Here, we report a case suggesting chronic infection with bacteria (Achromobacter xylosoxidans subsp. xylosoxidans) is one of the aggravating factors of CPFE.
【Title】Chronic Bacterial Infection as a Cause of Combined Pulmonary Fibrosis and Emphysema Worsening. Kei Ebisawa, Shinji Okada, Norihiro Yamada, Makoto Kobayashi, Yasuko Suzuki, Asami Satoh South Miyagi Medical Center, Okawara, Miyagi, Japan
【Introduction】 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. CT scan showed characteristic emphysema and lower-lobe honeycombing in both lungs. ml/year.
He started oxygen therapy a year ago.
His chest
The rate of decrease of his VC was 208
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. status.
On the 16th hospital day, he was discharged with improvement of respiratory function and respiratory
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. infection as a causative and worsening factor of CPFE.
We should be mindful of the possibility of chronic bacterial
【Reference】 Reference #1
Cottin V, Nunes H, Brillet P, et al. Combined pulmonary fibrosis and emphysema: a distinct
underrecognised entity. Eur Respir J 2005; 26:586-593 Reference #2
Lee CH, Kim HJ, Park CM, et al. The impact of combined pulmonary fibrosis and emphysema on
mortality. Int J Tuberc Lung Dis. 2011; 15:1111-6 Reference #3 Mayra Mejia, Guillermo Carrillo, Jorge Rojas-Serrano, et al. Idiopathic pulmonary fibrosis and emphysema: decreased survival associated with severe pulmonary arterial hypertension. Chest 2009; 136:10-15
DISCLOSURE: The following authors have nothing to disclose: Kei Ebisawa, Shinji Okada, Norihiro Yamada, Makoto Kobayashi, Yasuko Suzuki, and Asami Satoh.
