Makindo Medical Notes

🔥 Adult-Onset Still's Disease (AOSD) is a rare systemic autoinflammatory disorder. It classically presents with high spiking fevers, arthralgia/arthritis, and a distinctive salmon-pink rash. Often mimics viral infection → consider in PUO cases with negative autoantibodies.

ℹ️ About

• Typically affects young adults (16–35 yrs).
• Often begins as pyrexia of unknown origin (PUO).
• Suspected viral trigger in some cases.

👩‍⚕️ Clinical Features

• Daily spiking fevers up to 40°C 🌡️ with sweats & chills.
• Severe sore throat (common early feature).
• Rash: salmon-pink, evanescent, maculopapular (often on trunk & proximal limbs, worse with fever spikes).
• Weight loss, malaise, hepatosplenomegaly, lymphadenopathy.
• Arthralgia → chronic destructive arthritis in late disease.

🔬 Investigations

• RF and ANA negative (helps exclude RA & lupus).
• ↑ ESR, ↑ CRP.
• Leukocytosis (WCC often >40,000) with neutrophilia.
• Thrombocytosis.
• Ferritin markedly raised (>3000 ng/mL is highly suggestive).

⚠️ Complication: Macrophage Activation Syndrome (MAS)

• Life-threatening “cytokine storm” syndrome with pancytopenia, DIC, liver failure.
• Triggered by excessive immune activation.
• Treatment: High-dose corticosteroids ± immunosuppressants (e.g. ciclosporin, biologics).

💊 Management

• 🟢 Mild disease: NSAIDs (e.g. aspirin 1 g TDS, ibuprofen).
• 🟠 Moderate/severe: Corticosteroids (e.g. prednisolone 40–60 mg/day).
• ⚙️ Disease-modifying therapy: Methotrexate for steroid-sparing effect.
• 🧬 Biologics:
  • TNF inhibitors (etanercept, infliximab).
  • IL-1 inhibitors (anakinra, canakinumab).
  • IL-6 inhibitors (tocilizumab).