Natural history of mevalonate kinase deficiency: a literature review

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 investigators^a

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 %

Abbreviations: MKD mevalonate kinase deficiency, NSAIDs nonsteroidal anti-inflammatory drugs
^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

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