Background Cottin proposed that patients with emphysema of the superior lung field and fibrosis of the inferior lung field on thoracic CT should be regarded as having combined pulmonary fibrosis and emphysema (CPFE). They reported poor prognosis and marked reduction in pulmonary diffusion on a respiratory function test (1). Since then, various reports of CPFE have been published. However, there have been few reports on CPFE in patients with connective tissue disease (CTD).
Objectives To investigate the clinical characteristics of CPFE in patients with CTD who consulted our hospital and compare the prognoses.
Methods Of patients with interstitial pneumonia (IP) in the presence of rheumatoid arthritis (RA), dermatomyositis (DM), ANCA-associated vasculitis (AAV), or systemic sclerosis (SSc) who were treated in our hospital between August 1988 and October 2014, we identified the patients diagnosed of CPFE on thoracic HRCT findings. We retrospectively examined their clinical characteristics and prognoses. Patients in whom a low attenuation area accounted for 10% or more of the superior lung field on HRCT were regarded as having emphysema (2).
Results Overall, 512 patients had IP, and 116 had CPFE. RA-CPFE was detected in 61 (34.1%) of 179 patients with RA-IP. DM-CPFE was detected in 13 (11.6%) of 112 patients with DM-IP. AAV-CPFE was detected in 19 (20.7%) of 64 patients with AAV-IP. SSc-CPFE was detected in 23 (14.6%) of 157 patients with SSc-IP. CPFE was more frequent in RA and AAV patients. The mean age of the CPFE patients was 68.4±9.4 years. They consisted of 78 males and 38 females. There were 99 smokers (85.4%, Brinkman index: 904±514). Before the start of treatment, the LDH, KL-6, A-aDO2, %FVC, FEV1.0/FVC, RV/TLC, %DLco/VA, and eRVSP values were 245±101 U/mL, 841±783 U/mL, 37.1±30.0, 88.1±21.2%, 76.6±15.3%, 51.7±18.1%, 47.5±20.2%, and 32.3±11.9 mmHg, respectively. In the DM-CPFE and SSc-CPFE patients, the FEV1.0/FVC was higher than in the AAV-CPFE and RA-CPFE patients, and the %FVC and %DLco/VA were lower. Eighty-one patients had received PSL therapy, and 79 had received immunosuppressant. In 9, IVCY was combined. During the course, lung cancer was detected in 17 patients. Twenty-three patients died (exacerbation of IP: 12, lung cancer: 4, and others: 7). The mean survival was 12.5±1.1 years. The 3-, 5-, and 10-year survival rates after the start of treatment were 89.9, 75.3, and 66.5%, respectively. The prognosis of the DM-CPFE patients was poorer, and that of the SSc-CPFE patients was favorable. The prognoses of patients with A-aDO2 more than 35 and those with %DLco/VA less than 34 were significantly poor.
Conclusions CPFE with CTD was frequent in patients with RA or AAV. Its incidence was higher in males and smokers. Respiratory function tests showed reduction in diffusing capacity. The prognosis of the SSc-CPFE patients was favorable, whereas that of the DM-CPFE patients was poor. Prognostic factors were A-aDO2 and %DLco/VA.
Cottin V et al., Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity. Eur Respir J. 2005;26(4):586-93.
Mejía M et al., Idiopathic pulmonary fibrosis and emphysema: decreased survival associated with severe pulmonary arterial hypertension. Chest. 2009 Jul;136(1):10-5.
Disclosure of Interest None declared