Anthem Blue Cross California Spevigo (spesolimab-sbzo) Form

Overview Clinical criteria Overview Coding Document history References This document addresses the use of Spevigo (spesolimab-sbzo). Spevigo is a humanized, selective antibody that blocks the activation of the interleukin-36 receptor, a signaling pathway in the immune system involved in a number of autoimmune diseases. It is approved for the treatment of generalized pustular psoriasis (GPP) flares and is the first FDA-cleared treatment for this disease. Generalized pustular psoriasis (GPP) is a potentially life-threatening neutrophilic skin disease that is clinically distinct from plaque psoriasis. A preceding history of plaque psoriasis may or may not be present in individuals presenting with GPP. It is characterized by the development of widespread eruption of pustules and erythematous plaques which may be accompanied by fever, malaise, and/or extracutaneous manifestations including arthritis. The European Rare and Severe Psoriasis Expert Network (ERASPEN) define consensus diagnosis criteria as the following: Primary, sterile, macroscopically visible pustules on non-acral skin (excluding cases where pustulation is restricted to psoriatic plaques) • With or without systemic inflammation • With or without plaque-type psoriasis • Either relapsing (>1 episode) or persistent (>3 months). Within dermatology, acral skin relates to that of the distal extremities such as ears, fingers, toes, nose, etc. The clinical course of GPP can be relapsing with recurrent flares, or persistent with intermittent flares. There is a lack of high-quality data on efficacy of various treatments for GPP, but may include adjunctive topical therapy, phototherapy, and/or conventional immunosuppressants such as acitretin, cyclosporine or methotrexate. Certain biologics approved for treatment of psoriasis have been used, but data is lacking. Spevigo targets one of the underlying immunologic signaling pathways of the disease by blocking the IL-36 receptor. In a phase 2 trial, individuals randomized to one 900 mg IV infusion of spesolimab (n=35) or placebo (n=18) were treated when presenting with a moderate to severe GPP flare defined as a Generalized Pustular Psoriasis Physician Global Assessment (GPPGA) score of at least 3, GPPGA pustular subscore of at least 2, and 5% of body surface area (BSA) with erythema and the presence of pustules. At the end of week 1, 54% of individuals in the spesolimab group and 6% of those in the placebo group had a GPPGA pustulation subscore of 0 (no visible pustules). Currently, spesolimab is also under investigation for the prevention of GPP flares and for the treatment of other neutrophilic skin disease including palmoplantar pustulosis (PPP) and hidradenitis suppurativa (HS). Generalized Pustular Psoriasis Physician Global Assessment Score 0 (clear) 1 (almost clear) 2 (mild) Erythema Normal or post- inflammatory hyperpigmentation Faint, diffuse pink or slight red Light red 3 (moderate) Bright red 4 (severe) Deep fiery red Pustules Scaling No visible pustules No scaling or crusting Low density occasional small discrete pustules (noncoalescent) Moderate density grouped discrete small pustules (noncoalescent) High density pustules with some coalescence Very high-density pustules with pustular lakes Superficial focal scaling or crusting restricted to periphery of lesions Predominantly fine scaling or crusting Moderate scaling or crusting covering most or all lesions Severe scaling or crusting covering most or all lesions *Composite mean score = (erythema + pustules + scaling)/3; total GPPGA score given is 0 if mean = 0 for all three components, 1 if mean 0 to <1.5, 2 if mean 1.5 to <2.5, 3 if mean 2.5 to <3.5, 4 if mean ≥3.5. 1 Clinical Criteria When a drug is being reviewed for coverage under a member’s medical benefit plan or is otherwise subject to clinical review (including prior authorization), the following criteria will be used to determine whether the drug meets any applicable medical necessity requirements for the intended/prescribed purpose. Spevigo (spesolimab-sbzo) Requests for one initial 900 mg dose [2 vials] of Spevigo (spesolimab-sbzo) at the beginning of each Generalized Pustular Psoriasis (GPP) flare may be approved if the following criteria are met: I. II. AND III. AND IV. Individual is 18 years of age or older; AND Individual has a diagnosis of Generalized Pustular Psoriasis (GPP), as verified by (Bachelez 2021): A. The presence of primary, sterile, macroscopically visible pustules on non-acral skin; AND B. Pustulation that is NOT restricted to psoriatic plaques (i.e. occurs outside of psoriatic plaques); Individual is currently presenting with an acute flare of GPP of moderate to severe intensity, as defined by (Bachelez 2021): A. Generalized Pustular Psoriasis Physician Global Assessment (GPPGA) score of at least 3; AND B. GPPGA pustulation sub score of at least 2 (i.e. moderate to very high density pustules); AND C. Presence of fresh pustules (new appearance or worsening of pustules); AND D. At least 5% of Body Surface area (BSA) covered with erythema and the presence of pustules; If individual has previously received Spevigo treatment for a prior GPP flare*, individual achieved clinical response, as defined as achieving a GPPGA score of 0 or 1, to previous treatment but is now experiencing a new flare (Bachelez 2021). *Treatment for a prior flare may include up to two 900 mg infusions of Spevigo separated by 1 week. Requests for an additional 900 mg dose [2 additional vials] of Spevigo (spesolimab-sbzo) one week after the initial dose for treatment of the same GPP flare may be approved if the following criteria are met: I. Individual is still experiencing persistent symptoms of an acute flare of GPP of moderate to severe intensity, as defined by (Bachelez 2021): A. Generalized Pustular Psoriasis Physician Global Assessment (GPPGA) score of at least 2; AND B. GPPGA pustulation sub score of at least 2 (i.e. moderate to very high density pustules); AND II. Second infusion will take place no sooner than one week after the initial infusion. Requests for Spevigo (spesolimab-sbzo) may not be approved for the following: I. II. III. Individual has plaque psoriasis without pustules or with pustules restricted to psoriatic plaques; OR Tuberculosis, other active serious infections, or a history of recurrent infections; OR If initiating therapy for a new flare, individual has not had a tuberculin skin test (TST) or a Centers for Disease Control (CDC-) and Prevention- recommended equivalent to evaluate for latent tuberculosis prior; OR IV. When the above criteria are not met and for all other indications. Approval Duration: 1 week per infusion Quantity Limits Spevigo (spesolimab-sbzo) Quantity Limit Spevigo (spesolimab-sbzo) 450mg/7.5 mL vial 2 vials [1 carton] per year*^ Drug Limit Override Criteria *Requests for 2 additional vials (1 additional carton) one week after the initial dose for treatment of the same GPP flare may be approved if the following criteria are met: I. Individual is still experiencing persistent symptoms of an acute flare of GPP of moderate to severe intensity, as defined by (Bachelez 2021): A. Generalized Pustular Psoriasis Physician Global Assessment (GPPGA) score of at least 2; AND B. GPPGA pustulation sub score of at least 2 (i.e. moderate to very high density pustules); AND II. Second infusion will take place no sooner than one week after the initial infusion. ^May approve additional vial fills [2 vials, plus 2 additional vials one week later] per criteria above for each subsequent Generalized Pustular Psoriasis (GPP) flare. 2 Coding The following codes for treatments and procedures applicable to this document are included below for informational purposes. Inclusion or exclusion of a procedure, diagnosis or device code(s) does not constitute or imply member coverage or provider reimbursement policy. Please refer to the member's contract benefits in effect at the time of service to determine coverage or non-coverage of these services as it applies to an individual member. HCPCS J1747 Injection, spesolimab-sbzo, 1 mg [Spevigo] ICD-10 Diagnosis L40.1 Generalized pustular psoriasis Document History Revised: 11/17/2023