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Extent of the Honeycombing Area and the Presence of Traction Bronchiectasis Are Risk Factors for Poorer Prognosis in Patients with Pleuroparenchymal Fibroelastosis
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A1621 - Extent of the Honeycombing Area and the Presence of Traction Bronchiectasis Are Risk Factors for Poorer Prognosis in Patients with Pleuroparenchymal Fibroelastosis
Author Block: M. Namba, T. Masuda, K. Yamaguchi, S. Sakamoto, Y. Horimasu, S. Miyamoto, T. Nakashima, H. Iwamoto, S. Ohshimo, K. Fujitaka, H. Hamada, N. Hattori; Hiroshima University Hospital, Hiroshima, Japan.
Introduction: Pleuroparenchymal fibroelastosis (PPFE) is classified as a rare idiopathic interstitial pneumonia characterized by markedly thickened visceral pleura and prominent subpleural fibrosis predominantly in the upper lobes. In some cases of PPFE, interstitial fibrosis is accompanied with pleuroparenchymal thickening. To date, however, there has been no previous study to examine whether the extent of high-resolution computed tomography (HRCT) findings including interstitial fibrosis is a risk factor for poorer prognosis of PPFE. We therefore retrospectively investigated whether the extent of HRCT findings could identify risk factors for poorer prognosis. Materials and Methods: Between January 2008 and December 2016, patients who were diagnosed with PPFE on HRCT at Hiroshima University Hospital were enrolled. The extent of honeycombing, reticulation, consolidation, and ground-glass attenuation (GGA), the presence of cystic changes and traction bronchiectasis, and the diameter of the pleural thickening were evaluated in six areas in bilateral lungs. The HRCT scores were determined by the following formula: 100 × sum of the extent of the HRCT findings/lung area using a visual score method. Results: Fifteen patients were enrolled in this study, and 9 patients died. All 3 enrolled women survived during the observation period. On the contrary, 9 out of 12 enrolled men (75%) deceased during the observation period. The body mass index in non-survivors was significantly lower when compared to survivors. There were no other significant differences in the patient’s characteristics including age, smoking history, and KL-6, between the two groups. The median HRCT scores of honeycombing and number of areas with traction bronchiectasis were significantly higher in non-survivors when compared to survivors (p = 0.006 and p = 0.014, respectively). On Cox regression analysis, a honeycombing area score > 0.83% and the number of traction bronchiectasis areas ≥ 5 were significant risk factors for poorer prognosis (hazard ratio [95% confidence interval]: 9.711 [1.708-182.294], p = 0.008; 4.642 [1.061-31.818], p = 0.041, respectively). Conclusions: The honeycomb area and presence of traction bronchiectasis on HRCT may predict poorer prognosis in patients with PPFE.
Author Block: M. Namba, T. Masuda, K. Yamaguchi, S. Sakamoto, Y. Horimasu, S. Miyamoto, T. Nakashima, H. Iwamoto, S. Ohshimo, K. Fujitaka, H. Hamada, N. Hattori; Hiroshima University Hospital, Hiroshima, Japan.
Introduction: Pleuroparenchymal fibroelastosis (PPFE) is classified as a rare idiopathic interstitial pneumonia characterized by markedly thickened visceral pleura and prominent subpleural fibrosis predominantly in the upper lobes. In some cases of PPFE, interstitial fibrosis is accompanied with pleuroparenchymal thickening. To date, however, there has been no previous study to examine whether the extent of high-resolution computed tomography (HRCT) findings including interstitial fibrosis is a risk factor for poorer prognosis of PPFE. We therefore retrospectively investigated whether the extent of HRCT findings could identify risk factors for poorer prognosis. Materials and Methods: Between January 2008 and December 2016, patients who were diagnosed with PPFE on HRCT at Hiroshima University Hospital were enrolled. The extent of honeycombing, reticulation, consolidation, and ground-glass attenuation (GGA), the presence of cystic changes and traction bronchiectasis, and the diameter of the pleural thickening were evaluated in six areas in bilateral lungs. The HRCT scores were determined by the following formula: 100 × sum of the extent of the HRCT findings/lung area using a visual score method. Results: Fifteen patients were enrolled in this study, and 9 patients died. All 3 enrolled women survived during the observation period. On the contrary, 9 out of 12 enrolled men (75%) deceased during the observation period. The body mass index in non-survivors was significantly lower when compared to survivors. There were no other significant differences in the patient’s characteristics including age, smoking history, and KL-6, between the two groups. The median HRCT scores of honeycombing and number of areas with traction bronchiectasis were significantly higher in non-survivors when compared to survivors (p = 0.006 and p = 0.014, respectively). On Cox regression analysis, a honeycombing area score > 0.83% and the number of traction bronchiectasis areas ≥ 5 were significant risk factors for poorer prognosis (hazard ratio [95% confidence interval]: 9.711 [1.708-182.294], p = 0.008; 4.642 [1.061-31.818], p = 0.041, respectively). Conclusions: The honeycomb area and presence of traction bronchiectasis on HRCT may predict poorer prognosis in patients with PPFE.
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