Companies in #Pulmonary Hypertension with Interstitial Lung Disease

Liquidia is a biopharmaceutical company that develops, manufactures and commercializes products for rare cardiopulmonary diseases, built around its proprietary PRINT particle-engineering technology for uniform drug particles with controlled size and shape. Its lead product, Yutrepia, is an inhaled dry-powder formulation of treprostinil approved for pulmonary arterial hypertension and for pulmonary hypertension associated with interstitial lung disease, and the company promotes Remodulin, a treprostinil injection, under an agreement with Sandoz. A third program, L606, is an investigational liposomal, sustained-release inhaled treprostinil licensed from Pharmosa Biopharm in Phase 3 development for PAH and PH-ILD. Founded in 2004 and headquartered in Morrisville, North Carolina, Liquidia listed on Nasdaq in July 2018 and turned sharply profitable in 2026 on Yutrepia's commercial ramp — second-quarter 2026 revenue reached roughly $171.7 million with net income of about $74.7 million. The equity carries a concentrated legal tail: on September 30, 2026 a Delaware federal court held two claims of a United Therapeutics patent valid and infringed by Yutrepia in the PH-ILD indication, while invalidating the other four asserted claims; the company has said it will appeal and may remove the PH-ILD indication from the label, and sought remedies threaten product availability.

MannKind is a biopharmaceutical company focused on inhaled therapeutic products for endocrine and orphan lung diseases, built on its proprietary Technosphere dry-powder inhalation platform. Its commercial portfolio includes Afrezza, the only FDA-approved inhaled mealtime insulin, and the V-Go wearable insulin delivery device; the company also manufactures Tyvaso DPI, an inhaled treprostinil powder commercialized by United Therapeutics, from which MannKind earns royalties and manufacturing revenue, and it has expanded into heart-failure fluid management with FUROSCIX and its ReadyFlow autoinjector. The pipeline extends the same platform into interstitial lung disease with MNKD-201 (inhaled nintedanib), which reported positive Phase 1b data in 2026, and into metabolic disease through a September 2026 licensing and collaboration agreement with Rose Pharma for ROSE-010, an inhaled rapid-acting GLP-1 for weight management. Founded in February 1991 by Alfred E. Mann and headquartered in Danbury, Connecticut, the company reported second-quarter 2026 revenue of $109.4 million, up 43% year over year, at a gross margin above 79%, while still recording a small operating loss and negative free cash flow. A Phase 3 program in nontuberculous mycobacterial lung disease with nebulized clofazimine (MNKD-101) was discontinued for futility in November 2025, with the NTM effort redirected to an earlier dry-powder formulation.