Vol 77, No 2 (2009)
CASE REPORTS
Published online: 2009-03-16
Submitted: 2013-02-22
Combined pulmonary fibrosis and emphysema - case report and literature review
Monika Kosacka, Anna Brzecka, Renata Jankowska, Jerzy Lewczuk, Ewa Mroczek, Bożena Weryńska
Pneumonol Alergol Pol 2009;77(2):205-210.
Vol 77, No 2 (2009)
CASE REPORTS
Published online: 2009-03-16
Submitted: 2013-02-22
Abstract
We describe the case of a 61-year-old male patient, in which the search for the cause of chronic respiratory failure, severe
pulmonary hypertension and secondary erythrocytosis resulted in a diagnosis of combined pulmonary fibrosis and emphysema
(CPFE). This is a unique, recently characterised syndrome with upper-lobe emphysema and pulmonary fibrosis of the
lower lungs. The cause is unknown, but one of the main risk factor remains smoking. The patient was a heavy smoker (over
40 pack-years). He complained of dyspnoea on exertion and cough. Physical examination revealed basal crackles and
cyanosis. The patient had severe reduction in diffusing capacity, out of proportion to his lung volumes (DLCO 27% of
predicted value, FEV1 2.95 l (100%), FVC 4.41 l (118%), FEV1/FVC (66%). The blood gas showed hypoxemia (pO2 37 mm Hg),
hypocapnia and respiratory alkalosis. Diagnosis was based on chest computer tomography, which revealed upper lobe
emphysema and lower lobe ground glass changes and honeycombing. Severe pulmonary hypertension (SPAP 80 mm Hg)
was confirmed by echocardiography and right cardiac catherisation. The patient received long-term oxygen therapy, inhaled
corticosteroid and Ca-blocker.
Abstract
We describe the case of a 61-year-old male patient, in which the search for the cause of chronic respiratory failure, severe
pulmonary hypertension and secondary erythrocytosis resulted in a diagnosis of combined pulmonary fibrosis and emphysema
(CPFE). This is a unique, recently characterised syndrome with upper-lobe emphysema and pulmonary fibrosis of the
lower lungs. The cause is unknown, but one of the main risk factor remains smoking. The patient was a heavy smoker (over
40 pack-years). He complained of dyspnoea on exertion and cough. Physical examination revealed basal crackles and
cyanosis. The patient had severe reduction in diffusing capacity, out of proportion to his lung volumes (DLCO 27% of
predicted value, FEV1 2.95 l (100%), FVC 4.41 l (118%), FEV1/FVC (66%). The blood gas showed hypoxemia (pO2 37 mm Hg),
hypocapnia and respiratory alkalosis. Diagnosis was based on chest computer tomography, which revealed upper lobe
emphysema and lower lobe ground glass changes and honeycombing. Severe pulmonary hypertension (SPAP 80 mm Hg)
was confirmed by echocardiography and right cardiac catherisation. The patient received long-term oxygen therapy, inhaled
corticosteroid and Ca-blocker.
Keywords
combined pulmonary fibrosis and emphysema; pulmonary hypertension; chronic respiratory failure
Title
Combined pulmonary fibrosis and emphysema - case report and literature review
Journal
Advances in Respiratory Medicine
Issue
Vol 77, No 2 (2009)
Pages
205-210
Published online
2009-03-16
Bibliographic record
Pneumonol Alergol Pol 2009;77(2):205-210.
Keywords
combined pulmonary fibrosis and emphysema
pulmonary hypertension
chronic respiratory failure
Authors
Monika Kosacka
Anna Brzecka
Renata Jankowska
Jerzy Lewczuk
Ewa Mroczek
Bożena Weryńska
About this article