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Yogeswaran, A; Hassoun, PM; Saleh, K; Fünderich, M; Balasubramanian, A; Konswa, Z; Kiely, DG; Lawrie, A; Thenappan, T; Eichstaedt, CA; Grünig, E; Wilkins, MR; Howard, L; Olschewski, H; Kovacs, G; Cajigas, HR; Frantz, R; Sabbour, H; Sweatt, AJ; Zamanian, RT; Arvanitaki, A; Giannakoulas, G; Elwing, J; Jose, A; Beckmann, S; Olsson, KM; Stadler, S; Held, M; Halank, M; Ewert, R; Behr, J; Milger-Kneidinger, K; Pausch, C; Pittrow, D; Majeed, RW; Wilhelm, J; Ghofrani, HA; Grimminger, F; Tello, K; Hoeper, MM; Seeger, W, , PVRI-GoDeep-Consortium.
Hemodynamics and Phosphodiesterase-5 Inhibitor Treatment Associated with Survival in ILD-PH: A PVRI GoDeep Meta-Registry Analysis.
Am J Respir Crit Care Med. 2025; Doi: 10.1164/rccm.202412-2371OC
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Co-Autor*innen der Med Uni Graz
Kovacs Gabor
Milger-Kneidinger Katrin
Olschewski Horst
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Abstract:
BACKGROUND: Pulmonary hypertension (PH) in interstitial lung disease (ILD) lacks approved therapies. The PVRI GoDeep meta-registry collects real-world data of PH patients from international PH referral centers. METHODS: ILD-PH patients and relevant subgroups (IIP, IPF) were stratified by pulmonary vascular resistance (PVR). Kaplan-Meier survival analyses, adjusted Cox proportional hazards models, additionally accounting for immortal time bias, sensitivity analyses, Heller explained relative risk statistics and target trial emulation framework analysis were employed. RESULTS: Among 34,482 patients, 940 hemodynamically fully characterized incident ILD-PH patients (median age 67[59,74] years) were identified. 62% had severe ILD-PH with PVR >5 WU and poor survival rates (29% and 18% at 3 and 5 years), significantly worse than ILD-PH patients with PVR ≤5 WU and PAH patients. Survival was poorest in severe IPF-PH. 59% of all ILD-PH patients received PH-targeted therapy, predominantly phosphodiesterase-5 inhibitors (PDE5i). PDE5i-treatment was consistently associated with significantly improved survival in patients with severe PH (hazard ratios 0.537[0.370,0.781], 0.461[0.233,0.913] and 0.435[0.215,0.8] for IIP-PH, IPF-PH and IIP-PH on nintedanib/pirfenidone background therapy), but not in patients with less severe hemodynamic impairment, supported by sensitivity analyses, Heller statistics and target trial emulation framework analysis. The survival statistics of PDE5i-treated IIP-PH/IPF-PH patients were validated in the independent COMPERA registry. Combination therapy with PDE5i and inhaled prostacyclin analogue was superior to monotherapy using PDE5i (HR 0.341 [0.205, 0.566]). CONCLUSION: Prognosis in ILD-PH was generally very poor, related to PH severity. PDE5i treatment in severe IIP-PH and IPF-PH was associated with improved survival, to be further verified in controlled trials.

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