ArticleRespirology case reports2024
Effectiveness of upfront triple oral combination therapy with additional pirfenidone in a patient with severe pulmonary hypertension associated with lung diseases.
Article in Respirology case reports, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.
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Who cites it
2 citing papers in PubMed.
- Emerging biomaterials and bio-nano interfaces in pulmonary hypertension therapy: transformative strategies for personalized treatment.Frontiers in bioengineering and biotechnology · 2025Review
- Effectiveness of upfront triple oral combination therapy with additional pirfenidone in a patient with severe pulmonary hypertension associated with lung diseases.Respirology case reports · 2024Article
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12 authors.
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Abstract
Diagnosis and treatment of pulmonary hypertension (PH) in patients with lung diseases (PH-LD) remain unestablished and pose significant challenges. In this report, we present a case of a 77-year-old patient with an indeterminate for usual interstitial pneumonia pattern along with chronic obstructive pulmonary disease, who developed groups 1 and 3 PH. Following diagnosis, upfront triple oral combination therapy (UTOCT) with macitentan, sildenafil, and selexipag was initiated. Stability in disease progression was achieved over 4 years with the addition of pirfenidone to address interstitial lung disease progression. To the best of our knowledge, this represents the first reported case of PH-LD, where disease control was maintained with the addition of pirfenidone to UTOCT. This case suggests that some patients with PH-LD, presenting with groups 1 and 3 PH, may benefit from combined UTOCT and antifibrotic agents, potentially improving symptoms and extending their prognosis.
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