CurrentBlue Cross Blue Shield - IowaPolicy 05.05.02

Joenja (leniolisib) for activated PI3K delta syndrome (APDS)

Defines coverage criteria, documentation, prescribing specialty, and quantity limits for Joenja (leniolisib) for members with activated PI3K delta (PI3Kδ) syndrome (APDS) age ≥12 and ≥45 kg under Wellmark/Blue Cross Blue Shield - Iowa.

Policy Summary

PayerBlue Cross Blue Shield - Iowa

PolicyJoenja (leniolisib) for activated PI3K delta syndrome (APDS)

Policy CodePolicy 05.05.02

Change TypeNo material changes

Effective DateAug 4, 2023

Next Review Date

Key ActionObtain prior authorization; initial approval may be granted for 6 months when all specified criteria are met.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

6 monthsinitial auth

12 monthscontinuation auth

≥12min age

≥45 kgmin weight

60/30dqty limit

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Initial Therapy

Covered when ALL of the following are met

Initial Approval for Joenja: A. Diagnosis confirmed by detection of a mutation of either PIK3CD or PIK3R1 gene; B. Member has clinical manifestations compatible with APDS (for example: history of repeated oto‑sinopulmonary infections, lymphoproliferation, autoimmunity such as cytopenia, enteropathy, or organ dysfunction); C. Member is 12 years of age or older and weighs ≥45 kg.

Authorization of 6 months may be granted when all criteria are met.

Continuation Therapy

Covered when ALL of the following are met

Continuation Therapy: Member is currently receiving Joenja and is experiencing benefit from therapy as evidenced by improvement in one or more clinical manifestations of disease (for example: decrease in oto‑sinopulmonary infections, decrease in lymphoproliferation, improvement in autoimmunity such as cytopenia).

Authorization of 12 months may be granted.

This policy may not apply to members covered under the Federal Employee Program (FEP). Benefit determinations are based on the applicable contract language in effect at the time services are rendered; exclusions, limitations, or exceptions specific to a member’s contract may alter coverage. Wellmark/Blue Cross Blue Shield — Iowa will determine medical necessity only if the benefit exists and no contract exclusions are applicable.

Use of Joenja (leniolisib) is considered not medically necessary for members who do not meet the specified initial or continuation criteria outlined in this policy. Requests that do not document fulfillment of the coverage criteria (including evidence of clinical benefit for continuation requests) may be denied as not medically necessary.

Prescriber and Provider Requirements

Prior Authorization

Prior authorization required; time-limited approvals

Prior authorization is required. Initial authorization may be granted for 6 months when all specified criteria are met; continuation authorization may be granted for 12 months when the member is experiencing benefit from therapy.

Billing Rule

Dosing limits may apply

Approvals may be subject to dosing limits in accordance with FDA-approved labeling, accepted compendia, and/or evidence‑based practice guidelines.

Documentation Required

Required documentation for initial and continuation requests

For initial requests: submit medical record documentation confirming clinical manifestations of APDS (e.g., cytopenia, enteropathy, organ dysfunction) and testing confirming a mutation of either PIK3CD or PIK3R1. For continuation requests: submit documentation (e.g., chart notes) showing improvement in one or more clinical manifestations of disease (e.g., decreased infections, decreased lymphoproliferation, improved autoimmunity).

Initial: medical record evidence of clinical manifestations (e.g., cytopenia, enteropathy, lung or liver dysfunction)
Initial: testing confirming a mutation of PIK3CD or PIK3R1
Continuation: documentation of clinical benefit (e.g., decreased oto‑sino‑pulmonary infections, decreased lymphoproliferation, improvement in autoimmunity)

Denial Risk

Denial risk if criteria not met

Use of Joenja is considered not medically necessary for members who do not meet the specified initial or continuation criteria.

Background

Activated phosphoinositide 3-kinase delta (PI3Kδ) syndrome (APDS) is an immune dysregulation disorder caused by mutations in PIK3CD or PIK3R1 that leads to recurrent oto‑sinopulmonary infections, lymphoproliferation, autoimmunity (for example, cytopenias), and organ dysfunction. Joenja (leniolisib) is indicated for treatment of APDS in appropriate patients and, when authorized, aims to reduce infections, lymphoproliferation, and autoimmune manifestations.

For continuation of therapy, authorization of 12 months may be granted for members currently receiving Joenja who are experiencing benefit from therapy, as evidenced by improvement in one or more clinical manifestations of disease (for example, decrease in oto‑sinopulmonary infections, decrease in lymphoproliferation, or improvement in autoimmunity). Note that benefit determination and applicability of this policy depend on the member’s contract; this policy may not apply to FEP members.

Definitions

Definition of APDS (PIK3CD or PIK3R1 mutation)

DefinitionActivated phosphoinositide 3-kinase delta (PI3Kδ) syndrome (APDS) is confirmed by detection of a mutation in either PIK3CD or PIK3R1.

Typical clinical featuresClinical manifestations include recurrent oto‑sinopulmonary infections, lymphoproliferation, autoimmunity (e.g., cytopenia), enteropathy, and organ dysfunction.

Age indication (FDA)Indicated for adults and pediatric patients 12 years of age and older.