Adult-Onset Still's Disease-A Complex Disease, a Challenging Treatment.
Int J Mol Sci
; 23(21)2022 Oct 24.
Article
in English
| MEDLINE | ID: covidwho-2118692
ABSTRACT
Adult-onset Still's disease (AOSD) is a systemic inflammatory disorder with an unknown cause characterized by high-spiking fever, lymphadenopathy, hepatosplenomegaly, hyperferritinemia, and leukocytosis. The clinical course can be divided into three significant patterns, each with a different prognosis Self-limited or monophasic, intermittent or polycyclic systemic, and chronic articular. Two criteria sets have been validated. The Yamaguchi criteria are the most generally used, although the Fautrel criteria offer the benefit of adding ferritin and glycosylated ferritin values. AOSD's pathogenesis is not yet completely understood. Chemokines and pro-inflammatory cytokines, including interferon (IFN)-γ, tumor necrosis factor α (TNFα), interleukin (IL)-1, IL-6, IL-8, and IL-18, play a crucial role in the progression of illness, resulting in the development of innovative targeted therapeutics. There are no treatment guidelines for AOSD due to its rarity, absence of controlled research, and lack of a standard definition for remission and therapy objectives. Non-steroidal anti-inflammatory drugs (NSAIDs), corticosteroids (CS), and conventional synthetic disease-modifying antirheumatic drugs (csDMARDs) are used in AOSD treatment. Biological therapy, including IL-1, IL-6, IL-18, and IL-17 inhibitors, as well as TNFα or Janus-kinases (JAKs) inhibitors, is administered to patients who do not react to CS and csDMARDs or achieve an inadequate response.
Keywords
Full text:
Available
Collection:
International databases
Database:
MEDLINE
Main subject:
Still's Disease, Adult-Onset
/
Antirheumatic Agents
Type of study:
Diagnostic study
/
Experimental Studies
/
Prognostic study
Limits:
Adult
/
Humans
Language:
English
Year:
2022
Document Type:
Article
Affiliation country:
Ijms232112810
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