Published in:
Open Access
01-11-2013 | Meeting abstract
P02-008 - Dramatic response to canakinumab in MKD
Authors:
B Makay, E Ünsal
Published in:
Pediatric Rheumatology
|
Special Issue 1/2013
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Excerpt
Mevalonate kinase deficiency-associated periodic fever syndrome (MKD) is a systemic autoinflammatory disease caused by mutations in the mevalonate kinase gene (MVK), previously named "hyper-IgD syndrome" due to its characteristic increase in serum IgD level. The patients suffer recurrent fever attacks every 2-8 weeks beginning from infancy, often precipitated by immunizations, infections or emotional stress. Fever lasts 2-7 days and can be accompanied by malaise, headache, diarrhea, abdominal pain, vomiting, skin rashes, arthralgia, arthritis, tender lymphadenopathy and hepatosplenomegaly. …