Catastrophic adult-onset Still’s disease as a distinct life-threatening clinical subset: case–control study with dimension reduction analysis

Anaïs Wahbi (1) , Benoît Tessoulin (1) , Cédric Bretonnière (1) , Julien Boileau (2) , Dorothée Carpentier (3) , Olivier Decaux (4) , Laurence Fardet (5) , Guillaume Geri (6) , Pascal Godmer (7) , Cécile Goujard (8) , Hervé Maisonneuve (9) , Arnaud Mari (10) , Jacques Pouchot (11) , Jean-Marc Ziza (12) , Sophie Georgin-Lavialle (13) , Mohamed Hamidou (1) , Antoine Néel (1)

1 CHU Nantes - Centre Hospitalier Universitaire de Nantes = Nantes University Hospital
2 CH Morlaix
3 CHU Rouen
4 Centre Hospitalier Universitaire de Rennes [CHU Rennes] = Rennes University Hospital [Ponchaillou]
5 Hôpital Henri Mondor
6 Hôpital Ambroise Paré [AP-HP]
7 UBS Vannes - Université de Bretagne Sud - Vannes
8 Hôpital Bicêtre [AP-HP, Le Kremlin-Bicêtre]
9 CHD Vendée - Centre Hospitalier Départemental - Hôpital de La Roche-sur-Yon
10 CHU de Saint-Brieuc
11 HEGP - Hôpital Européen Georges Pompidou [APHP]
12 Groupe Hospitalier Diaconesses Croix Saint-Simon
13 CHU Tenon [AP-HP]

Objectives: Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder. Diagnosing AOSD can be challenging, as disease presentation and clinical course are highly heterogeneous. For unclear reasons, a few patients develop life-threatening complications. Our objective was to determine whether these cases resulted from therapeutic delay or could represent a peculiar AOSD subset. Methods: We conducted a multicentre retrospective study of 20 AOSD patients with organ failure requiring intensive care unit admission and 41 control AOSD patients without organ failure. Clinico-biological data at hospital admission were explored using supervised analyses and unsupervised dimension reduction analysis (factor analysis of mixed data, FAMD). Results: Disease duration before admission was shorter in patients with life-threatening AOSD (median, 10 vs 20 days, p = 0.007). Disease duration before AOSD therapy initiation also tended to be shorter (median, 24 vs 32 days, p = 0.068). Despite this shorter disease duration, FAMD, hierarchical clustering and univariate analyses showed that these patients exhibited distinctive characteristics at first presentation, including younger age; higher frequency of splenomegaly, liver, cardiac and/or lung involvement; less frequent arthralgia; and higher ferritin level. In multivariate analysis, 3 parameters predicted life-threatening complications: lack of arthralgia, younger age and shorter time between fever onset and hospitalisation. Conclusion: This study suggests that life-threatening complications of AOSD occur very early, in a peculiar subset, which we propose to name catastrophic adult-onset Still's disease (CAOSD). Its exact burden may be underestimated and remains to be clarified through large multicentre cohorts. Further studies are needed to identify red flags and define the optimal therapeutic strategy.

Mots clés: Adult-onset Still's disease; Reactive haemophagocytic syndrome; Shock; Acute respiratory failure; Differential diagnosis; Intensive care unit; Case-control study; Clusters; Cytokine storm

Domaines: Pneumologie et système respiratoire

Anaïs Wahbi, Benoît Tessoulin, Cédric Bretonnière, Julien Boileau, Dorothée Carpentier, et al.. Catastrophic adult-onset Still’s disease as a distinct life-threatening clinical subset: case–control study with dimension reduction analysis. Arthritis Research & Therapy, 2021, 23 (1), ⟨10.1186/s13075-021-02631-7⟩. ⟨hal-03405903⟩