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Last Review Date: 04/04/2024

Date of Origin: 10/03/2022

Dates Reviewed: 10/2022, 10/2023, 04/2024

1. Length of Authorization ¹

• Treatment of GPP Flare: Coverage will be provided for two doses (900mg each) and may not be renewed.
• Treatment of GPP When Not Experiencing a Flare: Coverage will be provided for 6 months and may be renewed.

2. Dosing Limits

A. Quantity Limit (max daily dose) [NDC Unit]:

• Spevigo 150 mg/mL single-dose pre-filled syringe for subcutaneous use
  • Loading: 4 syringes x 1 dose only
  • Maintenance: 2 syringes every 4 weeks
• Spevigo 450 mg/7.5 mL single-dose vial for intravenous use: 4 vials one time only

B. Max Units (per dose and over time) [HCPCS Unit]:

• Treatment of GPP Flare (IV formulation ONLY)
  • 900 billable units (900 mg) on day 1 and 8
• Treatment of GPP When Not Experiencing a Flare (SQ formulation ONLY)
  • Loading: 600 mg x 1 dose only
  • Maintenance: 300 mg every 28 days

3. Initial Approval Criteria ¹

Coverage is provided in the following conditions:

• Patient is at least 12 years of age and weighs at least 40 kg; AND
• Patient has received all age-appropriate vaccinations according to current immunization guidelines prior to initiating treatment; AND

Universal Criteria ^1-3,6

• Patient does not have any of the following conditions:

• Synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome
• Primary erythrodermic psoriasis vulgaris
• Primary plaque psoriasis vulgaris without presence of pustules or with pustules that are restricted to psoriatic plaques**
  • Drug-triggered Acute Generalized Exanthematous Pustulosis (AGEP)**; AND

• Patient has been evaluated and screened for the presence of latent tuberculosis (TB) infection prior to initiating treatment and will receive ongoing monitoring for presence of TB during treatment; AND
• Patient does not have an active infection, including clinically important localized infections; AND
• Patient will not receive live vaccines (viral and/or bacterial) during therapy; AND
• Patient is not on concurrent treatment with an IL-inhibitor, TNF-inhibitor, biologic response modifier or other non-biologic agent (e.g., apremilast, abrocitinib, tofacitinib, baricitinib, upadacitinib, deucravacitinib, etc.); AND
• Patient will not be on concomitant treatment with systemic immunosuppressants (e.g., retinoids, cyclosporine, methotrexate, etc.) or other topical agents (e.g., corticosteroids, calcipotriene, tacrolimus, etc.); AND

**NOTE: Only applies to patients receiving treatment for a GPP flare

Generalized Pustular Psoriasis (GPP) † Ф ^1-3,6

• Patient is experiencing an acute, moderate-to-severe intensity disease flare; AND
  • Presence of a disease flare is confirmed by the following:
    • Generalized Pustular Psoriasis Physician Global Assessment (GPPPGA) total score of at least 3 (moderate) [the total GPPPGA score ranges from 0 (clear) to 4 (severe)] ¥; AND
    • Presence of fresh pustules (new appearance or worsening of pustules); AND
    • GPPPGA pustulation sub score of at least 2 (mild); AND
    • At least 5% of body surface area (BSA) covered with erythema and the presence of pustules; OR
• Patient is NOT currently experiencing a disease flare; AND
  • Physician has assessed baseline disease severity utilizing an objective measure/tool (e.g., GPPPGA, Dermatology Quality of Life Index (DLQI), Psoriasis Symptom Scale, etc.); AND
  • Patient has a GPPPGA total score of 0 or 1 ¥; AND

• Patient has least 2 GPP flares of moderate-to-severe intensity with fresh pustulation (new appearance or worsening) in the past

† FDA Approved Indication(s); ‡ Compendia Recommended Indication(s); Ф Orphan Drug

4. Renewal Criteria ¹

Coverage can be renewed based upon the following criteria:

• Patients continues to meet universal and other indication-specific relevant criteria identified in section III; AND
• Absence of unacceptable toxicity from the drug. Examples of unacceptable toxicity include: infections, hypersensitivity reactions [including anaphylaxis and delayed reactions such as drug reaction with eosinophilia and systemic symptoms (DRESS)], etc.; AND

Treatment of GPP Flare

• Coverage may not be renewed

Treatment of GPP When Not Experiencing a Flare

• Disease response compared to baseline, as indicated by a decrease in number and/or frequency of GPP flares, stabilization or improvement in GPPPGA total score , improvement in Dermatology Quality of Life Index (DLQI), and/or improvement in Psoriasis Symptom Scale (PSS)

Initiating/Reinitiating Subcutaneous Maintenance Therapy after Treatment of a GPP Flare

• After receiving intravenous treatment for a GPP flare, patients may be initiated on subcutaneous maintenance therapy (Refer to Section III for criteria and Section V for dosing); OR
• Patients experiencing a GPP flare while receiving subcutaneous maintenance therapy may receive up to two intravenous doses to treat the flare (Refer to Section III for criteria and Section V for dosing)

5. Dosage/Administration ¹

6. Billing Code/Availability Information

HCPCS Code(s):

• J1747 – Injection, spesolimab-sbzo, 1 mg; 1 billable unit = 1 mg (IV formulation ONLY)
• J3490 – Unclassified Drugs (SQ formulation ONLY)

NDC(s):

6. Spevigo 150 mg/mL two-pack single-dose pre-filled syringe for subcutaneous use: 0597-0620-xx
7. Spevigo 450 mg/7.5 mL (60 mg/mL) two-pack single-dose vial for intravenous use: 00597-0035-xx
8. References

1. Spevigo [package insert]. Ridgefield, CT; Boehringer Ingelheim Pharmaceuticals, Inc.; March 2024. Accessed March 2024.
2. Bachelez H, Choon SE, Marrakchi S, et al; Effisayil 1 Trial Investigators. Trial of Spesolimab for Generalized Pustular Psoriasis. N Engl J Med. 2021 Dec 23;385(26):2431-2440. doi: 10.1056/NEJMoa2111563.
3. Choon SE, Lebwohl MG, Marrakchi S, et al. Study protocol of the global Effisayil 1 Phase II, multicentre, randomised, double-blind, placebo-controlled trial of spesolimab in patients with generalized pustular psoriasis presenting with an acute flare. BMJ Open. 2021 Mar 30;11(3):e043666. doi: 10.1136/bmjopen-2020-043666.
4. Navarini AA, Burden AD, Capon F, et al. European consensus statement on phenotypes of pustular psoriasis. J Eur Acad Dermatol Venereol. 2017 Nov;31(11):1792–1799. Crossref. PubMed. ISI.
5. Fujita H, Terui T, Hayama K, et al. Japanese guidelines for the management and treatment of generalized pustular psoriasis: the new pathogenesis and treatment of GPP. J Dermatol. 2018 Nov;45(11):1235–1270. Crossref. PubMed. ISI.
6. Morita A, Choon SE, Bachelez H, et al. Design of Effisayil™ 2: A Randomized, Double-Blind, Placebo-Controlled Study of Spesolimab in Preventing Flares in Patients with Generalized Pustular Psoriasis. Dermatol Ther (Heidelb). 2023 Jan;13(1):347-359. doi: 10.1007/s13555-022-00835-6. Epub 2022 Nov 5. PMID: 36333618; PMCID: PMC9823166.
7. Burden AD, Bachelez H, Choon SE, et al. The Generalized Pustular Psoriasis Physician Global Assessment (GPPGA) score: online assessment and validation study of a specific measure of GPP disease activity, British Journal of Dermatology, Volume 189, Issue 1, July 2023, Pages 138–140, https://doi.org/10.1093/bjd/ljad071.

Appendix 1 – Covered Diagnosis Codes

Appendix 2 – Centers for Medicare and Medicaid Services (CMS)

The preceding information is intended for non-Medicare coverage determinations. Medicare coverage for outpatient (Part B) drugs is outlined in the Medicare Benefit Policy Manual (Pub. 100-2), Chapter 15, §50 Drugs and Biologicals. In addition, National Coverage Determinations (NCDs) and/or Local Coverage Determinations (LCDs) may exist and compliance with these policies is required where applicable. Local Coverage Articles (LCAs) may also exist for claims payment purposes or to clarify benefit eligibility under Part B for drugs which may be self-administered. The following link may be used to search for NCD, LCD, or LCA documents: https://www.cms.gov/medicare-coverage-database/search.aspx. Additional indications, including any preceding information, may be applied at the discretion of the health plan.

Medicare Part B Covered Diagnosis Codes (applicable to existing NCD/LCD/LCA): N/A

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