Video

What are the typical clinical manifestations observed in patients with Still’s disease?

Assoc. Prof. Bas Vastert and Prof. Eugen Feist

PP-26831

Transcript

Bas Vastert

[00:08] So, in a typical patient, we of course observed the triad of spiking fever, clear arthritis with the rash and severe systemic inflammation. But in that as much as around 25 to 30 % of the patients, arthritis really develops really later in the disease and sometimes never. And those patients particularly pose difficulty for clinicians to diagnose. I think in the new EULAR/PreS recommendations, we really learned from the adult perspective that arthritis is not a prerequisite anymore. So even in these patients, we now can diagnose Still’s disease. And I think in particular also for these patients, so without arthritis at first sight, the use of biomarkers and of a clear inflammatory systemic response is really, really useful.

[01:10] Maybe you can tell something about these figures in the adult spectrum Eugen.

Eugen Feist

[01:16] Yeah, it is very similar. But in contrast to the paediatric definition of the disease, in adults, arthritis was never a prerequisite (De Matteis, 2024), so we were fine with the so-named Yamaguchi criteria if they were fulfilled. Usually, the diagnosis was also fulfilled after exclusion of course of other differential diagnosis which are of importance like infections or neoplasias. So, this has been done of course in the clinic but after fulfilment of the Yamaguchi criteria where arthritis is not necessary to have the diagnosis was made in adults (Kumar, 2012).

[02:00] And you mentioned already biomarkers. And indeed, there is one very easy biomarker to capture in addition to c-reactive protein and this biomarker is ferritin. And then ferritin is I think available everywhere and in typical patients with Still’s disease, at least this marker is usually sky high. So, it goes also with disease activity and helps us to make the diagnosis also early and to differentiate it from other causes of inflammatory diseases (Mehta, 2012).

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