Combined pulmonary fibrosis and emphysema: a retrospective analysis of clinical characteristics, treatment and prognosis.
Combined pulmonary fibrosis and emphysema (CPFE) is increasingly acknowledged as a separate syndrome with distinct clinical, physiological and radiological characteristics. We sought to identify physiologic and radiographic indices that predict mortality in CPFE.



Data on clinical characteristics, pulmonary function, high-resolution computed tomography (HRCT) and treatment were compared between patients with usual interstitial pneumonia (UIP) plus emphysema (CPFE group) and those with IPF alone (IPF group). Composite physiologic index (CPI) and HRCT scores at diagnosis and during follow-up were assessed.



CPFE group (N = 87) was characterized by the predominance of males and smokers, who were less likely to have viral infection prior to the diagnosis, and display basal crackles, finger clubbing and wheeze, as compared to that in the IPF group (N = 105). HRCT and CPI scores increased over time in both groups. Moreover, CPFE group had a poorer prognosis, lower 5-year survival rate (43.42 % vs. 65.56 %; P < 0.05), and higher mortality (39.47 % vs. 23.33 %; P < 0.05) as compared to that in the IPF group. All CPFE patients received oxygen therapy, antibiotics and oral N-acetylcysteine; > 50 % received bronchodilators, 40 % received corticosteroids and 14 % needed noninvasive mechanical ventilation. On survival analyses, pulmonary arterial hypertension (PAH) and ≥ 5-point increase in CPI score per year were predictors of mortality in the CPFE group (hazard ratio [HR]: 10.29, 95 % Confidence Interval [CI]: 2.69-39.42 and HR: 21.60, 95 % CI: 7.28-64.16, respectively).



Patients with CPFE were predominantly male and smokers and exhibited distinct clinical, physiological and radiographic characteristics. They had a poorer prognosis than IPF. PAH and ≥ 5-point increase in CPI score per year were predictors of mortality in these patients. Future studies are needed to identify the optimal treatment approach to CPFE.
Department of Respiratory Medicine, First Affiliated Hospital of Harbin Medical University, 23 Youzheng Avenue, Nangang District, Harbin, Heilongjiang, China.
Lijuan
L
Zhang
Department of Respiratory Medicine, First Affiliated Hospital of Harbin Medical University, 23 Youzheng Avenue, Nangang District, Harbin, Heilongjiang, China.
Chunling
C
Zhang
Department of Respiratory Medicine, First Affiliated Hospital of Harbin Medical University, 23 Youzheng Avenue, Nangang District, Harbin, Heilongjiang, China.
Fushi
F
Dong
Department of Respiratory Medicine, First Affiliated Hospital of Harbin Medical University, 23 Youzheng Avenue, Nangang District, Harbin, Heilongjiang, China.
Qi
Q
Song
Department of Clinical Medicine, Harbin Medical University, Harbin, Heilongjiang, China.
Fangzhou
F
Chi
Department of Respiratory Medicine, First Affiliated Hospital of Harbin Medical University, 23 Youzheng Avenue, Nangang District, Harbin, Heilongjiang, China.
Lu
L
Liu
Department of Health Statistics, Harbin Medical University, Harbin, Heilongjiang, China.
Yupeng
Y
Wang
Department of Respiratory Medicine, First Affiliated Hospital of Harbin Medical University, 23 Youzheng Avenue, Nangang District, Harbin, Heilongjiang, China. chenchunli9559@163.com.
Chunli
C
Che
D000111:Acetylcysteine / Q000627:therapeutic use; D000368:Aged; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D002681:China; D005260:Female; D005541:Forced Expiratory Volume; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000150:complications* / Q000401:mortality* / Q000628:therapy; D008168:Lung / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D010102:Oxygen Inhalation Therapy; D011379:Prognosis; D011656:Pulmonary Emphysema / Q000150:complications* / Q000401:mortality* / Q000628:therapy; D012189:Retrospective Studies; D012720:Severity of Illness Index; D016019:Survival Analysis; D014057:Tomography, X-Ray Computed; D014797:Vital Capacity
D000900:Anti-Bacterial Agents; D000111:Acetylcysteine
Combined pulmonary fibrosis and emphysema; Computed tomography; Idiopathic pulmonary fibrosis; The composite physiologic index