Schnitzler syndrome refractory to anakinra: successful treatment with canakinumab

Figures & data

Figure 1. Histopathological pattern of leukocytoclasia without vasculitis. (A) Microscopic observation of leukocytoclasia without vasculitis. Evolution of SchS before and after canakinumab treatment. (B) Skin wounds before canakinumab treatment (side view). (C) Skin wounds before canakinumab treatment (dorsal view). (D) Skin recovery after one dose of canakinumab (side view). (E) Skin recovery after one dose of canakinumab (dorsal view).

Table 1. Summary of patient characteristics with diagnosis of Schnitzler syndrome.

	Rowczenio (3)	de Koning (4)	Krause (5)	Rossi-Semerano (6)	Pesek (18)	Thomsen (19)	Vander-schueren (14)
Number of patients	21	8	C: 7. P: 13	7	2	1	1
Age (y)	51.0 (36.8–78.9)	64.5 (51–75)	C: 62 (54–71) P: 64 (28–74)	55.3 (49.9–76.2)	55	51	49
Sex
Male	13	6	11	5	1	0	1
Female	8	2	9	2	1	1	0
Paraprotein
IgG	1	4	C: 1. P: 3	–	–	0	0
IgM	20	4	C: 5. P: 10	–	–	1	1
IgA	0	0	C: 1. P: 0	–	–	0	0
Response to anakinra	1 partial 19 complete 1 died	–	C: 3 P: 5	4 complete 1 inefficacy/loss of efficacy 2 adverse events	–	1 complete	1 partial
Time of anakinra use (y)	4.9 (1.3–9.6)	5.3 (0.3–7.0)	–	–	–	4	3
CRP (mg/L)	6.8 (1.8–25.7)	16.9 (1.6–33.3)	C: 9.3 (1.4–25.4) P: 3.1 (0.7–13.9)	–	–	–	>45
Hb (g/dL)	129.0 (95–154)	–	–	–	–	–	–
SAA (mg/L)	–	973.5 (72–2390)	C: 428 (69–857) P: 160 (2–1,310)	–	–	–	–

Data reported as median (range); data not reported: (–).

C: canakinumab group; CRP: C-reactive protein; Hb: hemoglobin; P: placebo group; SAA: serum amyloid A.

Data availability statement

Data sharing not applicable to this article as no datasets were generated or analyzed during the current study.