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Table 2 Response to treatment in 67 patients with MKD from the Eurofever registry and in combined data of 187 patients from a literature review (19 papers) by the Eurofever registry investigatorsa

From: Natural history of mevalonate kinase deficiency: a literature review

Medication Data source No. of patients Treated patients Complete response Partial response Failure
    No. % No. % No. % No. %
NSAIDs Eurofever 67 39 58 % 5 13 % 25 64 % 9 23 %
Corticosteroids Eurofever 67 33 49 % 8 24 % 22 67 % 3 9 %
  Literature 187 56 30 % 0 0 % 35 63 % 21 38 %
Colchicine Eurofever 67 17 25 % 0 0 % 6 35 % 11 65 %
  Literature 187 60 32 % 1 2 % 11 18 % 48 80 %
Statins Eurofever 67 11 16 % 0 0 % 3 27 % 8 73 %
  Literature 187 31 17 % 0 0 % 10 32 % 21 68 %
Anakinra Eurofever 67 27 40 % 6 22 % 18 67 % 3 11 %
  Literature 187 35 19 % 12 34 % 16 46 % 7 20 %
Canakinumab Eurofever 67 2 3 % 1 50 % 1 50 % 0 0 %
  Literature 187 3 2 % 2 67 % 1 33 % 0 0 %
Rilonacept Eurofever 67 1 1 % 0 0 % 1 100 % 0 0 %
Etanercept Eurofever 67 17 25 % 1 6 % 10 59 % 6 35 %
  Literature 187 27 14 % 6 22 % 9 33 % 12 44 %
Infliximab Eurofever 67 1 1 % 0 0 % 0 0 % 1 100 %
Adalimumab Eurofever 67 2 3 % 0 0 % 1 50 % 1 50 %
  Literature 187 3 2 % 1 33 % 1 33 % 1 33 %
  1. Abbreviations: MKD mevalonate kinase deficiency, NSAIDs nonsteroidal anti-inflammatory drugs
  2. aAdapted from ter Haar et al. [18]. Response to treatment was classified as complete remission, partial remission, failure or worsening. Complete remission was defined as no signs of active disease and the normalization of reported inflammatory markers, allowing for the persistence of sequelae. Some patients received more than one treatment