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KMID : 0338420210360000132
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The Korean Journal of Internal Medicine
2021 Volume.36 No. 0 p.132 ~ p.141
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Pleuroparenchymal fibroelastosis in Korean patients: clinico-radiologic-pathologic features and 2-year follow-up
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Lee Jae-Ha
Chae Eun-Jin
Song Joon-Seon
Kim Mi-Ae
Song Jin-Woo
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Abstract
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Background/Aims: Pleuroparenchymal fibroelastosis (PPFE) is a type of rare idiopathic interstitial pneumonia that is characterized by predominantly upper lobe involvement with pleural fibrosis and subjacent parenchymal fibrosis. This study aimed to determine the clinico-radiologic-pathologic features and prognosis of Korean patients with PPFE.
Methods: A total of 26 patients who were confirmed to have PPFE by lung biopsy, were included, and their clinico-radiologic-pathologic findings were retrospectively analyzed.
Results: The mean follow-up period was 23.8 months, and the mean age of the patients was 62.5 years; 61.5% were men and 50% were smokers. Cough and dyspnea were the most frequent presenting symptoms, and restrictive pattern was the most common observation in lung function. In 84.6% of the subjects, lower lobe involvement was found on chest computed tomography, and the usual interstitial pneumonia (UIP) pattern was the most common (59.1%). Among patients whose lower lobe was biopsied (n = 13), the UIP pattern was the most common (46.2%). Patients with lower lobe involvement were older and walked a shorter distance during the 6-minute walk test, compared to those without. Spontaneous pneumothorax was a common complication (26.9%), and 15.4% of the patients died mostly due to pneumonia (100%). The 1- and 3-year survival rates were 90.2% and 84.5%, respectively.
Conclusions: Clinical features of Korean patients with PPFE were similar to those reported previously; however, lower lobe involvement was more frequent. During follow-up, one-fourth of the patients experienced pneumothorax and one-fifth died from pneumonia.
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KEYWORD
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Idiopathic interstitial pneumonias, Pulmonary fibrosis, Pleural diseases, Survival analysis
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