed AOSD in the setting of refractory and recurrent genital HPV infection, demonstrating a possible link between HPV infection and AOSD...
April 2016: Medicine (Baltimore)
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