CurrentPremera BluecrossPolicy N/A

Pharmacologic Treatment of Interstitial Lung Disease

Defines medical necessity criteria, dosing/quantity limits, benefit application (pharmacy vs medical), investigational exclusions, and reauthorization rules for multiple drugs used to treat IPF, PPF, SSc-ILD, and PH-ILD.

Policy Summary

PayerPremera Bluecross

PolicyPharmacologic Treatment of Interstitial Lung Disease

Policy CodePolicy N/A

Change TypeMultiple material updates (coverage, dosing, codes)

Effective DateMay 1, 2026

Next Review Date

Key ActionSubmit prior authorization with HRCT within 24 months, documentation of progression (for PPF: ≥2 of 3 domains within 12 months), prior therapies tried when required, and lab evidence for acute phase reactants for tocilizumab agents; initial and reauthorizations may be approved up to 12 months when supported.

POLICY UPDATE CHANGES

Updated Ofev description to progressive pulmonary fibrosis (PPF) and required progression within last 12 months documented by at least 2 of 3 domains.

Added coverage criteria for Jascayd (nerandomilast) for PPF and updated Jascayd IPF criteria requiring prior use of pirfenidone and Ofev unless contraindicated.

Added Tyenne (tocilizumab-aazg) SC coverage criteria for SSc-ILD and added Avtozma (tocilizumab-anoh) and Yutrepia (treprostinil).

Clarified that re-authorizations and non-formulary exception reviews may be approved up to 12 months; initial authorization durations for Tyvaso/Tyvaso DPI/Yutrepia updated from 6 to 12 months.

Updated Actemra/Tyenne coverage criteria to add elevated acute phase reactants (CRP, ESR, platelet thresholds) and remove DLCO ≥ 45% requirement.

Updated coding: added HCPCS Q5135 (10/01/24) and Q5156 (11/01/25); added Yutrepia to J3490 parenthetical.

MultipleCovered Indications (IPF/PPF/SSc-ILD/PH-ILD)

Coverage Summary

Effective May 1, 2026 (last reviewed Apr 14, 2026). This policy defines medical necessity criteria for multiple agents treating idiopathic pulmonary fibrosis (IPF), progressive pulmonary fibrosis (PPF), systemic sclerosis-associated ILD (SSc-ILD), and pulmonary hypertension-associated ILD (PH-ILD) and summarizes program-level constraints including product-specific dose and quantity limits, prohibited combinations (e.g., nintedanib with pirfenidone; tocilizumab with nintedanib for SSc-ILD), the requirement for chest HRCT within 24 months for IPF/PPF documentation, and a maximum reauthorization/approval duration of up to 12 months where noted.

Numeric and clinical thresholds

FVC thresholds (tocilizumab - SSc-ILD)>= 55% predicted

FVC thresholds (Esbriet / pirfenidone - IPF)>= 50% predicted

DLCO thresholds (Esbriet / pirfenidone - IPF)DLCO 30-79% predicted

FVC thresholds (pirfenidone / Jascayd / Ofev - PPF/IPF)>= 45% predicted for PPF and Jascayd; Ofev IPF >= 50% predicted

DLCO thresholds (PPF / Jascayd / Ofev)DLCO >= 25% predicted for PPF/Jascayd; Ofev PPF DLCO 30-79% predicted

Physiologic progression definitionDecline in FVC >= 5% predicted OR decline in DLCO >= 10% predicted

Radiologic progression requirement (PPF/IPF)HRCT showing increased reticulation, increased traction bronchiectasis/bronchiolectasis, new/increased honeycombing, or increased lobar volume loss

Symptom progression domain (PPF)Worsening respiratory symptoms (e.g., dyspnea and cough)

PPF progression timingDisease progressed within the last 12 months documented by at least 2 of 3 domains (symptoms, physiologic, radiologic)

HRCT recency requirementHRCT performed within the past 24 months (required for IPF and for updated PPF/IPF criteria)

Ofev dose limit300 mg per day (150 mg twice daily)

Pirfenidone (Esbriet) dose limit2,403 mg per day (801 mg three times daily)

Pirfenidone quantity limit90 capsules/tablets per 30 days

Jascayd dose options18 mg/day (monotherapy or with nintedanib) or 36 mg/day when used with pirfenidone

Jascayd quantity limit60 tablets per 30 days

Ofev combination restrictionNot to be used in combination with pirfenidone (for IPF/SSc-ILD/PPF as specified)

Tocilizumab acute phase reactantsOne of: CRP >= 6 mg/mL; ESR >= 28 mm/h; platelet count >= 330 x 10^9/L

Tocilizumab reauthorization FVC ruleChart notes should document decrease in FVC not greater than 10% from previous year for reauthorization

Tyvaso INCREASE dosing referenceTarget 9 breaths (54 mcg) per session up to 12 breaths (72 mcg) per session, four times daily (study dosing)

Tyvaso / Yutrepia initial auth durationInitial authorization updated to 12 months (previously 6 months)

Drug-specific Medical Necessity Criteria

Ofev (nintedanib) — Progressive Pulmonary Fibrosis (PPF)

ALL of the following

The individual is aged 18 years or older.
Has a confirmed diagnosis of progressive pulmonary fibrosis (PPF).
Progression within the last 12 months documented by at least 2 of the following 3 domains:
- Worsening respiratory symptoms (e.g., increased dyspnea or cough).
- Physiologic progression: decline in FVC of ≥5% predicted OR decline in DLCO of ≥10% predicted.

Prior Authorization, Documentation & Billing Actions

Prior Authorization

Prior authorization required

Prior authorization is required for all covered ILD therapies listed in this policy. Include documentation of the confirmed diagnosis (see Diagnosis and testing documentation below), the most recent HRCT report (within required timeframe per drug), objective measures of disease severity and progression (FVC, DLCO, 6MWD where applicable), prior therapy history, and relevant laboratory testing for biologics (e.g., baseline and periodic liver enzymes, CBC for tocilizumab). For tocilizumab specifically include indication for SSc‑ILD, FVC % predicted, acute phase reactant results, and documentation the prescriber is a pulmonologist or rheumatologist.

Diagnosis: confirmed diagnosis per drug-specific criteria (IPF, PPF, SSc‑ILD) with exclusion of alternative causes where required
Imaging: HRCT report (timing per drug — generally within 24 months for pirfenidone/Jascayd/IPF; for progression assessments include interval comparison)
Physiology: FVC % predicted and DLCO % predicted (include dates); 6MWD when requested for Tyvaso reauthorization
Prior therapies: trials and responses or intolerances (e.g., prior generic pirfenidone trial before brand Esbriet; prior pirfenidone and nintedanib trials before Jascayd)
Labs for tocilizumab: baseline and periodic liver function tests and CBC; documentation of elevated acute phase reactants as required for SSc‑ILD

Coding and Claiming Guidance

HCPCS / J-codes and other injectable codes listedHCPCS

J3262	Injection, tocilizumab (Actemra), 1 mg
Q5135	Injection, tocilizumab-aazg (Tyenne), biosimilar, 1 mg
Q5156	Injection, tocilizumab-anoh (Avtozma), biosimilar, 1 mg (new code effective 10/01/25)
J7686	Treprostinil (Tyvaso), inhalation solution, FDA-approved final product, non-compounded, administered through DME, unit dose form, 1.74 mg
J3490	Unclassified drug (use to report: Yutrepia)

HCPCS / J-code updates and additionsmixed

Q5135	Added (coding update 10/01/24)
Q5156	Added for Avtozma (tocilizumab-anoh) (11/01/25)
J3490	Yutrepia was added to the parenthetical of J3490 (11/01/25)

Coding updates: new HCPCS Q5135 was added effective 10/01/24 and HCPCS Q5156 was added effective 11/01/25. Guidance notes using J3490 with a parenthetical for Yutrepia when applicable. Benefit application guidance distinguishes pharmacy-managed agents (generic/brand pirfenidone, Esbriet, Jascayd, Ofev, Tyvaso DPI) versus agents managed through both pharmacy and medical benefit (Actemra, Avtozma, Tyenne, Tyvaso, Yutrepia) as documented in provider actions.

Background and Evidence Summary

Background: the policy addresses antifibrotic agents (nintedanib/Ofev, pirfenidone/Esbriet and generic/brand pirfenidone), the PDE4 inhibitor Jascayd (nerandomilast), IL‑6 receptor antagonists (tocilizumab and biosimilars), and inhaled prostacyclin therapies (treprostinil formulations: Tyvaso, Tyvaso DPI, Yutrepia). Diagnostic and classification references include the ATS/ERS/JRS/ALAT guidance for IPF diagnosis and the 2013 ACR/EULAR systemic sclerosis classification referenced for SSc-ILD. Pivotal evidence summarized includes the INPULSIS trials demonstrating nintedanib reduced annual FVC decline, CAPACITY/ASCEND and related trials supporting pirfenidone efficacy, FIBRONEER trials supporting Jascayd efficacy on FVC, the ACTEMRA SSc trials showing benefit in SSc-ILD FVC measures, and the INCREASE study demonstrating inhaled treprostinil improved 6MWD in PH-ILD.

Study	Key result
INPULSIS-1 & INPULSIS-2
Nintedanib 150 mg BID reduced annual FVC decline by 125.5 mL/year (INPULSIS-1) and 93.7 mL/year (INPULSIS-2) versus placebo (both p<0.001).
FIBRONEER-IPF (Jascayd)
Jascayd 18 mg demonstrated smaller mean FVC decline over 52 weeks: 106 mL (18 mg) and 122 mL (9 mg) vs 170 mL with placebo; primary endpoint met.
ASCEND / CAPACITY (pirfenidone)
Pirfenidone 2,403 mg/day showed significant FVC benefit: CAPACITY 004 absolute mean FVC difference 4.4% (72 weeks) and ASCEND 4.8% (72 weeks); mixed results across trials.
FIBRONEER-ILD (Jascayd in PPF)
Jascayd reduced FVC decline at 52 weeks in PPF: mean declines 86 mL (18 mg) and 69 mL (9 mg) vs 152 mL with placebo (treatment differences 65 mL and 83 mL).
INCREASE (Tyvaso)
In PH-ILD, inhaled treprostinil (Tyvaso) produced a placebo-corrected median improvement in 6MWD of 21 meters at Week 16 (p=0.004).
Study WA29767 / Actemra (tocilizumab)
In SSc-ILD subgroup, tocilizumab showed differences in mean changes to Week 48 vs placebo of 6.47% ppFVC and 241 mL observed FVC.

Key Definitions

Definitions / brief disease descriptions and the policy progression definition:

IPF: idiopathic pulmonary fibrosis — a chronic, progressive fibrosing interstitial pneumonia typically with a HRCT and/or histopathologic pattern of usual interstitial pneumonia (UIP), diagnosed after exclusion of other known causes per ATS/ERS/JRS/ALAT criteria.

PPF: progressive pulmonary fibrosis — chronic fibrosing ILD with documented progression; for coverage the policy requires disease progression within the last 12 months documented by ≥2 of the 3 domains: (1) worsening respiratory symptoms (e.g., dyspnea, cough); (2) physiological progression demonstrated by decline in FVC by ≥5% predicted OR decline in DLCO by ≥10% predicted; and (3) radiologic progression on HRCT (examples include increased reticulation, increased traction bronchiectasis/bronchiolectasis, new or increased honeycombing, or increased lobar volume loss).

SSc-ILD: systemic sclerosis-associated interstitial lung disease — interstitial lung disease occurring in individuals meeting the 2013 ACR/EULAR classification criteria for systemic sclerosis.

Revision History

05/01/26revisedLatest

Updated Ofev description to progressive pulmonary fibrosis (PPF) and required progression within last 12 months documented by at least 2 of 3 domains.

11/01/25added

Updated Actemra/Tyenne coverage criteria to add elevated acute phase reactants (CRP, ESR, platelet thresholds) and remove DLCO ≥ 45% requirement.

04/01/25revised

Clarified that re-authorizations can be approved for up to 12 months and non-formulary exception review authorizations for all drugs listed may be approved up to 12 months; clarified medications are subject to FDA dosing/administration.

Policy Summary

PayerPremera Bluecross

PolicyPharmacologic Treatment of Interstitial Lung Disease

Policy CodePolicy N/A

Change TypeMultiple material updates (coverage, dosing, codes)

Effective DateMay 1, 2026

Next Review Date

On This Page

Jascayd (nerandomilast) — PPF and IPF

Jascayd (nerandomilast) — Progressive Pulmonary Fibrosis (PPF) and Idiopathic Pulmonary Fibrosis (IPF)

ALL of the following

The individual is aged 18 years or older.
Diagnosis
- Has a confirmed diagnosis of idiopathic pulmonary fibrosis (IPF) documented by: exclusion of other known causes of ILD AND presence of HRCT pattern of usual interstitial pneumonia (UIP) OR specific combinations of HRCT and histopathology if lung tissue sampling performed.
- Has a confirmed diagnosis of progressive pulmonary fibrosis (PPF) (see progression criteria).
HRCT of the chest performed within the last 24 months documenting fibrotic ILD.
Prescribed by or in consultation with a pulmonologist.
For IPF: Forced vital capacity (FVC) ≥45% predicted and DLCO ≥25% predicted. For PPF: FVC ≥45% predicted and DLCO ≥25% predicted.
PPF-specific progression
- Disease has progressed within the last 12 months despite appropriate management of the underlying ILD.
- Progression documented by at least 2 of the following 3 domains: worsening respiratory symptoms; physiologic decline (FVC decline ≥5% predicted or DLCO decline ≥10% predicted); radiologic progression on HRCT.
Patient has tried pirfenidone and nintedanib (Ofev) unless contraindicated, or had intolerance/inadequate response. Jascayd may be used in combination with pirfenidone or nintedanib if there is documented worsening of symptoms and/or lung function.
Dose options
- 18 mg per day.
- 36 mg per day when used in combination with pirfenidone (to account for interactions).
Quantity limits: up to 60 tablets per 30 days when used for IPF; up to 90 capsules/tablets per 30 days in other dosing scenarios per formulary limits (document quantity requested).

Tyvaso / Tyvaso DPI / Yutrepia (treprostinil) — PH‑ILD and PAH

Tyvaso (treprostinil) Inhalation Solution, Tyvaso DPI, and Yutrepia (treprostinil) — Pulmonary Hypertension associated with ILD (PH-ILD) and Pulmonary Arterial Hypertension (PAH)

ALL of the following

Indication: treatment of pulmonary hypertension associated with interstitial lung disease (PH‑ILD) or pulmonary arterial hypertension (PAH) as appropriate to the specific product labeling or INCREASE study context.
Prescribed by or in consultation with a pulmonologist or a cardiologist experienced in treating pulmonary hypertension.
Dose and administration consistent with FDA labeling or clinical trial evidence (INCREASE study used inhaled treprostinil titrated to effect; document starting dose, titration plan, and target dose).
Initial authorization may be granted up to 12 months when documentation supports indication and initial response/tolerability assessment plan.
Quantity limits: follow product-specific maximum daily dose per labeling (document requested quantity).
For PH‑ILD, reference INCREASE trial dosing and outcomes in the clinical record; ensure inhalation device formulation matches prescribing and billing (Tyvaso vial inhalation solution vs Tyvaso DPI vs Yutrepia cartridge/formulation).

Prior Authorization

Initial authorization duration

Initial approvals are typically granted for up to 12 months. Requests should include baseline objective measures (FVC, DLCO, HRCT) and prior therapy history to support starting therapy.

Standard initial authorization period: up to 12 months
Include baseline FVC and DLCO values and most recent HRCT with initial request

Prior Authorization

Reauthorization requirements

Reauthorization requires documentation of clinical benefit or lack of unacceptable decline. Provide interval objective data (FVC, DLCO, symptoms, HRCT when relevant) demonstrating stability or improvement. For tocilizumab and pirfenidone, show FVC stability or improvement as part of the rationale for continued therapy.

Provide interval FVC % predicted and DLCO % predicted with dates
Document symptom course (e.g., dyspnea, cough) and any radiologic change on HRCT
For tocilizumab/pirfenidone: evidence of clinical benefit or FVC stabilization compared with baseline

Prior Authorization

Tyvaso reauthorization requirement

Tyvaso reauthorization requires objective functional improvement. Include pre‑ and post‑treatment 6‑minute walk distance (6MWD) demonstrating the required improvement threshold per medical policy.

Submit baseline 6MWD and most recent 6MWD with dates
Document concomitant therapies and clinical status at time of reauthorization

Billing Rule

Dose and quantity limits

Dose and quantity limits apply per product — follow the Dosage and Quantity Limit table when preparing claims. Key limits summarized below should be used to verify dosing and to prevent over‑provisioning.

Pirfenidone (brand & generic): limit 2,403 mg/day (801 mg TID); quantity limit 90 tablets/capsules per 30 days
Jascayd (nerandomilast): quantity limit 60 tablets per 30 days; dosing 18 mg/day or 36 mg/day when used with pirfenidone
Ofev (nintedanib) products: follow product‑specific dosing and quantity caps in the table
Tyvaso and Tyvaso DPI products: dose/quantity limits per product — reference Dosage and Quantity Limit table

Billing Rule

Benefit application (pharmacy vs medical)

Benefit administration differs by product. Some agents are pharmacy‑managed while others are billed to medical benefit; a few products may be handled in mixed settings depending on formulation and site of care. Verify benefit assignment prior to claim submission.

Pharmacy‑managed products: most oral agents (pirfenidone, Jascayd, Ofev) are typically pharmacy benefit
Medical‑managed products: some injectable or inhaled therapies (certain formulations of tocilizumab, Tyvaso depending on setting) may be billed to medical benefit
Mixed handling: check specific product NDC/HCPCS and site of care to determine pharmacy vs medical billing

Documentation Required

Authorization duration and reauthorization

Authorizations (initial and reauthorization) may be granted up to 12 months. Reauthorization requests must include the documentation described above (objective measures, symptom status, HRCT comparison where applicable) to support continued medical necessity.

Maximum authorization duration per approval: up to 12 months
Reauthorization documentation: interval FVC/DLCO, symptom assessment, HRCT comparisons when used to demonstrate progression or stability

Billing Rule

Dose and quantity limits (billing specifics)

Billing-specific dose and quantity rules must be applied at claim adjudication. Ensure claims reflect per‑product dose caps and the correct claim type (pharmacy vs medical). Overlapping supply limits across benefits (e.g., pharmacy dispensed oral agents vs medical‑billed injectables) should be reconciled before submitting.

Apply per‑product dosing and quantity caps when adjudicating pharmacy or medical claims
Reference which products and claim types are impacted (oral agents: pharmacy; injectables/inhaled: may be medical)
Ensure units billed match approved quantity limits and dosing frequency