Kashizaki Fumihiro, Matsumoto Sachiko, Miyasaka Atsushi, Tsuchiya Nanami, Osada Reeko, Kaneko Mai, Yumoto Kentaro, Chen Hao, Konishi Kenji, Koizumi Harumi, Takahashi Kenichi, Kaneko Takeshi
Department of Respiratory Medicine Yokohama Minami Kyosai Hospital Yokohama Japan.
Department of Respiratory Medicine Seirei Yokohama Hospital Yokohama Japan.
Respirol Case Rep. 2024 Aug 26;12(8):e70010. doi: 10.1002/rcr2.70010. eCollection 2024 Aug.
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.
肺部疾病合并肺动脉高压（PH-LD）患者的诊断和治疗仍未明确，且面临重大挑战。在本报告中，我们呈现了一例77岁患者，其具有不典型的普通间质性肺炎模式并伴有慢性阻塞性肺疾病，该患者同时患有1型和3型肺动脉高压。确诊后，开始使用马昔腾坦、西地那非和司来帕格进行初始三联口服联合治疗（UTOCT）。通过加用吡非尼酮来应对间质性肺病进展，在4年时间里实现了疾病进展的稳定。据我们所知，这是首例报告的PH-LD病例，通过在UTOCT基础上加用吡非尼酮维持了疾病控制。该病例表明，一些同时患有1型和3型肺动脉高压的PH-LD患者可能从UTOCT与抗纤维化药物的联合治疗中获益，这有可能改善症状并延长预后。
