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Adult-onset Still Syndrome (AOSD) – Symptoms & Treatment
Adult-onset Still syndrome (AOSD) is a rare inflammatory disease in adults characterized by high fever, joint pain, and a salmon-colored skin rash.
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Adult-onset Still syndrome (AOSD) is a rare inflammatory disease in adults characterized by high fever, joint pain, and a salmon-colored skin rash.
Overview
Adult-onset Still syndrome (AOSD) is a rare, systemic inflammatory disease that primarily affects adults. It belongs to the group of autoinflammatory diseases, in which the innate immune system is dysregulated and triggers excessive inflammatory responses without the presence of detectable autoantibodies. AOSD is the adult form of systemic juvenile idiopathic arthritis (formerly known as systemic-onset juvenile rheumatoid arthritis), which was originally described in children.
Causes and Risk Factors
The exact cause of AOSD remains incompletely understood. It is believed that a genetic predisposition combined with environmental factors, particularly infections, may trigger the disease. The following factors are under discussion:
- Infections: Viral or bacterial infections (e.g., Epstein-Barr virus, Parvovirus B19) may act as triggers.
- Genetic factors: Certain HLA antigens (human leukocyte antigens) appear to play a role.
- Cytokine dysregulation: Elevated levels of interleukin-1, interleukin-6, and interleukin-18 are characteristic and promote the inflammatory response.
Symptoms
AOSD typically follows a relapsing course and presents with a characteristic triad of three cardinal symptoms:
- High fever: Daily fever spikes above 39 °C (102.2 °F), often occurring in the late afternoon or evening and resolving back to normal temperature.
- Salmon-colored skin rash: A transient, trunk-predominant rash that typically appears during fever episodes.
- Joint complaints: Arthralgia (joint pain) or arthritis (joint inflammation), frequently affecting the knees, wrists, and shoulders.
Additional possible symptoms include:
- Sore throat and enlarged lymph nodes
- Enlargement of the liver and spleen (hepatosplenomegaly)
- Serositis (inflammation of the pericardium or pleura)
- Muscle pain (myalgia)
Diagnosis
There is no specific laboratory test for AOSD. The diagnosis is established by excluding other conditions and applying clinical classification criteria. The most widely used are the Yamaguchi criteria (1992) and the Fautrel criteria (2002).
Typical Laboratory Findings
- Markedly elevated inflammatory markers: CRP, ESR (erythrocyte sedimentation rate), leukocytes
- Massively elevated ferritin (often above 10,000 μg/L) – considered an important diagnostic clue
- Elevated liver enzymes (transaminases)
- Absence of rheumatoid factor (RF) and antinuclear antibodies (ANA)
Further Diagnostic Workup
- Imaging (X-ray, ultrasound, MRI) to assess joints and internal organs
- Exclusion of infectious diseases, malignancies, and other autoimmune conditions
Treatment
Treatment of AOSD depends on disease severity and therapeutic response. The goal is to control inflammation and prevent organ damage.
Drug Therapy
- Non-steroidal anti-inflammatory drugs (NSAIDs): Used as a first measure in mild cases.
- Corticosteroids: The mainstay of treatment for moderate to severe disease; rapidly reduce inflammation.
- Methotrexate (MTX): Used in chronic cases and to reduce the need for corticosteroids.
- Biologics: In refractory cases, interleukin-1 inhibitors (e.g., anakinra, canakinumab) or interleukin-6 inhibitors (e.g., tocilizumab) are used.
Disease Course
AOSD may follow a monocyclic course (a single episode with full remission), a polycyclic course (recurring flares), or a chronic articular pattern. The course varies considerably between individuals.
Complications
A feared and potentially life-threatening complication is macrophage activation syndrome (MAS), also known as hemophagocytic lymphohistiocytosis. It is characterized by uncontrolled immune system activation and requires immediate intensive care treatment.
References
- Efthimiou P, Georgy S. Pathogenesis and management of adult-onset Still's disease. Seminars in Arthritis and Rheumatism. 2006;36(3):144–152.
- Feist E, Mitrovic S, Fautrel B. Mechanisms, biomarkers and targets for adult-onset Still's disease. Nature Reviews Rheumatology. 2018;14(10):603–618.
- Giacomelli R et al. Adult-onset Still's disease: an overview of its epidemiology, pathogenesis, diagnosis and treatment. Autoimmunity Reviews. 2018;17(6):601–612.
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