Adult Onset Still Disease Flashcards
What is Adult-Onset Still’s Disease (AOSD)?
AOSD is a rare auto-inflammatory disease that affects adults, characterized by fever, joint pain, and systemic symptoms like rash, lymphadenopathy, and organ enlargement (liver and spleen).
key symptoms
Mono/oligoarthritis, fever (lasting at least 2 weeks), evanescent erythematous rash, generalized lymphadenopathy, hepatomegaly/splenomegaly, and serositis.
What is a characteristic feature of the rash seen in AOSD?
The rash is evanescent, meaning it fades quickly, and is typically salmon-colored.
What are the typical fever patterns seen in AOSD?
High spiking fevers, often occurring in late afternoon or early evening.
What joints are commonly affected in AOSD?
AOSD typically involves large joints, with joint pain and swelling.
Besides joint pain and fever, what other systemic symptoms are associated with AOSD?
Generalized lymphadenopathy, hepatomegaly/splenomegaly, and serositis (inflammation of the lining around the lungs or heart).
What is the pediatric counterpart of Adult-Onset Still’s Disease (AOSD)?
Systemic Juvenile Idiopathic Arthritis (sJIA) is the pediatric counterpart of AOSD.
How is Adult-Onset Still’s Disease typically treated?
Treatment involves managing inflammation with NSAIDs, corticosteroids, and disease-modifying agents.
What type of pain is commonly associated with Adult-Onset Still’s Disease (AOSD)?
Muscle pain that comes and goes with fever, and can be severe enough to disrupt daily activities.
What is one of the first symptoms of AOSD related to the throat?
Sore throat is one of the first symptoms, often accompanied by swollen and tender lymph nodes in the neck.
What happens to internal organs like the liver and spleen in AOSD?
The liver and spleen are often enlarged in patients with AOSD.
How does fever present during an active episode of AOSD?
Most people develop a fever of at least 39°C (102.2°F) every day for at least a week, with peaks in the late afternoon or early evening, and possibly two fever spikes daily.
What type of rash is characteristic of AOSD?
A salmon-pink rash that appears with the fever, typically on the upper body, arms, or legs, but is rarely itchy.
What inflammation-related symptoms can occur in the lungs and heart?
Inflammation and sometimes fluid accumulation can occur around the heart and lungs, leading to chest pain and breathing difficulties.
How does AOSD affect the joints?
Patients experience achy and swollen joints, especially in the knees and wrists, which may be stiff, painful, and inflamed. This discomfort usually lasts at least two weeks.
How is AOSD classified?
AOSD is classified as an auto-inflammatory disease caused by innate immunity activation without infection.
What are the major pro-inflammatory cytokines involved in AOSD?
The major pro-inflammatory cytokines are IL-1beta, IL-18, IL-6, and TNF-alpha.
What triggers the inflammation in AOSD?
The trigger is likely a PAMP (Pathogen-Associated Molecular Pattern) or DAMP (Damage-Associated Molecular Pattern), not an infection.
Which gene polymorphisms and HLA haplotypes increase susceptibility to AOSD?
Polymorphisms in the MIF, IL-18, PERF1, and Munc13-4 genes, as well as HLA-Bw35, -DR2, -DR4, and -DRB1 haplotypes, increase susceptibility.
Which immune cells are heavily involved in AOSD, and what is their role?
Neutrophils are heavily involved due to the chemo-attractive effect of IL-1, and macrophages are activated by NK cells, which produce high levels of INF-gamma.
