Article
Immunology
Quentin Bodard, Vincent Langlois, Philippe Guilpain, Alain Le Quellec, Olivier Vittecoq, David Noel, Vincent Eble, Severine Josse, Jean Schmidt, Achille Aouba, Herve Levesque, Maelle Le Besnerais, Ygal Benhamou
Summary: Cardiac complications in patients with Adult-onset Still's disease (AOSD) are common, often life-threatening, and predictive of a refractory disease course. Systematic cardiac screening at diagnosis and early consideration of biologic therapy can improve patient outcomes.
JOURNAL OF AUTOIMMUNITY
(2021)
Review
Rheumatology
Ryohei Ono, Togo Iwahana, Shuji Toriumi, Kaoruko Aoki, Hirotoshi Kato, Kodai Kato, Masahiro Yasui, Yuzuho Nakagawa, Shunsuke Furuta, Hiroshi Nakajima, Yoshio Kobayashi
Summary: This study reports a rare case of a 43-year-old female with fulminant myocarditis with adult-onset Still's disease (AOSD). The patient had refractory AOSD and cardiogenic shock with significantly elevated ferritin levels. The case was successfully treated with canakinumab and mechanical circulatory support. A review of previous cases shows that myocarditis may be an early complication of AOSD, and the severity of AOSD may correlate with the severity of myocarditis.
CLINICAL RHEUMATOLOGY
(2023)
Article
Rheumatology
Corrado Campochiaro, Nicola Farina, Alessandro Tomelleri, Giacomo De Luca, Elena Baldissera, Giulio Cavalli, Lorenzo Dagna
Summary: The study assessed drug retention rates and reasons for discontinuation of biologic disease-modifying antirheumatic drugs in patients with adult onset Still's disease. The results showed that TCZ and ANK had similar and significantly higher drug retention rates compared to TNFi, and previous use of biologic agents did not affect drug retention rates.
SEMINARS IN ARTHRITIS AND RHEUMATISM
(2021)
Article
Medicine, General & Internal
Maurits A. Sikking, Sophie L. V. M. Stroeks, Michiel T. H. M. Henkens, Max F. G. H. M. Venner, Xiaofei Li, Stephane R. B. Heymans, Mark R. Hazebroek, Job A. J. Verdonschot
Summary: Dilated cardiomyopathy (DCM) has a genetic cause in up to 40% of cases, and the presence of cardiac inflammation is associated with a younger disease onset in genetic DCM patients. However, cardiac inflammation does not increase the risk of mortality, heart failure hospitalization, or life-threatening arrhythmias. This suggests that myocarditis may be an exogeneous trigger for DCM in patients with a genetic susceptibility or that cardiac inflammation represents an early phase of the disease.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Medicine, Research & Experimental
Larissa Valor-Mendez, Bernhard Manger, Alexander Cavallaro, Stephan Achenbach, Georg Schett, Jurgen Rech
Summary: This case report describes a 68-year-old Caucasian male initially diagnosed with acute endocarditis leading to severe aortic insufficiency, ultimately diagnosed with AOSD. The patient was treated with anakinra, but later developed dilated cardiomyopathy and gouty arthritis. Treatment required dosage adjustments, and the patient eventually passed away due to deteriorating cardiac disease.
EUROPEAN JOURNAL OF MEDICAL RESEARCH
(2021)
Review
Biochemistry & Molecular Biology
Luana Andreea Macovei, Alexandra Burlui, Ioana Bratoiu, Ciprian Rezus, Anca Cardoneanu, Patricia Richter, Andreea Szalontay, Elena Rezus
Summary: Adult-onset Still's disease is a systemic inflammatory disorder with unknown etiology, characterized by symptoms such as high fever, lymphadenopathy, hepatosplenomegaly, hyperferritinemia, and leukocytosis. Treatment options include NSAIDs, corticosteroids, csDMARDs, and biological therapy targeting various inflammatory cytokines.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Medicine, General & Internal
Toluwalase Awoyemi, Alexandra Conti, Frank G. Aguilar
Summary: This case report describes a young postpartum woman who was diagnosed with adult-onset Still's disease (AOSD) complicated by macrophage activation syndrome (MAS). The patient presented with a pruritic maculopapular rash, cytopenias, and elevated ferritin levels. The unique aspect of this case is the belief that pregnancy triggered the development of AOSD, as evidenced by the characteristics of the rash and the presence of MAS.
CLINICAL CASE REPORTS
(2023)
Review
Rheumatology
Yuning Ma, Jianfen Meng, Jinchao Jia, Mengyan Wang, Jialin Teng, Dehao Zhu, Chengde Yang, Qiongyi Hu
Summary: Adult-onset Still's disease is a rare auto-inflammatory disorder characterized by high fever, skin rash, polyarthritis, and other symptoms. Treatment options have expanded beyond traditional medications to include anti-cytokine biologic agents.
Review
Medicine, General & Internal
Stylianos Tomaras, Carl Christoph Goetzke, Tilmann Kallinich, Eugen Feist
Summary: Adult-onset Still's disease is a rare systemic autoinflammatory disease characterized by arthritis, spiking fever, skin rash, and elevated ferritin levels. Immune dysregulation plays a central role in the disease, involving pathogenic involvement of both arms of the immune system. Extensive research has led to a better understanding of AoSD and revolutionary advances in treatment.
JOURNAL OF CLINICAL MEDICINE
(2021)
Article
Immunology
Jianfen Meng, Yuning Ma, Jinchao Jia, Mengyan Wang, Jialin Teng, Hui Shi, Honglei Liu, Yutong Su, Junna Ye, Yue Sun, Xiaobing Cheng, Huihui Chi, Tingting Liu, Dehao Zhu, Zhuochao Zhou, Liyan Wan, Zhihong Wang, Fan Wang, Xin Qiao, Xia Chen, Hao Zhang, Zihan Tang, Chengde Yang, Qiongyi Hu
Summary: This study compared the cytokine and ferritin levels between severe COVID-19 and active AOSD patients, revealing significant differences in IL-6, IL-10, and ferritin levels, laying the groundwork for developing therapeutic strategies targeting the hyperinflammatory process in COVID-19.
FRONTIERS IN IMMUNOLOGY
(2021)
Review
Rheumatology
Bruno Fautrel, Jacoby Patterson, Catherine Bowe, Mick Arber, Julie Glanville, Stuart Mealing, Viviam Canon-Garcia, Laura Fagerhed, Hilde Rabijns, Roberto Giacomelli
Summary: This systematic review evaluated the efficacy and safety of biologic disease modifying anti-rheumatic drugs (bDMARDs) for adult-onset Still's disease (AOSD). The review included three randomized controlled trials, one retrospective case series, and 17 case series. Limited data from randomized controlled trials showed that bDMARDs may be effective compared to conventional DMARDs or placebo. However, the lack of standardized assessment criteria hindered comparisons between studies. Uncontrolled retrospective case series suggested that bDMARDs were effective in AOSD, but no comparative data were available. Standardization of therapeutic response definition and larger comparative studies are needed to determine the most effective bDMARDs for AOSD.
SEMINARS IN ARTHRITIS AND RHEUMATISM
(2023)
Review
Medicine, General & Internal
Nicolas Poursac, Itsaso Odriozola, Marie-Elise Truchetet
Summary: Adult-onset Still's disease is a complex autoinflammatory disease with a multifactorial etiology. This review provides a detailed overview of the different complementary tests used in diagnosing and managing the disease, including their value in positive diagnosis, differential diagnosis, and screening for complications. A structured algorithm is proposed for clinical practice, and potential new complementary examinations are discussed.
JOURNAL OF CLINICAL MEDICINE
(2022)
Article
Rheumatology
Giacomo De Luca, Anna Palmisano, Corrado Campochiaro, Davide Vignale, Giulio Cavalli, Elisa Bruno, Silvia Sartorelli, Arianna Ferlito, Giovanni Peretto, Simone Sala, Marco Matucci-Cerinic, Lorenzo Dagna, Antonio Esposito
Summary: This study investigates the performance of different mapping techniques included in the revised Lake Louise criteria for the identification of myocarditis in systemic sclerosis (SSc) patients. The results show that T2 mapping has good diagnostic accuracy in recognizing myocardial inflammation in SSc.
Article
Rheumatology
Mengzhu Zhao, Di Wu, Min Shen
Summary: A total of 187 patients with adult-onset Still's disease (AOSD) were studied, revealing a 7.5% prevalence of neurological involvement. Common symptoms included fever, rash, liver dysfunction, arthralgia/arthritis, and lymphadenopathy. Treatment strategies mainly consisted of high-dose corticosteroids and immunosuppressive agents, with a 100% clinical remission rate.
Article
Rheumatology
Helen L. Leavis, Paul L. A. van Daele, Catharina Mulders-Manders, Renee Michels, Abraham Rutgers, Elizabeth Legger, Marc Bijl, Elisabeth A. Hak, Wai-Kwan Lam-Tse, Femke Bonte-Mineur, Peter Fretter, Anna Simon, Pieter van Paassen, Marlies C. van der Goes, Marcel Flendrie, Ward Vercoutere, Antoine W. T. van Lieshout, Arjen Leek, Sebastiaan J. Vastert, Sander W. Tas
Summary: This study used a Delphi panel to reach consensus on diagnostic and treatment strategies for adult-onset Still's disease. The panel agreed on recommendations for diagnosis and therapy, including the use of biologics and reduction of glucocorticoid use. The results can serve as a basis for a treatment guideline.
