An autopsy case of desquamative interstitial pneumonia comorbid with severe pulmonary hypertension
Hiroki Takechia,b Hiroshi Kuraishia Yuki Todorokia Ryota Ozawaa Manabu Yamamotoa Ichiro Itoc
aDepartment of Pulmonary Medicine, Nagano Red Cross Hospital
bDepartment of Pulmonary Medicine, Saku Central Hospital Advanced Care Center
cDepartment of Pathology, Nagano Red Cross Hospital
After developing a chronic cough, a 46-year-old male was diagnosed with desquamative interstitial pneumonia (DIP) by thoracoscopic lung biopsy 16 years ago. Despite smoking cessation and systemic steroid therapy, symptoms of right heart failure began to appear three years ago. He was referred to our hospital because of his worsening respiratory condition. Right heart catheterization revealed severe pulmonary hypertension, and he died irrespective of treatment with pulmonary vasodilators and diuretics.
In some DIP patients, fibrosis progresses even after smoking cessation and steroid therapy, leading to severe respiratory failure and pulmonary hypertension. In patients with progressive fibrosis, it is especially important to evaluate pulmonary hypertension, and lung transplantation should be considered in severe cases.
Desquamative interstitial pneumonia (DIP) Pulmonary hypertension
Received 8 Sep 2023 / Accepted 1 Nov 2023
AJRS, 13(2): 59-63, 2024