Literature DB >> 35602819

Combined Pulmonary Fibrosis and Emphysema and Digital Clubbing.

Taha F Rasul1, Daniel R Bergholz2, Randal Rovinski3, Sana Gulraiz4, Ernesto Fonts5.   

Abstract

Combined pulmonary fibrosis and emphysema (CPFE) is an underrecognized syndrome that involves simultaneous restrictive-obstructive lung disease. The prognosis is poor, and it frequently occurs with comorbidities. Heavy or former smoking is a major risk factor, and computed tomography (CT) typically shows lower zone fibrosis and upper zone emphysema. Chronic respiratory failure, pulmonary hypertension, and lung carcinoma are major causes of mortality. Diagnosis of CPFE should be combined with palliative care due to the high mortality of the condition, especially in the case of delayed diagnosis. We present the case of a 73-year-old male with a history of non-small cell lung cancer, 50 pack-year smoking, and cervical spine injury (CSI) with a late diagnosis of CPFE. After presenting to the emergency department for an acute exacerbation of dyspnea and hypoxia, he was initially treated with a congestive heart failure protocol. Further examination showed mixed pulmonary function tests as well as digital clubbing, and a CT scan showed changes indicative of advanced bullous emphysema diffusely throughout both lungs with an upper lobe predominance and basilar fibrosis. He was diagnosed with CPFE and immediately treated for both restrictive and obstructive lung diseases with supplemental oxygen, albuterol, ipratropium, corticosteroids, systemic antibiotics, as well as provided with palliative consultation. His previous history and CSI delayed diagnosis, as his lung restriction was likely assumed to be from impaired chest wall mobility rather than CPFE. This case highlights the presentation of a relatively rare disease that was confounded by comorbidities.
Copyright © 2022, Rasul et al.

Entities:  

Keywords:  cervical spine injury; combined pulmonary fibrosis emphysema; cpfe; delayed diagnosis; multidisciplinary; multidisciplinary decision-making

Year:  2022        PMID: 35602819      PMCID: PMC9115620          DOI: 10.7759/cureus.24231

Source DB:  PubMed          Journal:  Cureus        ISSN: 2168-8184


  18 in total

1.  High-resolution computed tomographic characteristics in acute farmer's lung and in its follow-up.

Authors:  Y Cormier; M Brown; S Worthy; G Racine; N L Müller
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2.  Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity.

Authors:  V Cottin; H Nunes; P-Y Brillet; P Delaval; G Devouassoux; I Tillie-Leblond; D Israel-Biet; I Court-Fortune; D Valeyre; J-F Cordier
Journal:  Eur Respir J       Date:  2005-10       Impact factor: 16.671

Review 3.  Combined pulmonary fibrosis and emphysema syndrome: a review.

Authors:  Matthew D Jankowich; Sharon I S Rounds
Journal:  Chest       Date:  2012-01       Impact factor: 9.410

4.  Interleukin-1beta causes pulmonary inflammation, emphysema, and airway remodeling in the adult murine lung.

Authors:  Urpo Lappalainen; Jeffrey A Whitsett; Susan E Wert; Jay W Tichelaar; Kristina Bry
Journal:  Am J Respir Cell Mol Biol       Date:  2005-01-24       Impact factor: 6.914

Review 5.  Pulmonary talcosis: imaging findings.

Authors:  Edson Marchiori; Sílvia Lourenço; Taisa Davaus Gasparetto; Gláucia Zanetti; Cláudia Mauro Mano; Luiz Felipe Nobre
Journal:  Lung       Date:  2010-02-13       Impact factor: 2.584

6.  Quantitative relation between emphysema and lung mineral content in coalworkers.

Authors:  J Leigh; T R Driscoll; B D Cole; R W Beck; B P Hull; J Yang
Journal:  Occup Environ Med       Date:  1994-06       Impact factor: 4.402

7.  Cause of regional ventilation-perfusion mismatching in patients with idiopathic pulmonary fibrosis: a combined CT and scintigraphic study.

Authors:  N H Strickland; J M Hughes; D A Hart; M J Myers; J P Lavender
Journal:  AJR Am J Roentgenol       Date:  1993-10       Impact factor: 3.959

8.  Heterogeneity in combined pulmonary fibrosis and emphysema.

Authors:  Matthew D Jankowich; Michael Polsky; Michael Klein; Sharon Rounds
Journal:  Respiration       Date:  2007-08-06       Impact factor: 3.580

9.  HRCT and histopathological evaluation of fibrosis and tissue destruction in IPF associated with pulmonary emphysema.

Authors:  Paola Rogliani; Marco Mura; Paolo Mattia; Amedeo Ferlosio; Gianfranco Farinelli; Salvatore Mariotta; Paolo Graziano; Gabriella Pezzuto; Alberto Ricci; Cesare Saltini; Augusto Orlandi
Journal:  Respir Med       Date:  2008-08-23       Impact factor: 3.415

10.  Lung cancer in combined pulmonary fibrosis and emphysema: a large retrospective cohort analysis.

Authors:  Faria Nasim; Teng Moua
Journal:  ERJ Open Res       Date:  2020-12-14
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