Clinical characteristics and treatment of elderly onset adult-onset Still’s disease

• Published in: Scientific reports 2022-04, Vol.12 (1), p.6787-6787, Article 6787
• Main Authors: Kishida, Dai, Ichikawa, Takanori, Takamatsu, Ryota, Nomura, Shun, Matsuda, Masayuki, Ishii, Wataru, Nagai, Tatsuo, Suzuki, Sadahiro, Ueno, Ken-ichi, Tachibana, Naoki, Shimojima, Yasuhiro, Sekijima, Yoshiki
• Format: Article
• Language: English
• Subjects: 692/4023/1670; 692/699/249/2510; Adult; Age; Aged; Angina pectoris; Cell activation; Cytomegalovirus; Cytomegalovirus Infections - complications; Disseminated intravascular coagulation; Drug dosages; Glucocorticoids; Hospitals; Humanities and Social Sciences; Humans; Hypertension; Immunosuppressive Agents - therapeutic use; Infections; Inflammatory diseases; Laboratories; Macrophage Activation Syndrome - complications; Macrophage Activation Syndrome - drug therapy; Macrophages; Medicine; multidisciplinary; Neutrophils; Patients; Pharyngitis; Pleurisy; Retrospective Studies; Review boards; Rheumatology; Science; Science (multidisciplinary); Still's Disease, Adult-Onset - complications; Still's Disease, Adult-Onset - drug therapy; Still's Disease, Adult-Onset - epidemiology
• ISSN: 2045-2322; 2045-2322
• DOI: 10.1038/s41598-022-10932-3

Adult-onset Still’s disease (AOSD)—a systemic inflammatory disease—often occurs at a young age. Recently, elderly onset patient proportion has been increasing; however, data are limited. To evaluate the characteristics of elderly patients with AOSD in a multicenter cohort, we retrospectively analyzed 62 patients with AOSD at five hospitals during April 2008–December 2020. Patients were divided into two groups according to age at disease onset: younger-onset (≤ 64 years) and elderly onset (≥ 65 years). Clinical symptoms, complications, laboratory findings, treatment, and outcomes were compared. Twenty-six (41.9%) patients developed AOSD at age ≥ 65 years. The elderly onset group had a lower frequency of sore throat (53.8% vs. 86.1%), higher frequency of pleuritis (46.2% vs. 16.7%), and higher complication rates of disseminated intravascular coagulation (30.8% vs. 8.3%) and macrophage activation syndrome (19.2% vs. 2.8%) than the younger onset group. Cytomegalovirus infections were frequent in elderly onset patients (38.5% vs. 13.9%) but decreased with early glucocorticoid dose reduction and increased immunosuppressant and tocilizumab use. Elderly AOSD is not uncommon; these patients have different characteristics than younger-onset patients. Devising a way to control disease activity quickly while managing infections may be an important goal in elderly AOSD.