CurrentCignaPolicy N/A

Immune Disorder - Joenja - (IP0568)

Defines Cigna prior authorization and coverage criteria for Joenja (leniolisib) for treatment of activated PI3Kδ syndrome (APDS) in patients ≥12 years and ≥45 kg, including initial and continuation approval durations and required prescriber specialty.

Policy Summary

PayerCigna

PolicyImmune Disorder - Joenja - (IP0568)

Policy CodePolicy N/A

Change TypeName changespecialist list narrowedcontinuation criteria added

Effective DateSep 1, 2024

Next Review Date

Key ActionObtain prior authorization and document pathogenic PI3Kδ variant and clinical response; ensure prescriber is an immunologist or physician who treats primary immune deficiencies.

SourceLink

POLICY UPDATE CHANGES

Policy name updated from 'Leniolisib' to 'Immune Disorder - Joenja.' and effective date set to 09/01/2024; review date 06/27/2024.

Specialist list narrowed by removing pulmonologist, gastroenterologist, hematologist, infectious diseases physician, and medical geneticist; now requires immunologist or physician treating primary immune deficiencies.

For patients currently receiving Joenja, added requirement that patient has been established on therapy for at least 6 months and note to refer to initial therapy if <6 months or restarting.

Added note including examples of positive clinical response for continuation criteria.

Multiple later revision entries indicate 'No criteria changes' on 4/3/2025, 6/1/2025, 4/2/2026, and 5/1/2026.

1Covered Indication (APDS)

12Min Age (years)

45Min Weight (kg)

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Coverage Summary

Defines Cigna prior authorization and coverage criteria for Joenja (leniolisib) for treatment of activated PI3Kδ syndrome (APDS) in patients aged ≥ 12 years and weighing ≥ 45 kg. Initial therapy approvals are for 6 months when all initial criteria are met; continuation (for patients currently receiving Joenja) approvals are for 1 year when continuation criteria are met, including having been established on therapy for ≥ 6 months. The policy requires the medication to be prescribed by or in consultation with an immunologist or a physician who treats primary immune deficiencies.

FDA-Approved Indication - Activated phosphoinositide 3-kinase delta syndrome (APDS)

Approve for the duration noted if the patient meets the following (A or B):

ANY of the following

A) Initial Therapy (Approve for 6 months)
- Patient is ≥ 12 years of age>= 12 years
- Patient weighs ≥ 45 kg>= 45 kg
- Patient has a genetic phosphoinositide 3-kinase delta (PI3Kδ) pathogenic variant in PIK3CD and/or PIK3R1 genes
- Patient has at least one clinical finding or manifestation consistent with APDS
  Examples include recurrent sinopulmonary infections, recurrent herpesvirus infections, lymphadenopathy, hepatomegaly, splenomegaly, nodular lymphoid hyperplasia, autoimmunity, cytopenias, enteropathy, bronchiectasis, and organ dysfunction.
- Medication is prescribed by or in consultation with an immunologist or a physician who treats patients with primary immune deficiencies
  Specialist list was narrowed; pulmonologist, gastroenterologist, hematologist, infectious disease physician, and medical geneticist were removed.
B) Continuation Therapy (Patient currently receiving Joenja) (Approve for 1 year)
- Patient has been established on therapy for at least 6 months>= 6 months
  A patient who has received < 6 months of therapy or who is restarting therapy should be considered under Initial Therapy criteria.
- Patient is ≥ 12 years of age>= 12 years
- Patient weighs ≥ 45 kg>= 45 kg
- Patient has a genetic phosphoinositide 3-kinase delta (PI3Kδ) pathogenic variant in the PIK3CD and/or PIK3R1 genes

Not Medically Necessary / Exclusions

Use outside the FDA-approved indication

Joenja for any other use is considered not medically necessary

Provider Actions / Prior Authorization

Prior Authorization

Prior Authorization Required

Prior Authorization is required for prescription benefit coverage of Joenja. Approvals are provided for the specified durations: initial therapy approvals are for 6 months and continuation therapy approvals are for 1 year.

Documentation Required

Genetic testing documentation

Document the presence of a pathogenic PI3Kδ variant in the PIK3CD and/or PIK3R1 genes to meet the genetic criterion for approval.

Documentation Required

Clinical findings documentation

For initial approval, document at least one clinical manifestation consistent with APDS. For continuation approval, document evidence of a positive clinical response in signs and manifestations of APDS. Examples of clinical findings and positive responses include reduced lymph node size, reduced spleen size, decreased infection rate, decreased immunoglobulin M (IgM) levels, or reduced use of immunoglobulin replacement therapy.

Applicable Codes

DrugNDC

No codes listed

Related coding guidancemixed

No codes listed

Clinical Evidence

31Phase III randomized trial participants

37Open-label extension cohort (≥5 years exposure)

Phase III pivotal trial: a randomized, triple-blind, placebo-controlled study in 31 patients met both co-primary endpoints — reduced lymph node size and increased percentage of naïve B cells, with additional improvements in spleen size, lymphocyte subsets, cytopenias, and IgM levels.

Open-label extension interim analysis: an interim analysis of 37 patients with ≥5 years of leniolisib exposure demonstrated reductions in immunoglobulin replacement therapy use and in the annualized infection rate, with continued improvements in index lymph node size, IgM levels, and percentages of naïve and transitional B cells.

Regulatory reference: See FDA Prescribing Information — Joenja tablets, Pharming; May 2025.

Background & Definitions

Mechanism: Joenja (leniolisib) is a PI3Kδ inhibitor indicated for treatment of activated PI3Kδ syndrome (APDS).

Indication and disease: APDS is an ultrarare autosomal dominant primary immunodeficiency caused by pathogenic variants in PIK3CD or PIK3R1 that result in hyperactivation of the PI3Kδ pathway and lead to immune deficiency and dysregulation with manifestations such as recurrent infections, lymphadenopathy, hepatosplenomegaly, autoimmunity, cytopenias, enteropathy, bronchiectasis, and risk of organ damage or malignancy.

Efficacy summary: Clinical efficacy was demonstrated in a Phase III randomized trial showing significant reductions in lymphadenopathy and increases in naïve B cell percentages, with additional improvements in spleen size, lymphocyte subsets, cytopenias, and IgM; long-term open-label data show sustained immunologic benefit and reductions in infection rate and immunoglobulin replacement use.

APDS: Activated phosphoinositide 3-kinase delta syndrome, a genetic primary immunodeficiency due to PIK3CD or PIK3R1 variants.

Age>= 12 years

Weight>= 45 kg

Establishment on therapy for continuation>= 6 months

Revision History

2024-09-01name_change

Policy name updated from 'Leniolisib' to 'Immune Disorder - Joenja.' (effective 09/01/2024); review date 06/27/2024.

2024-09-01material_change