Pulmonary hypertension rapidly leading to mortality in a patient with interstitial pneumonia with autoimmune features

Abstract

Interstitial Pneumonia with Autoimmune Features (IPAF) is a newly defined connective tissue disease (CTD) related pulmonary involvement in 2015 by the European Respiratory Society and American Thoracic Society. It is used to define the subgroup of patients with no known CTD history. In this case report, we present a patient with IPAF diagnosis, who had rapid clinical deterioration after a COVID-19 infection, which was attributed to a newly diagnosed pulmonary hypertension. A 69-year-old male patient with chronic obstructive pulmonary disease (COPD) diagnosis was on the routine follow-up for a duration of five years. He had been diagnosed with type 2 diabetes mellitus and gout, with under constant follow-up for a known cardiovascular arterial disease history. During a routine pulmonary evaluation, the patient had stated a worsening exercise capacity and an increase in coughing. The requested high-resolution computed tomography showed emphysema at the upper lobes and a reticular pattern at the lower lobes, with honeycombing, traction bronchiectasis, and ground-glass findings. After a rheumatology consultation, which excluded the presence of an underlying rheumatological disease, the patient was diagnosed with IPAF. Under immunosuppressive treatment, the patient complained of increased dyspnea at a routine follow-up after a COVID infection in 2021, and antifibrotic treatment was initiated due to the progression of pulmonary fibrosis. During routine follow-up, further limitation in exercise capacity was evident, for which extra-pulmonary involvement was investigated. Pulmonary hypertension (PH) diagnosis was confirmed with vasoreactivity positivity; however, due to both progression in IPAF and concurrent PH, the patient was lost during the follow-up. As seen in this case, despite being stable for years, the addition of another comorbidity may rapidly worsen a patient’s otherwise stable clinical condition. While antifibrotic regimens may be used on a case-by-case basis, their effect on disease progression may not be sufficient to control an already present comorbidity, such as pulmonary hypertension.