Autoinflammatory disease Flashcards
Daily fever, usually in the evening. Fever resolves, then returns the next day.
Adult Onset Still Disease
Salmon color rash during fever, resolves when fever goes away.
Skin manifestation of Adult Onset Still Disease
Sore throat only during febrile period
Unique symptom of AOSD Adult Onset Still Disease
What is a lab value that is specifically associated with Adult Onset Still Disease?
Serum ferritin can be markedly elevated.
Normal ferritin level 40-200
How would you treat mild Adult Onset Still Disease?
Colchicine or NSAIDs. More persistent disease can be treated with inhibition of interleukin-1 therapy: anakinra, canakinumab, or rilonacept.
Serious complication of Adult Onset Still Disease
Macrophage Activation Syndrome
(hemophagocytic lymphohistiocytosis) [HLH}
How does Macrophage Activation Syndrome present?
Bone marrow failure (low red blood cells, white blood cells, and platelets. Persistent fever.
Bone marrow biopsy reveals macrophages consuming hematologic cells in what syndrome?
Macrophage Activation Syndrome in Adult Onset Still Disease.
Inflammatory labs in Macrophage Activation Syndrome.
High CRP but low ESR (fibrinogen is low)
Describe five things about Adult Onset Still Disease.
quotidian fever >39°C, arthralgias, typical rash, and a neutrophilic leukocytosis lasting greater than 2 weeks
When withdrawal from prednisone in acute Adult Onset Still Disease, what can result?
Macrophage Activation Syndrome
Criteria for MAS?
persistent fever, neurologic symptoms, hepatomegaly, cytopenias (anemia, low WBC count, low platelets), elevated serum transaminases, low fibrinogen, and extremely high ferritin.
