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

Expert Rev Clin Immunol. 2015 Jun;11(6):749-61. doi: 10.1586/1744666X.2015.1037287. Epub 2015 May 6.

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.

Keywords: AOSD; CD163; adult-onset Still’s disease; ferritin; interleukin-18; procalcitonin; s100A12; s100A8/9.

Publication types

  • Review

MeSH terms

  • Animals
  • Antigens, CD / metabolism
  • Antigens, Differentiation, Myelomonocytic / metabolism
  • Arthritis / complications
  • Arthritis / diagnosis*
  • Biomarkers / metabolism
  • Calcitonin / metabolism
  • Calcitonin Gene-Related Peptide
  • Diagnosis, Differential
  • Exanthema / complications
  • Exanthema / diagnosis*
  • Ferritins / metabolism
  • Fever / complications
  • Fever / diagnosis*
  • Genetic Predisposition to Disease
  • Humans
  • Infections / complications
  • Infections / diagnosis*
  • Interleukin-18 / metabolism
  • Protein Precursors / metabolism
  • Receptors, Cell Surface / metabolism
  • S100 Proteins / metabolism
  • Still's Disease, Adult-Onset / complications
  • Still's Disease, Adult-Onset / diagnosis*

Substances

  • Antigens, CD
  • Antigens, Differentiation, Myelomonocytic
  • Biomarkers
  • CALCA protein, human
  • CD163 antigen
  • Interleukin-18
  • Protein Precursors
  • Receptors, Cell Surface
  • S100 Proteins
  • Calcitonin
  • Ferritins
  • Calcitonin Gene-Related Peptide