Adult Onset Still's Disease and Autoinflammation
1Lincoln Medical & Mental Health Center; Division of Rheumatology, Weill Cornell Medical College, Cornell University, New York, NY 10021, USA
2Department of Rheumatology, Hospital Pitié-Salpétrière, 47-83 Boulevard de l'Hôpital, 75013 Paris, France
3Department of Experimental Physiology, School of Medicine, University of Athens, Athens, Greece
4Regeneron; Rockefeller University, New York, NY, USA
5Robert Wood Johnson Medical School, UMDNJ, New Brunswick, NJ, USA
Adult Onset Still's Disease and Autoinflammation
Description
Adult Onset Still's Disease (AOSD) is a rare systemic inflammatory disease characterized by fever, rash, and arthritis. The exact pathogenesis and etiologic factors responsible for the clinical features remain to be determined. Recent immunological research has been carried out to evolve the well-documented role of systemic inflammation and immunoregulatory abnormalities by implicating the inflammasome into the disease pathogenesis. Laboratory abnormalities that are nonspecific but may be indicative of the disease include leukocytosis, elevation of ferritin, and other acute phase reactants. Diagnosis is basically clinical and requires exclusion of infection, malignancy, or other rheumatic disorders. The mainstay therapy initially involves corticosteroids and methotrexate irrespectively of the disease pattern. In refractory cases, biologic agents like anti-TNF, anti-IL1, and anti-IL6 have been used with promising results.
We invite authors to submit original research articles and review articles describing the complex pathophysiology, protean clinical manifestations, and current and emerging therapies for AOSD. Manuscripts on the closely related juvenile disease form, that is, systemic onset Juvenile Idiopathic Arthritis (soJIA) will also be considered. Potential topics include, but are not limited to:
- The pathophysiology of the autoinflammatory diseases, where AOSD belongs, including data on the following:
- The regulation of the inflammasome
- The interplay of Th1/Th17 immunity and cytokine biology
- The epidemiology of AOSD
- The diverse clinical manifestations and complications of AOSD and any form of therapy for AOSD including medications targeting specific organs/symptoms
- The overall AOSD prognosis in terms of morbidity and mortality
- The available disease modifying therapies including immunomodulatory treatments and/or biologic therapies
- The potential evolution of therapies based on preclinical, clinical, or translational studies related to new therapies for AOSD
Manuscripts may also focus on the diverse clinical manifestations of AOSD and any form of therapy for AOSD including medications targeting specific organs/symptoms; disease modifying therapies; immunomodulatory treatments; and/or biologic therapies. Manuscripts can describe preclinical or clinical studies related to new therapies for AOSD.
Articles published in this special issue will not be subject to the journal's Article Processing Charges.
Before submission authors should carefully read over the journal's Author Guidelines, which are located at http://www.hindawi.com/journals/iji/guidelines/. Prospective authors should submit an electronic copy of their complete manuscript through the journal Manuscript Tracking System at http://mts.hindawi.com/ according to the following timetable: