各位委員您好，
以下Statements為經過第二次GPP共識會議後討論修訂的版本，協請委員們於7月14日(週五)以前完成。
5.1A-1. Systemic acitretin or and etretinate are have been shown to be effective in controlling flares in the acute phase or and in preventing flares as maintenance therapy in adult patients with GPP (Level of evidence: Low);. Evidence for combination use of these retinoids with biologics has been reported but evidence is insufficient scarce to make support any direct specific recommendation (Level of evidence: Low). Retinoids should be considered as a first-line treatment option although they should not be used in pregnant women
(Recommendation: Strong)
5.1A-2. Systemic acitretin or and etretinate are have been shown to be effective in controlling flares in the acute phase or and in precenting flares as maintenance therapy in infant/juvenile patients with GPP (Level of evidence: Low). Evidence for combination use of these retinoids with biologics therapies has been reported but evidence is insufficient scarce to make support any direct specific recommendation (Level of evidence: Low). Retinoids should be considered as a first-line treatment option.
5.1B-1. Cyclosporine has been shown to can be effective in controlling flares in the acute phase or and in preventing flares as maintenance therapy in adult patients with GPP including those with GPP of pregnancy (Level of evidence: Low). It may be considered as a treatment option for those who have an inadequate response or intolerance to, or are contraindicated for retinoids.
5.1B-2. Cyclosporine has been shown to can be effective in controlling flares in the acute phase or and in preventing flares as maintenance therapy in infant/juvenile patients with GPP (Level of evidence: Low). It may be considered as a treatment option for those who have an inadequate response or intolerance to, or are contraindicated for retinoids.
5.1C-1. Methotrexate has been shown to can be effective in controlling flares in the acute phase or and in preventing flares as maintenance therapy in adult patients with GPP including those who had inadequate response to retinoids or cyclosporine (Level of evidence: Low).
(Recommendation: Weak)
5.1C-2. Limited and low-quality data suggest that methotrexate may be a treatment option for infant/juvenile patients but the evidence does not support a firm specific recommendation (Level of evidence: Low).
5.1D. Limited and low-quality data suggest that oral steroids may be useful in controlling acute skin and systemic symptoms but are associated with substantial adverse events; the benefit-risk does not currently support the routine or long-term use of steroids in GPP (Level of evidence: Low).
5.1E. Use of dapsone in GPP has been mentioned in review articles but the lack of recent data are lacking and no and their scarcity make it difficult to make any direct specific recommendation can be made (Level of evidence: Low).
5.1G-2. Use of colchicine in GPP has been mentioned in a few reports studies but data are limited and of low quality; no their scarcity make it difficult to make any direct specific recommendation can be made (Level of evidence: Low).
5.2A. Limited data exist on the use of apremilast in GPP has been mentioned in few studies but their scarcity make it and no difficult to make any direct specific recommendation can be made (Level of evidence: Low).
6A. Topical steroid has been used as a part of the treatment regimen in controlling flares but evidence is insufficient scarce for to make any direct specific recommendation to be made (Level of evidence: Low).
6B. Topical active vitamin D3 has been used as a part of the treatment regimen for treating GPP but evidence is very scarce for to make any direct specific recommendation to be made (Level of evidence: Low). Topical vitamin D3 should be used cautiously on wounds or skin with extensive pustules (including lakes of pus); patients should be monitored for hypercalcemia and dose adjustment should be considered to manage the associated adverse events.
6C. Topical tacrolimus has been used as a part of the treatment regimen for treating GPP but there is insufficient evidence is very scarce to make any direct recommendation supporting its use (Level of evidence: Low).
7A. Although oral PUVA therapy has been used for the treatment of GPP in controlling flares as maintenance therapy in adult patients with GPP, there is insufficient but evidence is scarce to support make any direct specific recommendation (Level of evidence: Low). GPP flare-up after receiving PUVA treatment has also been reported (Level of evidence: Low). Oral PUVA therapy should not be used in pregnant women and infant/juvenile patients with GPP.
7B. Narrowband UV-B therapy has been used for the treatment of GPP in controlling flares as maintenance therapy in adults and infant/juvenile patients with GPP but there is insufficient evidence is scarce for any to make any direct specific recommendation to be made (Level of evidence: Low).
8A-1. TNF-α inhibitors have been shown to can be effective in controlling flares in the acute phase or and in preventing flares as maintenance therapy in adult patients with GPP including those with GPP of pregnancy; combination use of TNF-α inhibitors with methotrexate or retinoids has shown effectiveness in resolving pustules and preventing subsequent flares (Level of evidence: Moderate).
8A-2. TNF-α inhibitors have been shown to can be effective in controlling flares in the acute phase or and in preventing flares as maintenance therapy in infant/juvenile patients with GPP (Level of evidence: Low).
8B-1. Brodalumab (Level of evidence: Moderate), ixekizumab (Level of evidence: Low) and secukinumab (Level of evidence: Low) are effective in controlling flares in the acute phase and in preventing flares as maintenance therapy in adult patients with GPP, and may be considered as a treatment option for GPP.
(Recommendation: Strong for brodalumab; Weak for secukinumab and ixekizumab)
*Do you think spesolimab “should” be considered (8E-1-1) or “can” be considered (8E-1-2) as a first-line treatment option? Please vote for each statement and show the level of your acceptance. Thank you.
8E-1-1 Spesolimab is effective in controlling flares in the acute phase in adult patients with GPP (Level of evidence: High) and should be considered as a first-line treatment option.
8E-1-2 Spesolimab is effective in controlling flares in the acute phase in adult patients with GPP (Level of evidence: High) and can be considered as a first-line treatment option.