Chronic Bacterial Infection as a Cause of Combined Pulmonary Fibrosis and Emphysema Worsening.

SESSION TYPE: ILD Global Case Report PostersPRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PMINTRODUCTION: Cottin et al. (2005) characterized a syndrome of combined pulmonary fibrosis and emphysema (CPFE) as a smoking-related disease with severe dyspnea, unexpected subnormal spirometry data, severely impaired diffusion capacity of carbon monoxide (DLco), hypoxemia at exercise, and a characteristic CT finding indicating emphysema and lower-lobe fibrosis. Pulmonary hypertension contributes to the poor prognosis of CPFE. However, the pathophysiology of CPFE is unclear, and corticosteroids or immunosuppressants are reported to be of no significant benefit. Here, we report a case suggesting chronic infection with bacteria (Achromobacter xylosoxidans subsp. xylosoxidans) is one of the aggravating factors of CPFE.CASE PRESENTATION: A 77-year-old man, who has a history of smoking (49 packets/year) until the age of 69, was admitted to our hospital because of increasing dyspnea and rapid deterioration in vital capacity (VC) since 2010. He had been followed up in another hospital without treatment with steroids, immunosuppressants, or antibiotics since February 2004. His VC, forced expiratory volume in 1 second (FEV1.0), and DLco in March of 2010 were 3.29 L (106%), 2.65 L (128%), and 4.43 ml/min/mmHg (32.9%), respectively. His chest CT scan showed characteristic emphysema and lower-lobe honeycombing in both lungs. The rate of decrease of his VC was 208 ml/year. He started oxygen therapy a year ago. Due to increasing dyspnea, he was introduced to our hospital in November of 2010. His vital capacity was decreasing at a rate of 695 ml/year. WBC and CRP in peripheral blood half a year after introduction to our hospital were 6600/μl and 0.36 mg/dl, respectively, and there was no sign of infection. A bronchoscopy was performed and a lot of purulent sputum was found throughout every bronchus. Achromobacter xylosoxidans subsp. xylosoxidans, which is a non-fermentative gram-negative bacillus, was identified by culture. Antibiotic therapy with ceftazidime, but not with corticosteroid or immunosuppressant, was started. On the 16th hospital day, he was discharged with improvement of respiratory function and respiratory status. His condition exacerbated several times after leaving hospital and Achromobacter was sometimes found in his sputum. Antibiotic therapy succeeded each time following exacerbation. After introduction of antibiotic therapy, the rate of deterioration of his VC decreased to 161 ml/year.DISCUSSION: Most patients with CPFE have a history of smoking and are usually male, but no other background factor has been reported. In addition, there is no consensus regarding whether emphysema and fibrosis progress independently or whether they influence each other. Furthermore, the role of bacterial infection in the pathogenesis or pathophysiology of CPFE has not been thoroughly investigated. The report of Cottin et al. (2005) showed that the neutrophil cell count in bronchoalveolar lavage fluids ranged from 2 to 73%, indicating the inclusion of patients with possible bacterial infection. Additionally, Lee et al. (2011) reported that respiratory problems, including pneumonia, were a more common cause of death in CPFE than emphysema. Although it is rare to find IPF patients with chronic bacterial infection, it is much more frequent to find COPD patients with chronic bacterial infection. A consideration of the view that CPFE is a synergistic complication of IPF and COPD should take into account chronic bacterial infection in patients with CPFE. Obviously, corticosteroid or immunosuppressant therapy must be administered more carefully than in patients with IPF alone.CONCLUSIONS: We present a case of CPFE exhibiting chronic infection with Achromobacter xylosoxidans subsp. xylosoxidans. Antibiotics reduced the speed of deterioration of lung function, which indicates the possible involvement of chronic bacterial infection in the worsening of CPFE in this case. We should be mindful of the possibility of chronic bacterial infection as a causative and worsening factor of CPFE.1) Cottin V, Nunes H, Brillet P, et al. Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity. Eur Respir J 2005; 26:586-5932) Lee CH, Kim HJ, Park CM, et al. The impact of combined pulmonary fibrosis and emphysema on mortality. Int J Tuberc Lung Dis. 2011; 15:1111-63) Mayra Mejia, Guillermo Carrillo, Jorge Rojas-Serrano, et al. Idiopathic pulmonary fibrosis and emphysema: decreased survival associated with severe pulmonary arterial hypertension. Chest 2009; 136:10-15DISCLOSURE: The following authors have nothing to disclose: Kei Ebisawa, Shinji Okada, Norihiro Yamada, Makoto Kobayashi, Yasuko Suzuki, Asami SatohNo Product/Research Disclosure InformationSouth Miyagi Medical Center, Ogawara, Japan.