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Vol 77, No 2 (2009)
CASE REPORTS
Published online: 2009-03-16
Submitted: 2013-02-22
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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.

open access

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.

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Keywords

combined pulmonary fibrosis and emphysema; pulmonary hypertension; chronic respiratory failure

About this article
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

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