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

Abstract

Adult-onset Still's disease (AOSD) is a known cause of fever of unknown origin. It is characterized by a triad of symptoms: spiking fever (>39°C), salmon-colored rash and arthritis/arthralgia. On a predisposing genetic background, several conditions may act as trigger for disease and among these, infectious agents are the most important. Nowadays, a dichotomous view of AOSD has been introduced which distinguishes this entity in two subsets according to the clinical features and laboratory aspects: systemic or articular. As AOSD is a diagnosis of exclusion, specific biomarkers able to facilitate differential diagnosis are needed. A number of possible biomarkers have been proposed that will be discussed in detail in this review: ferritin, IL-18, procalcitonin, s100 proteins and sCD163.

Procalcitonin levels in bloodstream infections caused by different sources and species of bacteria

“…PCT levels can be useful not only for distinguishing between GNB and GPB infections, but also between GNB or GPB bacterial species. [4,9,13,16,19,20] 12 0.21 (0.07-2.03) Soft tissue infection [5,10,14,17,19,21] 12 0.82 (0.18-7.13) Pelvic infection [6,11,15,18,20,21] Thus, PCT levels are a marker for sepsis, which can inform decisions on the ideal antimicrobial treatment for patients.…”

“…An episode was defined as the time period associated with one or more positive BCs for the same organism(s) [12,13]. PCT levels may be altered in some non-infectious diseases, such as autoimmune disease [14][15][16][17][18] and malignant tumors [19][20][21]. Therefore, our exclusion criteria were: (1) a medical history of immune system disease (adult-onset Still's disease, rheumatoid arthritis, ankylosing spondylitis, systemic lupus erythematosus, vasculitis, or multiple sclerosis) [14][15][16][17][18]; and (2) history of malignant tumor (thyroid carcinoma or lung cancer) [19][20][21].…”

Response to Interleukin-1 Inhibitors in 140 Italian Patients with Adult-Onset Still’s Disease: A Multicentre Retrospective Observational Study

“…Subsequent phosphorylation of several kinases leads to translocation of NF-κB to the nucleus and final expression of a large portfolio of inflammatory genes (Weber et al, 2010). The IL-1 family comprises 11 members (Dinarello, 2011), and some investigators (Colafrancesco et al, 2012; Priori et al, 2014) have shown that IL-1α/β and IL-18 are crucial in the pathogenesis of AOSD and are valid serological biomarkers of the disease (Colafrancesco et al, 2015). …”

Ritka betegség, atípusos bőrtünetekkel: felnőttkori Still-betegség

“…Ezzel egyidőben a szérum ferritin egy izotípusának a glikozilált ferritinnek (GF) a szintje csökken . Egészséges egyénekben a GF a szérum ferritin 50-80%-t teszi ki, míg a betegség akut szaka alatt 20%-ra csökken, és a remissziók alatt a szérum ferritin szinttel ellentétben nem normalizálódik [10,15,16] . E két érték együttes vizsgálata tovább növeli a diagnosztika szenzitivitását [4,5] .…”

“…A szérum IL-18, prokalcitonin, S-100, szérum CD163 markerek szerepe és a diagnosztikus haszna még további vizsgálatokat igényel [15,16] .…”