Spesolimab, an interleukin-36 receptor monoclonal antibody, for the treatment of generalized pustular psoriasis

Figures & data

Figure 1. Typical appearance of generalized pustular psoriasis, with areas of edema, erythema, scaling and multiple pustules coalescing into lakes of pus.

Figure 2. Immuno-pathogenesis of generalized pustular psoriasis. Self-amplification of IL-36 and IL-17C in the absence of functional IL-36Ra leads to very high levels of CXCL8 (IL-8) and other CXCL chemokines that produce massive neutrophil influx. CXCL, C-X-C motif chemokine ligand; GPP, generalized pustular psoriasis; IL, interleukin; R, receptor. (adapted from [1]).

Table 1. Evidence for efficacy of biologic treatments targeting TNF?, IL-17, IL-23 in generalized pustular psoriasis.

	trial design	no. with GPP	dose	efficacy	license in GPP	reference
TNFi
Adalimumab	Multicentre, single arm, open-label	10	40-80 mg/2 weeks	7/10 responded at week 16	Japan	[27]
Certolizumab	Multicentre, open-label, randomized 1:1 to 2 different dose regimens of certolizumab	7	400 mg every 2 weeks to week 16; or 200 mg at weeks 0/2/4 then 400 mg every other week to week 16	7/7 in remission or improved at week 16	Japan	[28]
Etanercept	Retrospective case series	6	50 mg twice weekly	Stable disease in 6 patients at week 24		[29]
Infliximab	Retrospective case series	10	N/A	clinical remission in 8/10	Japan	[30]
IL-17i
brodalumab	Multicentre, single arm, open-label	12	140-210 mg at day 1, 7, 14 and then every 2 weeks	3/12 in remission and 10/12 improved at week 12	Japan, Taiwan, Thailand	[31]
Ixekizumab	Multicentre, single arm, open-label	5	160 mg, then 80 mg every 2 weeks to week 12, then 80 mg every 4 weeks from week 16 to week 52	1/5 resolved and 4/5 improved at week 12	Japan	[32,33]
Secukinumab	Multicentre, single arm, open-label	12	150 mg at weeks 1, 2, 3 and 4, then every 4 weeks	10/12 much improved at week 16	Japan	[34]
IL23i
Guselkumab	Multicentre, single arm, open-label	10	50 mg at weeks 0,4 and 8, then every 8 weeks. Increase to 100 mg every 8 weeks at week 20 if necessary.	At week 16, 7/10 responded (2 very much improved, 2 much improved, 3 minimally improved)	Japan	[35]
Risankizumab	Multicentre, open-label, randomized 1:1 to 2 different dose regimens of risankizumab	8	75 mg at weeks 0, 4 and then every 12 weeks; or 150 mg at weeks 0, 4 and then every 12 weeks	8/8 responded (at least minimally improved) at week 16	Japan	[36]
Ustekinumab	Retrospective case series	4	Various	4/4 responded		[37]

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