Case Report: Inhaled treprostinil: an alternative for the treatment of refractory exacerbation of interstitial lung disease

Acute exacerbation of interstitial lung disease is associated with poor prognosis. Evidence on the best therapeutic strategy for affected patients is lacking. Corticosteroids, broad-spectrum antibiotics, and oxygen therapy are the mainstay of treatment, although immunosuppressants can be added in refractory exacerbations. We present the case of an acute exacerbation in a patient with unclassifiable interstitial lung disease and a history of lymphoma in complete remission. Despite treatment with high-dose corticosteroids, antibiotics, rituximab, and cyclophosphamide, significant hypoxemia persisted, preventing discharge. Echocardiography indicated an intermediate probability of pulmonary hypertension. Subsequent right heart catheterization confirmed precapillary pulmonary hypertension. Inhaled treprostinil was started once other possible causes of pulmonary hypertension had been ruled out. The patient’s condition gradually improved. Treprostinil has been shown to be effective in the treatment of pulmonary hypertension associated with interstitial lung disease. Furthermore, its potential antifibrotic effect is currently being investigated. In the present case, we believe that treating both conditions led to clinical and radiological improvement. However, the limitations of a case report require more robust studies to be performed before firm clinical recommendations can be made.