Table 3.

Variables falling in the third quartile, considering the total score obtained for mevalonate kinase deficiency (MKD).

RankVariableScoreFrequency of CitationMedium Score
1Positive genetic test for MVK gene637738.7
2Increased urinary mevalonic acid during episodes352546.5
3Duration of attacks 3–7 days328496.7
5Disease onset < 1 yr§317398.1
6Increase of acute-phase reactants and serum amyloid A during fever episodes276485.8
7Increased IgD levels252455.6
8Presence of triggering factors (immunization, infection, minor trauma, surgery)218356.2
9Abdominal pain184454.1
10Lymphadenopathy (often painful)§150314.8
11Early disease onset§139255.6
12Cervical lymphadenopathy§137314.4
13Gastrointestinal manifestation§113215.4
15Maculopapular rash85194.5
17Mevalonate kinase activity80126.7
19Disease onset < 2 yrs§73126.1
20Irregular periodicity73193.8
21Self-limiting episodes72154.8
  • § Variable already present in the preliminary Eurofever criteria.