The anti–interleukin-1 antibody canakinumab was effective at controlling and preventing recurrence of flares in autoimmune inflammatory diseases: familial Mediterranean fever, mevalonate kinase ...deficiency, and the TNF receptor–associated periodic syndrome.
Adenosine deaminase 2 (ADA2) is a protein with at least two functions. It is a growth factor affecting leukocytes and endothelial cells and an enzyme that influences purine metabolism. This study ...shows that mutant ADA2 causes polyarteritis nodosa.
Polyarteritis nodosa, first described in 1866,
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is a systemic necrotizing vasculitis that affects medium and small muscular arteries.
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,
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The ensuing tissue ischemia can affect any organ, including the skin, musculoskeletal system, kidneys, gastrointestinal tract, and the cardiovascular and nervous systems. Polyarteritis nodosa is usually diagnosed in middle age or later but can appear in childhood.
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,
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,
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The diagnosis remains challenging despite classification criteria for adults
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and children,
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because polyarteritis nodosa frequently presents with nonspecific constitutional symptoms, and organ involvement and disease severity are highly varied. Polyarteritis nodosa is most often primary, but in adults it may be associated . . .
In two placebo-controlled trials, canakinumab, an anti-interleukin-1β monoclonal antibody, achieved a response, prevented flares, and allowed glucocorticoid tapering in patients with systemic ...juvenile idiopathic arthritis. Infection was more common with canakinumab than with placebo.
Systemic juvenile idiopathic arthritis (JIA), the most severe JIA subtype, is characterized by chronic arthritis; intermittently high, spiking temperatures; maculopapular rash; hepatosplenomegaly; lymphadenopathy; serositis; and a marked increase in the level of acute-phase reactants.
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–
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Complications of systemic JIA include growth impairment, osteoporosis, and the potentially lethal macrophage activation syndrome.
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–
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Until recently, systemic JIA was considered a therapeutic orphan, since the principal effective treatment was glucocorticoids, with their known toxicity and long-term growth and bone sequelae.
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Other therapeutic options include nonsteroidal antiinflammatory drugs (NSAIDs), methotrexate, and biologic agents. Both interleukin-6
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–
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and, more recently, interleukin-1
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–
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have been found . . .
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