The Spectrum of Still’s Disease: A Comparative Analysis of Phenotypic Forms in a Cohort of 238 Patients

Abstract

Still’s disease (SD) is a heterogeneous autoinflammatory disorder for which several phenotypes have been described. We conducted a retrospective study to re-evaluate the dichotomous view of the disease, to compare the juvenile and adult forms, and to look for prognostic factors. We collected data from ten French centers, seeking patients with a diagnosis of adult-onset SD (AOSD) or systemic juvenile idiopathic arthritis (sJIA). We identified 238 patients, 152 (64%) of whom had AOSD while 86 (36%) had sJIA. The median age at SD onset was 26.6 years. In patients with identifiable patterns, the course of SD was systemic in 159 patients (74%), chronic in 55 (26%). Sore throat and myalgia were more frequent in patients with AOSD. Abnormal liver tests, serum ferritin and C-reactive protein levels were higher in AOSD group. Fever and skin rash were predictive of complete remission or recovery and high lactate dehydrogenase level was a poor prognosis factor. Symptoms such as splenomegaly, skin rash, high polymorphonuclear neutrophils count and macrophage activation syndrome were predictive of a systemic phenotype. Overall, there were no major differences between sJIA and AOSD. Our results are consistent with the “biphasic” model of an autoinflammatory disease that can progress to chronic arthritis if not treated early.

Still’s disease continuum from childhood to elderly: data from the international AIDA Network Still’s disease registry

“… 26 Actually, our clinical findings are similar to that reported by Neau et al , who identified a similar clinical presentation between paediatric and adult groups, except for sore throat and myalgia, which were more frequent in adults in their study. 27 Neau et al 27 identified a statistically significant difference between paediatric and adult-onset groups in the CRP and serum ferritin levels. In this regard, ferritin levels have been distinguished between adult and elderly cases in our non-paediatric patients, probably affecting the difference between paediatric and adult groups.…”

Childhood rheumatic diseases: bites not only the joint, but also the heart

“…Among the JIA subtypes, systemic onset JIA (sJIA) is more likely to have pericardial effusion [ 105 ]. Recent data suggests pericarditis is present in 7.2% to 28% children with sJIA [ 106 , 107 ] and cardiac tamponade in 2.5% [ 108 ]. There is no difference in cardiac involvement among different subgroups of sJIA, with pericarditis seen in 19% of systemic subtype of sJIA and 15.4% in chronic course of sJIA [ 108 ].…”

“…Symptomatic myocarditis is seen in 3.5% of children with JIA [ 104 ]. More recent series have shown myocarditis to be complicate sJIA in 4.2% [ 108 ]. Echocardiographic evidence of subclinical myocarditis is seen in 10% in children with sJIA [ 104 ].…”

“…Coronary artery abnormalities on echocardiography have been described in four children with sJIA who were initially treated as KD but eventually went on to develop arthritis and resolution of coronary echogenicity and dilatation [ 113 ]. Pulmonary artery hypertension is also rarely present and has been described in 0.8% [ 108 ]. Arrhythmias have also rarely been reported in sJIA MAS [ 112 ].…”