Respiratory Today—May 22, 2026

This week’s Respiratory Research update highlights regulatory progress, late-stage clinical data, modality expansion, and biomarker-driven respiratory pipeline updates.

In Today’s Newsletter

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💊 JASCAYD approval in Japan [1] [Japan • 18 May 2026]

https://www.boehringer-ingelheim.com/human-health/lung-diseases/pulmonary-fibrosis/jascayd-approved-japan-new-ipf-and-ppf-treatment
Context: Japan’s MHLW approved JASCAYD, supported by Phase 3 FIBRONEER-IPF and FIBRONEER-ILD data showing slowed FVC decline.
Key point: Boehringer Ingelheim’s JASCAYD (nerandomilast) is an oral PDE4B inhibitor for adults with IPF and PPF.
Implication: May influence prescriber choice and payer reviews pending full data.

🫁 ALX1 Phase 1 inhaled nitric oxide prodrug data [2] [US • 18 May 2026]

https://www.vasttherapeutics.com/vast-therapeutics-presents-late-breaking-phase-1-data-for-inhaled-therapy-alx1-at-ats-2026/
Context: ALX1 was well tolerated across evaluated dose levels, with no dose-limiting toxicities and dose-proportional pharmacokinetics.
Key point: Vast Therapeutics evaluated ALX1, an inhaled nitric oxide prodrug, in 48 healthy volunteers.
Implication: May influence prescriber choice and payer reviews pending full data.

🧬 Efdoralprin alfa Phase 2 AATD data [3] [US • 18 May 2026]

https://www.sanofi.com/en/media-room/press-releases/2026/2026-05-18-19-30-00-3296985
Context: Efdoralprin alfa met the primary endpoint, achieving higher and sustained functional AAT trough levels versus standard of care.
Key point: Sanofi’s ElevAATe Phase 2 study compared efdoralprin alfa with plasma-derived augmentation therapy in AATD-related emphysema.
Implication: May influence prescriber choice and payer reviews pending full data.

🌬️ Tyvaso TETON-1 IPF data in NEJM [4] [US, Canada • 18 May 2026]

http://ir.unither.com/press-releases/2026/05-18-2026-211528317
Context: TETON-1 met its primary endpoint on absolute FVC at week 52 and reduced clinical worsening risk versus placebo.
Key point: United Therapeutics reported TETON-1 Phase 3 data for nebulized Tyvaso (treprostinil) in IPF.
Implication: May influence prescriber choice and payer reviews pending full data.

🧪 LAM-001 Phase 2a PH-ILD update [5] [US • 18 May 2026]

https://ir.quincetx.com/news-releases/news-release-details/quince-therapeutics-announces-clinically-meaningful-improvements
Context: Phase 2a data showed directional improvements across exercise capacity, hemodynamics, biomarkers, and FVC in a small open-label study.
Key point: Quince Therapeutics evaluated LAM-001, inhaled rapamycin, as add-on therapy in PAH and PH-ILD.
Implication: Could inform practice and payer discussions; interpretation depends on study design and confounding control.

🌫️ AERO-007 Phase 2a COPD data [6] [US • 18 May 2026]

https://www.biospace.com/press-releases/aerorx-therapeutics-presents-positive-phase-2a-data-for-inhaled-aero-007-the-first-potential-nebulized-laba-lama-fixed-dose-combination-in-development-for-copd
Context: AERO-007 produced rapid and sustained bronchodilation through 24 hours and was well tolerated in Phase 2a.
Key point: AeroRx tested AERO-007, a nebulized LABA plus LAMA fixed-dose combination, in moderate-to-severe COPD.
Implication: Could streamline initiation and adherence via remote prescribing and logistics.

✂️ BEAM-302 AATD base-editing update [7] [US • 18 May 2026]

https://investors.beamtx.com/news-releases/news-release-details/beam-therapeutics-presents-recently-reported-topline-clinical
Context: The update added safety, durability, and neutrophil elastase activity data from previously reported topline results.
Key point: Beam Therapeutics presented additional Phase 1/2 single-dose cohort data for BEAM-302 in AATD.
Implication: Signals pipeline investment and modality expansion.

🔁 WVE-006 RestorAATion-2 AATD update [8] [US • 18 May 2026]

https://ir.wavelifesciences.com/news-releases/news-release-details/wave-life-sciences-announces-positive-update-restoraation-2
Context: WVE-006 generated wild-type M-AAT, reduced Z-AAT, and showed sustained editing after dosing in reported cohorts.
Key point: Wave Life Sciences reported RestorAATion-2 Phase 1b/2a data for WVE-006, a GalNAc-RNA editing therapy for PiZZ AATD.
Implication: Signals pipeline investment and modality expansion.

🧬 KRRO-111 AATD development candidate [9] [US • 19 May 2026]

https://ir.korrobio.com/news-releases/news-release-details/korro-selects-krro-111-development-candidate-potential-treatment
Context: Preclinical data showed high SERPINA1 transcript editing and reduced Z-AAT production in a mouse model.
Key point: Korro Bio selected KRRO-111, a GalNAc-conjugated RNA-editing oligonucleotide, for AATD.
Implication: Signals pipeline investment and modality expansion.

🔬 Molgramostim IMPALA-2 biomarker data [10] [US • 20 May 2026]

https://investors.savarapharma.com/news/news-details/2026/Savara-Presented-New-Biomarker-Data-from-the-IMPALA-2-Phase-3-Clinical-Trial-of-Molgramostim-Inhalation-Solution-Molgramostim-in-Autoimmune-Pulmonary-Alveolar-Proteinosis-aPAP-at-the-American-Thoracic-Society-ATS-International-Conference-2026/default.aspx
Context: Molgramostim improved DLco% versus placebo and reduced disease-associated biomarkers in post-hoc analyses.
Key point: Savara presented IMPALA-2 Phase 3 biomarker data for molgramostim in autoimmune pulmonary alveolar proteinosis.
Implication: May influence prescriber choice and payer reviews pending full data.

🧫 BBT002 Phase 1 biomarker update [11] [US • 18 May 2026]

https://bambusatx.com/bambusa-therapeutics-presents-positive-preliminary-phase-1-bbt002-data-at-american-thoracic-society-2026-ats-2026-international-conference-and-announces-first-patient-dosed-in-phase-1b-2a-crswnp-tri/
Context: BBT002 showed sustained biomarker effects after a single dose and the first patient was dosed in a Phase 1b/2a CRSwNP trial.
Key point: Bambusa Therapeutics reported preliminary Phase 1 data for BBT002, a bispecific antibody targeting IL-4Rα and IL-5.
Implication: Signals pipeline investment and modality expansion.

🫧 ORJ-001 IPF clinical and preclinical data [12] [US • 19 May 2026]

https://oorjabio.com/wp-content/uploads/2026/05/Oorja-Bio-Unveils-Clinical-Data.pdf
Context: ORJ-001 achieved therapeutically relevant exposure, was well tolerated, and supported plans for a Phase 2 IPF trial.
Key point: Oorja Bio presented Phase 1 healthy-volunteer and preclinical data for ORJ-001, an AEC2-targeting peptide for IPF.
Implication: May influence prescriber choice and payer reviews pending full data.

Why it matters

IPF and fibrotic lung disease saw both regulatory and Phase 3 momentum, with JASCAYD in Japan and Tyvaso IPF data.
AATD remains highly active across modalities, including recombinant protein, DNA base editing, RNA editing, and RNA-repair oligonucleotides.
Inhaled and nebulized delivery featured prominently across COPD, PH-ILD, aPAP, and nitric oxide-based respiratory programs.
Several updates came from small, open-label, healthy-volunteer, preclinical, or post-hoc datasets, so study design remains central to interpretation.

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FAQ

What changed for JASCAYD (nerandomilast) in Japan?

Boehringer Ingelheim said Japan’s MHLW approved JASCAYD for adults with IPF and PPF. The approval was supported by FIBRONEER-IPF and FIBRONEER-ILD Phase 3 data. [1]

Is nebulized Tyvaso approved for IPF?

United Therapeutics said Tyvaso remains investigational for IPF. The company reported positive TETON-1 Phase 3 data and plans an FDA submission to add IPF to the label. [4]

How did Sanofi’s efdoralprin alfa perform in AATD?

In ElevAATe Phase 2, efdoralprin alfa achieved higher functional AAT trough levels versus standard plasma-derived therapy. The source says the primary endpoint was met. [3]

How are Wave’s WVE-006 and Korro’s KRRO-111 different from Beam’s BEAM-302?

WVE-006 and KRRO-111 are RNA-editing approaches, while BEAM-302 is a DNA base-editing therapy. All target AATD biology, but the provided sources report different stages and datasets. [7–9]

What did Savara report for molgramostim in aPAP?

Savara presented biomarker data from IMPALA-2, including improved DLco% and reductions in disease-associated biomarkers versus placebo. Some analyses were post-hoc. [10]

Which programs are moving toward later clinical testing?

Quince plans Phase 2b testing for LAM-001 in PH-ILD, Oorja plans Phase 2 testing for ORJ-001 in IPF, and Bambusa has begun Phase 1b/2a testing for BBT002 in CRSwNP. [5,11,12]

Entities / Keywords

Boehringer Ingelheim: JASCAYD, nerandomilast, PDE4B inhibitor, IPF, PPF, FIBRONEER-IPF, FIBRONEER-ILD
Vast Therapeutics: ALX1, nitric oxide prodrug, inhaled therapy, bronchiectasis, COPD
Sanofi: Efdoralprin alfa, ElevAATe, AATD, fAAT, plasma-derived augmentation therapy
United Therapeutics: Tyvaso, treprostinil, TETON-1, TETON-2, IPF, FVC
Quince Therapeutics: LAM-001, inhaled rapamycin, mTOR inhibitor, PAH, PH-ILD
AeroRx Therapeutics: AERO-007, LABA, LAMA, COPD, nebulized fixed-dose combination
Beam Therapeutics: BEAM-302, base editing, PiZ mutation, AATD
Wave Life Sciences: WVE-006, RestorAATion-2, GalNAc-RNA editing, AIMer, PiZZ AATD
Korro Bio: KRRO-111, OPERA, RNA editing, SERPINA1, ADAR, AATD
Savara: Molgramostim, IMPALA-2, aPAP, DLco%, GM-CSF
Bambusa Therapeutics: BBT002, IL-4Rα, IL-5, COPD, CRSwNP, Type 2 inflammation
Oorja Bio: ORJ-001, AEC2 cells, β1 integrin, IPF, Tenascin-C, SP-D