Adult-onset Still disease: a rare disorder with a potentially fatal outcome

Abstract

Purpose The aim of this study was to assess the clinical and laboratory features of a cohort of Italian patients with adult-onset Still disease (AOSD) with particular attention on possible life-threatening complications. Methods The clinical charts of 41 consecutive Italian patients with AOSD referred to our rheumatological department over the last 10 years were retrospectively examined. Data regarding clinical manifestations, laboratory features and complications were collected and compared with those reported in literature. Results The most frequent manifestations were: fever (90.2%), arthralgias (80.4%), skin rash (75.6%), sore throat (53.6%), arthritis (51.2%), lymphadenopathy (48.7%), hepatosplenomegaly (41.4%), myalgia (21.9%), fatigue (12%), diarrhoea and vomiting (9.7%), pleural effusion (9.7%), pericardial effusion (4.8%) and abdominal pain (2.4%). In two patients whose cases are described in detail; the course of the disease was complicated by disseminated intravascular coagulopathy, in one patient with a fatal outcome. ESR, CRP and leucocyte count mean values were 69.41 mm/h, 69.05 mg/l and 18,798.5 cell/mm 3 (neutrophils 84.64%), respectively. Serum ferritin levels were increased in 48.7% of patients while transaminases were elevated in 42.6% of patients (71% considering only patients in an active phase of disease). Conclusion The results of this study are in line with those reported for other cohorts of patients. Even if the prognosis of AOSD is considered favourable, the present study indicates that the disease is a troubling condition needing prompt intervention. Occasionally, AOSD may rapidly worsen with life-threatening events.

IL-18 Serum Level in Adult Onset Still’s Disease: A Marker of Disease Activity

“…In this study a significant correlation between IL-18 and some laboratory markers of disease activity (ferritin and CRP serum levels) was found. Indeed, it is well known that serum ferritin levels are typically increased during disease flares and this finding is useful for diagnosis and monitoring of disease [ 1 ]. Kawashima et al in 2001 determined IL-18 serum levels in 16 patients with AOSD, 34 with RA, 33 with SLE, 19 with systemic sclerosis (SSc), 21 with polymyositis/dermatomyositis, 28 with SS and in 53 healthy controls demonstrating, as we did in the present report, that IL-18 serum concentrations are higher in AOSD than in all the other conditions [ 19 ].…”

“…Adult Onset Still's Disease (AOSD) is a rare systemic inflammatory disease of unknown etiology.It is characterized by a typical clinical triad: high daily spiking fever, evanescent rash and arthritis or arthralgias, not necessarily present at the same time. A wide spectrum of other symptoms may occur: sore throat, lymphoadenopathy, hepatosplenomegaly, serositis, and myalgias; also pulmonary, cardiovascular, and kidney manifestations may be present occasionally representing severe life-threatening complications [ 1 ]. AOSD has been described for the first time by Bywaters in 1971 [ 2 ] who included in this entity all the patients that did not meet criteria of Rheumatoid Arthritis (RA) but showing the typical manifestations of the systemic form of juvenile rheumatoid arthritis.…”

Presentation and diagnosis of adult-onset Still’s disease: the implications of current and emerging markers in overcoming the diagnostic challenge

“…For those patients who present with arthritis as the predominant manifestation, the term 'chronic articular pattern' has been coined. As proven by epidemiological studies, every kind of joint is likely to be involved; nonetheless, a higher prevalence can be observed in knees, wrists, ankles and hands [10,24].…”

Anakinra Drug Retention Rate and Predictive Factors of Long-Term Response in Systemic Juvenile Idiopathic Arthritis and Adult Onset Still Disease

“…Systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still disease (AOSD) represent two multifactorial nonhereditary autoinflammatory disorders related to pediatric and adult patients, respectively, characterized by a defective control of innate immunity, as depicted for hereditary autoinflammatory disorders, and by daily high spiking fevers along with systemic features including serositis, evanescent rash, generalized lymphoadenopathy, and arthritis (Caso et al, 2014; Cimaz, 2016; Rigante, 2017; Giacomelli et al, 2018; Lyseng-Williamson, 2018). These two conditions are accompanied by a relevant risk of mortality (Priori et al, 2010; Kimura et al, 2013; Minoia et al, 2014; Kumar, 2016; Giacomelli et al, 2018), making a timely diagnosis as well as a prompt treatment mandatory. Treatment of both sJIA and AOSD has traditionally relied on nonsteroidal anti-inflammatory drugs, corticosteroids (CS), and conventional disease-modifying antirheumatic drugs (cDMARDs), with methotrexate being the most frequently used (Gerfaud-Valentin et al, 2014; Kumar, 2016).…”