Respirology Case Reports (Aug 2024)

Effectiveness of upfront triple oral combination therapy with additional pirfenidone in a patient with severe pulmonary hypertension associated with lung diseases

  • Fumihiro Kashizaki,
  • Sachiko Matsumoto,
  • Atsushi Miyasaka,
  • Nanami Tsuchiya,
  • Reeko Osada,
  • Mai Kaneko,
  • Kentaro Yumoto,
  • Hao Chen,
  • Kenji Konishi,
  • Harumi Koizumi,
  • Kenichi Takahashi,
  • Takeshi Kaneko

DOI
https://doi.org/10.1002/rcr2.70010
Journal volume & issue
Vol. 12, no. 8
pp. n/a – n/a

Abstract

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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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