Table 3.

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

Rank	Variable	Score	Frequency of Citation	Medium Score
1	Positive genetic test for MVK gene	637	73	8.7
2	Increased urinary mevalonic acid during episodes	352	54	6.5
3	Duration of attacks 3–7 days	328	49	6.7
4	Fever^§	327	42	7.8
5	Disease onset < 1 yr^§	317	39	8.1
6	Increase of acute-phase reactants and serum amyloid A during fever episodes	276	48	5.8
7	Increased IgD levels	252	45	5.6
8	Presence of triggering factors (immunization, infection, minor trauma, surgery)	218	35	6.2
9	Abdominal pain	184	45	4.1
10	Lymphadenopathy (often painful)^§	150	31	4.8
11	Early disease onset^§	139	25	5.6
12	Cervical lymphadenopathy^§	137	31	4.4
13	Gastrointestinal manifestation^§	113	21	5.4
14	Diarrhea^§	106	26	4.1
15	Maculopapular rash	85	19	4.5
16	Rash	83	21	4.0
17	Mevalonate kinase activity	80	12	6.7
18	Aphthosis^§	80	21	3.8
19	Disease onset < 2 yrs^§	73	12	6.1
20	Irregular periodicity	73	19	3.8
21	Self-limiting episodes	72	15	4.8