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Biomarkers for adult-onset still’s disease: up-to-date

When a patient with suspected adult-onset Still’s disease (AOSD) presents with decreased blood pressure, bacterial infection and malignancies should first be excluded based on laboratory results, blood and urine cultures, and imaging tests. Then, the patient may be diagnosed with AOSD with systemic inflammatory response syndrome (SIRS). The diagnosis of AOSD can be confirmed based on both the Yamaguchi criteria and the criteria for SIRS, with physical examination and laboratory results revealing inflammatory markers and ferritin, but no specific biomarkers for AOSD [1,2]. For the diagnosis and treatment of patients with suspected AOSD, a variety of new biomarkers have been identified for evaluating both the initial diagnosis and disease activity of AOSD. However, it remains uncertain whether these new biomarkers, including S100 protein, interleukin-6 (IL-6), and IL-18 are actually being used. AOSD is a rare systemic inflammatory disease characterised by a high spiking fever, arthralgia or arthritis, transient maculopapular skin rash, lymphadenopathy, and hepatosplenomegaly brought about by the accompanying cytokine storm. Clinical manifestations of AOSD vary widely, ranging from mild constitutional symptoms such as malaise and fatigue to life-threatening complications including SIRS, fulminant hepatitis, and macrophage activation syndrome (MAS). Laboratory abnormalities include leucocytosis with neutrophilia, thrombocytosis, elevated acute phase reactants, hyperferritinaemia, and elevated liver enzymes. This combination of heterogeneous symptoms, complex laboratory results, and variable disease courses makes the diagnosis of AOSD and prognosis of disease outcomes extremely difficult. Furthermore, AOSD is difficult to diagnose due to a wide range of diseases with similar phenotypes, such as lymphoproliferative diseases and bacterial or viral infections. Although early diagnosis leads to better results, diagnosis is often delayed due to persistent spiking fever, with most patients only diagnosed after the appearance of secondary symptoms, as well as exclusion of more common conditions, such as infections or other malignant diseases. Despite these difficulties, classification criteria for diagnosis of AOSD have been established, with the Yamaguchi criteria offering the highest sensitivity and accuracy for AOSD diagnosis [1]. However, these criteria are more useful as exclusion criteria, and do not provide insight into either disease progression or prognosis. Furthermore, there are no good biomarkers that can effectively distinguish AOSD from other infectious or inflammatory diseases. For example, higher levels of ferritin are observed in patients with AOSD, but the specificity of ferritin measurements for diagnostic purposes is relatively low [3]. Disease activity in AOSD is also difficult to evaluate, ranging from mild to serious manifestations. Therefore, accurate evaluation of disease activity is important for therapeutic decision-making. Certain acute phase reactants, including C-reactive protein (CRP) and ferritin, are used in laboratory monitoring of disease activity in AOSD, but these markers are nonspecific and are not well correlated with disease activity. Disease activity can be assessed using the Pouchot score, including elevated liver enzymes, pericarditis, myalgia, neutrophilia, and lymphadenopathy [4]. A recent study showed that a cut-off at 7.0 of the score had a strong prognostic impact in identifying patients at risk of AOSD-related death [5]. However, this score is rarely used due to the difficultly in checking for pericarditis and hepatomegaly as part of routine clinical follow-up. The recently introduced Auto-Inflammatory Disease Activity Index (AIDAI) has been validated for the evaluation of disease activity in four major hereditary recurrent fever syndromes [6]. This index is promising, but has not yet been evaluated for AOSD. Further study of the potential utility of the AIDAI with respect to AOSD is needed, as is a biomarker facilitating diagnosis, treatment decisions, and prediction of life-threatening complications of AOSD.