The American journal of the medical sciences · 2026
Management of Pulmonary Hypertension in Interstitial Lung Disease Without Access to Inhaled Treprostinil: A Case Series from a Resource-Limited Setting.
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Kabalak Pınar Akın, Söyler Yasemin, Cengiz Tuba İnal, Çelik Murat, Yılmaz Ülkü
Аннотация
BACKGROUND: Pulmonary hypertension (PH) may develop as a clinically important complication of parenchymal lung diseases, particularly in the setting of chronic hypoxemia and advanced interstitial lung disease. The management of interstitial lung disease-associated pulmonary hypertension (ILD-PH) remains challenging and requires a multidimensional assessment, including radiological findings, pulmonary function tests, comorbidities, and hemodynamic parameters.
OBJECTIVE: Although inhaled treprostinil is an approved treatment for ILD-PH, access to this therapy remains limited. We aimed to describe alternative management strategies and clinical outcomes in patients with ILD-PH in a resource-limited setting.
METHODS: This descriptive retrospective case series included four patients with different types of ILD: fibrotic hypersensitivity pneumonitis, combined pulmonary fibrosis and emphysema (CPFE), rheumatoid arthritis-associated ILD, and idiopathic pulmonary fibrosis (IPF). Demographic characteristics, hemodynamic data, treatment approaches, and clinical responses were evaluated.
RESULTS: Inhaled iloprost was initiated in patients with IPF and fibrotic hypersensitivity pneumonitis. Both patients tolerated the treatment well and remained functionally stable. However, the patient with CPFE was unable to tolerate inhaled prostanoid therapy and had a fatal outcome despite treatment with a phosphodiesterase-5 inhibitor. The patient with rheumatoid arthritis-associated ILD had severe fibrotic parenchymal involvement and demonstrated clinical stability during a 6-month follow-up period under phosphodiesterase-5 inhibitor therapy.
CONCLUSION: Patients with ILD-PH should be evaluated individually according to underlying lung pathology, disease phenotype, and treatment tolerability. Where inhaled treprostinil is unavailable, alternative therapies, such as inhaled iloprost or phosphodiesterase-5 inhibitors, may provide clinical benefits in selected patients. However, patients with CPFE may have poorer outcomes despite PH-targeted therapies.
OBJECTIVE: Although inhaled treprostinil is an approved treatment for ILD-PH, access to this therapy remains limited. We aimed to describe alternative management strategies and clinical outcomes in patients with ILD-PH in a resource-limited setting.
METHODS: This descriptive retrospective case series included four patients with different types of ILD: fibrotic hypersensitivity pneumonitis, combined pulmonary fibrosis and emphysema (CPFE), rheumatoid arthritis-associated ILD, and idiopathic pulmonary fibrosis (IPF). Demographic characteristics, hemodynamic data, treatment approaches, and clinical responses were evaluated.
RESULTS: Inhaled iloprost was initiated in patients with IPF and fibrotic hypersensitivity pneumonitis. Both patients tolerated the treatment well and remained functionally stable. However, the patient with CPFE was unable to tolerate inhaled prostanoid therapy and had a fatal outcome despite treatment with a phosphodiesterase-5 inhibitor. The patient with rheumatoid arthritis-associated ILD had severe fibrotic parenchymal involvement and demonstrated clinical stability during a 6-month follow-up period under phosphodiesterase-5 inhibitor therapy.
CONCLUSION: Patients with ILD-PH should be evaluated individually according to underlying lung pathology, disease phenotype, and treatment tolerability. Where inhaled treprostinil is unavailable, alternative therapies, such as inhaled iloprost or phosphodiesterase-5 inhibitors, may provide clinical benefits in selected patients. However, patients with CPFE may have poorer outcomes despite PH-targeted therapies.