Preprint Case Report Version 1 Preserved in Portico This version is not peer-reviewed

Unveiling the Mystery of Adult-Onset Still's Disease: a Compelling Case Report

Daniele Sola†
* ORCID logo ,
Carlo Smirne
ORCID logo ,
Francesco Bruggi
,
Chiara Bottino Sbaratta
,
Aubin Cardin Tamen Njata
,
Guido Valente
,
Maria Cristina Pavanelli
,
Rosetta Vitetta
,
Mattia Bellan
ORCID logo ,
Lorenzo De Paoli
,
Mario Pirisi
Version 1 : Received: 2 January 2024 / Approved: 3 January 2024 / Online: 3 January 2024 (09:44:12 CET)

A peer-reviewed article of this Preprint also exists.

Sola, D.; Smirne, C.; Bruggi, F.; Sbaratta, C.B.; Njata, A.C.T.; Valente, G.; Pavanelli, M.C.; Vitetta, R.; Bellan, M.; De Paoli, L.; Pirisi, M. Unveiling the Mystery of Adult-Onset Still’s Disease: A Compelling Case Report. Life 2024, 14, 195. Sola, D.; Smirne, C.; Bruggi, F.; Sbaratta, C.B.; Njata, A.C.T.; Valente, G.; Pavanelli, M.C.; Vitetta, R.; Bellan, M.; De Paoli, L.; Pirisi, M. Unveiling the Mystery of Adult-Onset Still’s Disease: A Compelling Case Report. Life 2024, 14, 195.

Abstract

Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder. Diagnosis can take a long time, especially in the presence of confounding factors, and it is, at least in part, of exclusion. AOSD is generally mild, however a small percentage of patients can develop a fearsome as well potentially fatal complication, the macrophage activation syndrome (MAS), which is also referred to as hemophagocytic lymphohistocytosis (HLH). This condition is correlated with a cytokine storm production and monocyte/macrophage overactivation, and typically occurs with rash, pyrexia, pancytopenia, hepatosplenomegaly and systemic involvement. Exitus occurs in approximately 10% of cases. The Histiocyte Society, which is a nonprofit organization committed to improving the lives of patients with histiocytic disorders, currently suggests etoposide in combination with dexamethasone for the treatment of HLH/MAS, although a multidisciplinary collaboration using the resources and expertise of several specialists (e.g. rheumatologist, infectiologist, critical care medicine specialist) is always recommended. Hereby we propose the detailed description of the clinical case of a previously healthy young woman in which MAS was the dramatic onset manifestation of AOSD and whose diagnosis posed a real clinical challenge.

Keywords

adult-onset Still's disease; macrophage activation syndrome; hemophagocytic lymphohistiocytosis; autoimmunity; autoinflammatory diseases; immune system; inflammation; steroids; fever of unknown origin; monocytes/macrophages

Subject

Medicine and Pharmacology, Internal Medicine

Copyright: This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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