IPF Drug Pipeline 2026: Nerandomilast & Phase 2 Candidates

Quick Answer: IPF therapy saw a decade-first wave of approvals in 2025-2026: Nerandomilast (preferential PDE4B inhibitor) received US FDA approval in October 2025 for adult IPF AND adult PPF, and inhaled Treprostinil's TETON-2 met its primary endpoint with publication in NEJM 2026 (PMID 41812190). Phase 2/2a readouts from ENV-101 (Hedgehog), Buloxibutid (AT2R) and Rentosertib (TNIK) now show FVC improvement signals, prompting a shift in research framing from "progression suppression" toward "disease modification" — though these Phase 2 signals require Phase 2b/3 confirmation and have not been adopted into guidelines.

1. Introduction: IPF Drug Development in the Post-Nerandomilast Era (2026 Update)

Idiopathic Pulmonary Fibrosis (IPF) is a chronic, progressive fibrotic interstitial lung disease with an extremely poor prognosis following diagnosis. Historically, the therapeutic strategy for IPF has been focused on slowing the rate of lung function decline — termed "progression suppression." Since the establishment of pirfenidone and nintedanib as Standard of Care (SoC), there had been an "innovation gap" of approximately 10 years with no new drug approvals.

In 2025-2026, this stagnation began to break. Nerandomilast (Jascayd), a preferential PDE4B inhibitor, received US FDA approval in October 2025 for adult IPF and adult PPF (see our detailed Nerandomilast analysis), and inhaled treprostinil's TETON-2 trial was published in N Engl J Med 2026 (Nathan SD et al, PMID 41812190). Meanwhile, Phase 2/2a data from multiple candidates suggest not just disease stabilization, but FVC improvement signals — though all such signals come from small, short-term, exploratory studies pending Phase 2b/3 confirmation.

This report comprehensively analyzes the clinical trial results and mechanisms of action (including preclinical data) across the IPF pipeline, with a translational perspective connecting each drug's pathophysiological target with its clinical outcomes.

2. Current Approved Therapies and Standard of Care

Pirfenidone and nintedanib were the two drugs approved for IPF treatment before October 2025 and continue to anchor guideline-recommended therapy; nerandomilast's incorporation into guidelines will follow at the pace of each society's revision cycle. Both pirfenidone and nintedanib slow disease progression but do not halt it, and tolerability concerns remain.

2.1 Pirfenidone

• Brand Name: Pirespa (Japan), Esbriet (US/EU)
• Developer: Shionogi (Japan), Roche/Genentech (Global)

Pirfenidone is the world's first approved antifibrotic agent for IPF, possessing a pleiotropic mechanism of action.

2.1.1 Clinical Efficacy and Safety

Pirfenidone's approval was primarily based on the multinational Phase 3 ASCEND and CAPACITY trials. In ASCEND, the pirfenidone group demonstrated significant suppression of FVC decline at 52 weeks compared to placebo, reducing the risk of disease progression by approximately 48%. Pooled analyses also suggested reductions in all-cause and IPF-related mortality, and respiratory-related hospitalization risk.

Japan-Specific Dosing Notably, the approved dose in Japan (1,200–1,800 mg/day) differs from the Western standard (2,403 mg/day). Japanese Phase 3 trials showed significant FVC preservation at both dose levels, and Korean real-world data confirmed efficacy and tolerability at ≤1,800 mg/day, suggesting that Asian patients may achieve therapeutic effects at lower doses.

2.1.2 Mechanism of Action (Preclinical Evidence)

• TGF-β Pathway Suppression: Inhibits TGF-β production and activation, blocking fibroblast-to-myofibroblast differentiation and collagen synthesis.
• Anti-inflammatory Cytokine Modulation: Reduces IL-1β, IL-6, TNF-α, IFN-γ, and MCP-1 in bleomycin-induced lung fibrosis models.
• Direct Collagen Fibril Inhibition: Recent research reveals pirfenidone directly inhibits collagen type I fibril formation, with electron microscopy showing shortened and thinned collagen fiber bundles.

2.1.3 Safety Considerations

Key adverse effects include photosensitivity, rash, nausea, and GI symptoms. Approximately 15% of patients in ASCEND/CAPACITY discontinued treatment due to side effects.

2.2 Nintedanib

• Brand Name: Ofev
• Developer: Boehringer Ingelheim

Nintedanib is a tyrosine kinase inhibitor (TKI) targeting multiple growth factor receptors involved in fibrosis.

2.2.1 Clinical Efficacy and Indication Expansion

In the Phase 3 INPULSIS trials, nintedanib (150 mg BID) significantly reduced the annual rate of FVC decline in IPF patients. Pooled analysis showed FVC decline of -113.6 mL/year vs -223.5 mL/year for placebo — approximately 50% suppression.

Expansion to PF-ILD Nintedanib is uniquely approved for progressive fibrosing interstitial lung diseases (PF-ILD) and systemic sclerosis-associated ILD (SSc-ILD), clinically proving that antifibrotic therapy can be effective across different etiologies sharing the common pathology of progressive fibrosis.

2.2.2 Mechanism of Action (Preclinical Evidence)

Nintedanib potently inhibits three receptor tyrosine kinases:

1. PDGFR-α/β
2. FGFR-1/2/3
3. VEGFR-1/2/3

• In vitro: Suppresses TGF-β-stimulated collagen I/III/V expression, fibronectin secretion, and collagen chaperone (FKBP10, HSP47) expression in IPF patient-derived fibroblasts.
• In vivo: Significantly reduces α-SMA expression and tissue fibrosis in bleomycin-induced and joint contracture models.

2.2.3 Safety and Drug Interactions

Diarrhea occurs in >60% of patients. Hepatic function monitoring is required. Pharmacokinetic studies confirm that co-administration with pirfenidone does not significantly alter either drug's plasma levels — an important foundation for future combination therapy.

3. Phase 3 Breakthroughs: The First New Approvals in a Decade

3.1 Nerandomilast (Jascayd): Preferential PDE4B Inhibition

• Developer: Boehringer Ingelheim
• Approval: US FDA approved (October 2025) for adult IPF and adult PPF as standalone monotherapy indications (DailyMed label^{[ref-Jascayd]}). Outside the US (Japan, EU, etc.), regulatory review or filing status varies by region and a specific timeline cannot be inferred from public information.
• Deep Dive: Nerandomilast (PDE4B Inhibitor): Mechanism, Clinical Data & Preclinical Evidence

Nerandomilast (BI 1015550) is an oral preferential phosphodiesterase 4B (PDE4B) inhibitor — the wording used in the Phase 2 NEJM 2022 paper title "Trial of a Preferential Phosphodiesterase 4B Inhibitor." By preferentially targeting the PDE4B subtype (highly expressed in inflammatory cells and fibroblasts), it is designed to mitigate the central side effects associated with PDE4D blockade by non-selective PDE4 inhibitors. As an oral drug, systemic exposure exists; it is not lung-restricted.

3.1.1 Phase 3 FIBRONEER-IPF Results

The pivotal trial (NCT05321069) enrolled 1,177 IPF patients and was stratified by background antifibrotic therapy; 77.7% were on pirfenidone or nintedanib at baseline^{[ref-FIBRONEER-IPF]}.

• Primary Endpoint (FVC Change at week 52, adjusted means) per N Engl J Med 2025;392(22):2193-2202:
  • Nerandomilast 18 mg: -114.7 mL (95% CI -141.8 to -87.5); p<0.001 vs placebo
  • Nerandomilast 9 mg: -138.6 mL (95% CI -165.6 to -111.6); p=0.02 vs placebo
  • Placebo: -183.5 mL (95% CI -210.9 to -156.1) The pattern of FVC-decline reduction was broadly consistent across strata defined by background antifibrotic use.
• Secondary Endpoints: The composite of "acute exacerbation, respiratory hospitalization, or death" did not show a clear between-group difference, leaving long-term follow-up as an open question.

3.1.2 Expansion to PPF

The parallel FIBRONEER-ILD trial (NCT05321082, Maher TM et al, N Engl J Med 2025, PMID 40388329) showed reduction of FVC decline in PPF patients. Based on these results, the US FDA approved nerandomilast for adult PPF as well, and the current Jascayd label (DailyMed) lists both "treatment of idiopathic pulmonary fibrosis in adult patients" and "treatment of progressive pulmonary fibrosis in adult patients" as standalone monotherapy indications^{[ref-Jascayd]}.

3.1.3 Preclinical Mechanism

• PDE4B selectivity: Elevates intracellular cAMP, suppressing pro-inflammatory cytokines (TNF-α, IL-6) and inhibiting fibroblast proliferation and myofibroblast differentiation.
• Bleomycin/silica models: Suppresses macrophage and neutrophil infiltration, dramatically reduces TGF-β1 and IL-6 expression, and inhibits NLRP3 inflammasome activation.
• Human cells: Induces myofibroblast dedifferentiation and enhances endothelial barrier function in IPF patient-derived fibroblasts.

3.2 Inhaled Treprostinil (Tyvaso): Dual Targeting of Vasculature and Fibrosis

• Developer: United Therapeutics
• Status: TETON-2 published in N Engl J Med 2026 (Nathan SD et al, PMID 41812190). TETON-1 is at United Therapeutics topline investor release stage, with PubMed publication not yet confirmed at the time of this article — treat as topline data only. FDA sNDA filing planned Q3 2026
• Deep-dive article: Inhaled Treprostinil (Tyvaso): TETON-1/2 Results in IPF

3.2.1 Phase 3 TETON-2 Results

TETON-2 (NCT05255991, Nathan SD et al, NEJM 2026)^{[ref-TETON-2]} randomized n=593 IPF patients (298 treprostinil + 295 placebo); 75.4% were on background antifibrotic therapy.

• Primary Endpoint: Between-group difference in absolute FVC change at week 52: +95.6 mL (95% CI 52.2 to 139.0; P<0.001) in favor of treprostinil (treprostinil median -49.9 mL vs placebo -136.4 mL).
• Background-therapy subgroup signal: A subgroup analysis suggested a more pronounced treatment effect in patients on background antifibrotic therapy. This is consistent with a hypothesis of additive activity via a different (cAMP) mechanism, but a "synergistic" pharmacological interaction has not been formally demonstrated — "additive effect suggested by subgroup analysis" is the precise framing.
• Other endpoints: Clinical worsening was lower with treprostinil (27.2% vs 39.0%, p=0.02).

3.2.2 Preclinical Mechanism

• Direct antifibrotic action: Elevates cAMP via IP, EP2, DP1 receptors and PPARs, potently suppressing TGF-β and PDGF-induced fibroblast proliferation and collagen synthesis.
• Mitochondrial function restoration: Normalizes fusion/fission abnormalities in IPF-derived fibroblasts.

4. Promising Phase 2 Candidates: FVC Improvement Signals Awaiting Replication

Phase 2/2a data have begun to show FVC improvement signals (rather than just stabilization). However, all are short-term, small-sample exploratory trials and cannot establish "reversal" or "cure" without Phase 2b/3 replication.

4.1 BMS-986278 (Admilparant): LPA1 Antagonist

• Developer: Bristol Myers Squibb
• Phase 2 Data (Corte TJ et al, Am J Respir Crit Care Med 2025;211(2):230-238, PMID 39393084)^{[ref-admilparant]}: 60 mg BID reduced FVC decline at 26 weeks in IPF and PPF cohorts vs placebo.
• Phase 3: ALOFT-IPF and ALOFT-PPF trials ongoing (completion ~2026–2027).

4.2 ENV-101 (Taladegib): HRCT Structural Signal Alongside FVC Improvement

• Developer: Endeavor BioMedicines
• Target: Hedgehog (Hh) pathway
• Phase 2a Data (Maher TM et al, Lancet Respir Med 2025;13(11):1001-1010, PMID 41043447)^{[ref-ENV-101]}: n=41 (taladegib 21 + placebo 20), 12-week trial. FVC %predicted +1.9% (taladegib) vs -1.3% (placebo). AI-analyzed HRCT showed total lung capacity +206.67 mL vs -55.58 mL and reduced pulmonary vascular volume.
• Important caveat: At n=41 in an exploratory Phase 2a, these structural findings cannot be generalized as "fibrosis reversal." Replication in Phase 2b WHISTLE-PF (NCT06422884) is required.

4.3 Buloxibutid (C21): AT2 Receptor-Mediated Tissue Repair Hypothesis

• Developer: Vicore Pharma
• Phase 2a (AIR Trial): FVC reported to increase by +216 mL from baseline over 36 weeks based on Vicore Pharma company disclosure. As of this article, no peer-reviewed primary publication is identifiable on PubMed, so this figure should be treated as company / conference-derived data. Historical-control comparisons rest entirely on the AIR trial's own analysis.
• Mechanism (hypothesis): AEC2 protection and regeneration; biomarker analysis suggested elevated MMP-13 (preclinical / translational range).
• Current: Phase 2b ASPIRE (NCT06588686, 52-week placebo-controlled) ongoing, with no published results yet.

4.4 ISM001-055 (Rentosertib): AI-Discovered Target

• Developer: Insilico Medicine
• Target: TNIK (Traf2- and NCK-Interacting Kinase) — a kinase positioned at the intersection of TGF-β, Wnt, and YAP/TAZ signaling
• Phase 2a Data (Xu Z, Ren F, et al, Nat Med 2025;31(8):2602-2610, PMID 40461817, NCT05938920)^{[ref-rentosertib]}: n=71 (18+18+18 across three dose groups + 17 placebo) Phase 2a trial. Primary endpoint was safety (TEAE rate); FVC was an exploratory/secondary outcome. 60 mg QD: FVC +98.4 mL (95% CI 10.9 to 185.9) vs placebo -20.3 mL at 12 weeks.
• Interpretation caveat: Primary endpoint was safety; FVC improvement is exploratory and n=71 is small. The result represents proof-of-concept of the AI-discovery process rather than established efficacy.
• Significance: One of the first cases where an AI-discovered target and AI-designed molecule produced an early-phase safety + exploratory FVC signal in human trials.

4.5 Other Notable Candidates

• Axatilimab (Syndax/Incyte): Anti-CSF-1R antibody targeting pro-fibrotic macrophages. MAXPIRe Phase 2 trial ongoing for IPF.
• HZN-825 (Amgen/Horizon): Another LPA1 antagonist in Phase 2b.

5. Lessons from Failures: Late-Stage Discontinuations

5.1 Pamrevlumab (ZEPHYRUS-1)

• Target: CTGF
• Did not show significance at 48 weeks (p=0.29, company disclosure). Proposed design-related explanations include possible regression-to-the-mean in the smaller Phase 2 and difficulty detecting add-on effects on top of SoC — these are hypotheses raised in discussion rather than confirmed mechanistic conclusions.

5.2 Bexotegrast (BEACON-IPF)

• Target: αvβ6/αvβ1 integrin
• An interim analysis showed an unfavorable safety signal (respiratory hospitalization/death) compared to placebo despite earlier FVC trends, and the trial was terminated early (company disclosure). One possible mechanistic explanation is that integrins play roles in pulmonary immune defense and homeostasis beyond TGF-β activation, and that strong inhibition may have compromised those functions — but a definitive mechanism for the safety signal has not been established.

5.3 TTI-101 (REVERT)

• Target: STAT3
• Per company disclosure, did not demonstrate FVC efficacy and had high dropout rates from GI side effects. The smaller-than-expected placebo decline was also raised as an interpretation point for trial design.

6. Conclusion and Future Outlook (Updated April 2026)

2025 was a landmark year for IPF drug development, and 2026 is the year of clinical implementation:

• New approvals expanding the toolbox: Following nerandomilast's US approval (October 2025, IPF + PPF as standalone monotherapy indications), regulatory review or filing status in regions outside the US (Japan, EU) varies by agency. Inhaled treprostinil's US sNDA is planned for Q3 2026. Because FIBRONEER-IPF allowed background antifibrotic therapy, combination use is reflected in trial design; how these drugs will be combined in actual practice will depend on evolving guidelines and prescribing data.
• FVC improvement signals — replication phase: Phase 2/2a data from ENV-101, buloxibutid, and ISM001-055 are short-term, small-sample exploratory readouts (n=41–71) showing FVC improvement signals. "Reversal" or "cure" cannot be claimed from these data; the next milestones are Phase 2b/3 replication trials (WHISTLE-PF, ASPIRE, ALOFT-IPF) emerging from 2026 onward.
• Lessons from Failures: Bexotegrast's early termination is an important signal about potential safety risks of strong pathway inhibition, though the underlying mechanism has not been definitively established. Future development demands careful consideration of target biology complexity.

The future direction of IPF therapy is often described in terms of precision medicine — selecting optimal molecular-targeted drug combinations based on individual patient profiles. This is an aspirational research and development framework rather than an established guideline-recommended algorithm at present.

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Appendix: IPF Drug Comparison Tables

Table 1: Currently Approved IPF Therapies (as of April 2026)

Drug (Generic)	Brand	Mechanism	Approval (JP/US/EU)	Key Clinical Reference
Pirfenidone	Pirespa / Esbriet	Pleiotropic antifibrotic (TGF-β, collagen)	✓/✓/✓	ASCEND (NEJM 2014; PMID: 24836312)
Nintedanib	Ofev	TKI (PDGFR/FGFR/VEGFR)	✓/✓/✓	INPULSIS (NEJM 2014; PMID: 24836313)
Nerandomilast	Jascayd	Preferential PDE4B inhibitor	US: adult IPF + PPF approved (Oct 2025). JP/EU: under varying regional review/filing[ref-Jascayd]	FIBRONEER-IPF (NCT05321069). Deep dive →

Table 2: Promising Phase 3/2 Clinical Candidates

Stage	Drug (Code)	Target/Mechanism	Key Result
Approved (US)	Nerandomilast (BI 1015550)	Preferential PDE4B inhibitor	FIBRONEER-IPF (Ph3): 18 mg -114.7 mL vs placebo -183.5 mL (p<0.001, PMID 40387033). US adult IPF + PPF approved (Oct 2025).
Ph3	Treprostinil (inhaled)	Prostacyclin/cAMP	TETON-2 (Ph3, n=593, PMID 41812190): between-group difference +95.6 mL (95% CI 52.2-139.0; P<0.001). TETON-1 topline release only.
Ph3	BMS-986278 (Admilparant)	LPA1 antagonist	Ph2 (Corte TJ, AJRCCM 2025;211(2):230-238, PMID 39393084): FVC decline reduction over 26 weeks. ALOFT (Ph3) ongoing.
Ph2b	ENV-101 (Taladegib)	Hedgehog inhibitor	Ph2a, n=41 (Maher TM, Lancet Respir Med 2025;13(11):1001-1010, PMID 41043447): FVC %pred +1.9% vs -1.3%; TLC +206.67 mL vs -55.58 mL. Exploratory; WHISTLE-PF Ph2b for replication.
Ph2b	Buloxibutid (C21)	AT2R agonist	Ph2a (AIR): FVC +216 mL over 36 weeks reported via Vicore Pharma company disclosure; no PubMed-indexed primary publication identified. ASPIRE Ph2b ongoing, results pending.
Ph2a	ISM001-055 (Rentosertib)	TNIK inhibitor (AI-designed)	Ph2a, n=71 (Xu Z, Ren F, Nat Med 2025;31(8):2602-2610, PMID 40461817): primary endpoint = safety. FVC 12-week +98.4 mL (95% CI 10.9-185.9) vs placebo -20.3 mL (exploratory). AI-discovery PoC.

Table 3: Key Preclinical References

Drug	Preclinical Model	Key Findings	PMID
Pirfenidone	Bleomycin mouse	TGF-β suppression, cytokine reduction	Multiple
Nintedanib	Bleomycin mouse/rat	PDGFR/FGFR/VEGFR inhibition, α-SMA reduction	Multiple
Nerandomilast	Bleomycin mouse (SSc-ILD)	TGF-β1/Smad pathway suppression	39438343
Treprostinil	Bleomycin mouse	Lung injury, vascular remodeling attenuation	31819797
BMS-986278	Bleomycin mouse	Fibrosis suppression, LPA signal blockade	34726410
ENV-101	Phase 2a clinical	Hedgehog inhibition, FVC improvement, TLC increase	41043447

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Related Articles

• IPF Clinical Trials Review: Phase 2-3 Key Candidates — Detailed analysis of FIBRONEER, TETON, and other IPF clinical trials
• Inhaled Treprostinil (Tyvaso): TETON-1/2 Results in IPF — Deep-dive on the prostacyclin strategy for IPF
• Galectin-3 Complete Guide — GB0139 (inhaled Gal-3 inhibitor) drug development in IPF
• Integrated Stress Response (ISR) and Fibrosis — ABBV-CLS-7262 (ISRIB derivative) for IPF
• HIF Pathway and Fibrosis — HIF-2α vascular remodeling in IPF with pulmonary hypertension
• Lung Fibrosis Mouse Model Selection Guide 2026 — Bleomycin/Silica/FITC/aged model comparison aligned with Jenkins 2017 ATS guidance
• IPF vs PPF: Progressive Pulmonary Fibrosis Explained — Shared fibrotic pathway, INBUILD trial, and nintedanib indication expansion

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FAQ

Q: Can Nerandomilast (Jascayd) be combined with existing pirfenidone or nintedanib? A: FIBRONEER-IPF (NCT05321069) was stratified by background antifibrotic therapy, and 77.7% of patients were on pirfenidone or nintedanib at baseline (PMID 40387033). The FVC-preservation effect pattern was broadly consistent across these strata. The current Jascayd label (DailyMed setid fa1b14c6-957f-d326-5099-911bfe33e391) describes adult IPF and adult PPF as standalone monotherapy indications; the label does not explicitly prohibit combination with existing antifibrotics, and the trial design itself included background-therapy use. GI tolerability monitoring (diarrhea, which is the most common adverse event) remains essential.

Q: Is the FVC "improvement" seen with ENV-101 (Hedgehog inhibitor) truly evidence of fibrosis reversal? A: A Phase 2a 12-week readout of FVC %pred +1.9% (vs −1.3% placebo) and TLC +206.67 mL (vs −55.58 mL) on AI-based HRCT (PMID 41043447) shows a signal beyond simple progression suppression. However, the trial enrolled only n=41 (taladegib 21 + placebo 20), and is an exploratory Phase 2a. Calling this "fibrosis reversal" requires replication in Phase 2b WHISTLE-PF (NCT06422884). The current data are best characterized as hypothesis-generating.

Q: Inhaled Treprostinil (Tyvaso) is a PAH drug — what role does it play in IPF? A: TETON-2 (n=593, PMID 41812190) showed a between-group difference of +95.6 mL (95% CI 52.2 to 139.0; P<0.001) across a patient population in which 75.4% were on background antifibrotic therapy. A subgroup analysis suggested a more pronounced treatment effect in patients on background therapy, consistent with additive activity via a different (cAMP) mechanism. Calling this a "synergistic" pharmacological interaction would overstate the evidence; "additive effect suggested by subgroup analysis" is the precise framing. FDA sNDA filing is expected in Q3 2026.

Q: Why did Pamrevlumab (anti-CTGF) and Bexotegrast (αvβ6 integrin inhibitor) fail in Phase 3? A: For Pamrevlumab, proposed design-related explanations include regression-to-the-mean inflating the Phase 2 effect and difficulty detecting add-on benefit on top of SoC. For Bexotegrast, one mechanistic hypothesis is that strong integrin blockade may have compromised pulmonary immune defense, contributing to the observed safety signal — but the definitive mechanism for the safety signal has not been established. Both cases underscore the importance of translational modeling for biologically complex targets.

Q: Which IPF drug targets should the field watch most closely today? A: Three tiers: (1) PDE4B (approved, expanding), (2) LPA1 (BMS-986278 in Phase 3), and (3) Hedgehog / AT2R / TNIK (late Phase 2 candidates suggesting disease modification). In particular, the AI-discovered TNIK inhibitor ISM001-055 — converging TGF-β, Wnt, and YAP/TAZ at a single upstream node — is a target to follow.

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References

Clinical Trial Registrations (ClinicalTrials.gov)

• FIBRONEER-IPF: NCT05321069
• FIBRONEER-ILD (PPF): NCT05321082
• TETON-2 (Treprostinil): NCT05255991
• TETON-1 (Treprostinil, US/Canada): NCT04708782
• ALOFT-IPF (BMS-986278): NCT06003426
• ENV-101 Phase 2a: NCT04968574
• WHISTLE-PF (ENV-101 Phase 2b): NCT06422884
• ASPIRE (Buloxibutid): NCT06588686
• Rentosertib Phase 2a (China): NCT05938920

Key Publications (PubMed)

• King TE Jr, et al. A Phase 3 Trial of Pirfenidone in Patients with Idiopathic Pulmonary Fibrosis. N Engl J Med. 2014;370(22):2083-92. PMID: 24836312
• Richeldi L, et al. Efficacy and Safety of Nintedanib in Idiopathic Pulmonary Fibrosis. N Engl J Med. 2014;370(22):2071-82. PMID: 24836313
• Richeldi L, Azuma A, Cottin V, et al. Nerandomilast in Patients with Idiopathic Pulmonary Fibrosis (FIBRONEER-IPF). N Engl J Med. 2025;392(22):2193-2202. PMID: 40387033 / NCT05321069
• FDA. Jascayd (nerandomilast) Prescribing Information. Boehringer Ingelheim Pharmaceuticals; revised December 2025. DailyMed current label — Indications: adult IPF and adult PPF (both as standalone monotherapy).
• Nathan SD, Smith P, Deng C, et al. Inhaled Treprostinil for Idiopathic Pulmonary Fibrosis (TETON-2). N Engl J Med. 2026. PMID: 41812190 / NCT05255991
• Corte TJ, Behr J, Cottin V, et al. Efficacy and Safety of Admilparant, an LPA1 Antagonist, in Pulmonary Fibrosis: A Phase 2 Randomized Clinical Trial. Am J Respir Crit Care Med. 2025;211(2):230-238. PMID: 39393084 / NCT04308681
• Maher TM, Goldin JG, Hood J, et al. Taladegib for the treatment of idiopathic pulmonary fibrosis (ENV-IPF-101): a multicentre, randomised, double-blind, placebo-controlled, phase 2a trial. Lancet Respir Med. 2025;13(11):1001-1010. PMID: 41043447 — n=41 (exploratory Phase 2a).
• Xu Z, Ren F, et al. A generative AI-discovered TNIK inhibitor for idiopathic pulmonary fibrosis: a randomized phase 2a trial. Nat Med. 2025;31(8):2602-2610. PMID: 40461817 / NCT05938920 — n=71, primary endpoint was safety; FVC was exploratory.
• Nerandomilast SSc-ILD preclinical study. Clin Rheumatol. 2024. PMID: 39438343
• Nerandomilast fibrotic rats study. Br J Pharmacol. 2024. PMID: 39183442
• Treprostinil bleomycin mouse study. Pulm Circ. 2019;9(4). PMID: 31819797
• Corboz MR, et al. Therapeutic administration of inhaled INS1009 inhibits bleomycin-induced pulmonary fibrosis in rats. Pulm Pharmacol Ther. 2018;49:95-103. PMID: 29408757
• BMS-986278 discovery and preclinical study. J Med Chem. 2021;64(21):15883-15911. PMID: 34726410