Deterioration of Respiratory Symptoms in Uncontrolled CPFE: A Case Report
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Introduction: Chronic obstructive pulmonary disease (COPD) has a global impact on health and increases healthcare costs. Combined pulmonary fibrosis and emphysema (CPFE) combines interstitial lung disease (ILD) and emphysema. Excessive short-acting β2-agonist (SABA) usage escalates exacerbation risk, affecting prognosis.
Case: A 44-year-old former smoker presented with worsening shortness of breath, cough, and weight loss. Examination showed respiratory distress, including wheezing. Initially, he was diagnosed with tuberculosis (TB) and COPD exacerbation. He regularly used jet nebulizers without medical supervision, and during the initial treatment, he developed acute respiratory failure, leading to acidosis. Treatment involved SABA and short-acting muscarinic-antagonist (SAMA), steroids, and oxygen therapy. In November 2023, his condition worsened, requiring emergency treatment. Radiological findings indicated CPFE. Management included nebulized medications, intravenous steroids, and antibiotics. Despite challenges, he rarely attended follow-up appointments after showing improvement, missing scheduled rehabilitation therapy, inhaler monitoring, comorbid therapy, and home oxygen monitoring.
Conclusion: Combined pulmonary fibrosis and emphysema combines ILD and emphysema, causing severe respiratory impairment. Management mirrors that of COPD, involving inhalers, corticosteroids, and oxygen therapy.
Wong AW, Liang J, Cottin V, et al. Diagnostic Features in Combined Pulmonary Fibrosis and Emphysema: A Systematic Review. Annals of the American Thoracic Society 2020; 17: 1333–1336. [PubMed]
Hage R, Gautschi F, Steinack C, et al. Combined Pulmonary Fibrosis and Emphysema (CPFE) Clinical Features and Management. Int J Chron Obstruct Pulmon Dis 2021; 16: 167–177. [PubMed]
Cottin V, Selman M, Inoue Y, et al. Syndrome of Combined Pulmonary Fibrosis and Emphysema: An Official ATS/ERS/JRS/ALAT Research Statement. Am J Respir Crit Care Med 2022; 206: e7–e41. [PubMed]
Choi JY, Song JW, Rhee CK. Chronic Obstructive Pulmonary Disease Combined with Interstitial Lung Disease. Tuberc Respir Dis (Seoul) 2022; 85: 122–136. [PubMed]
Sangani R, Ghio A, Culp S, et al. Combined Pulmonary Fibrosis Emphysema: Role of Cigarette Smoking and Pulmonary Hypertension in a Rural Cohort. Int J Chron Obstruct Pulmon Dis 2021; 16: 1873–1885. [PubMed]
Ni H, Wei Y, Yang L, et al. An Increased Risk of Pulmonary Hypertension in Patients with Combined Pulmonary Fibrosis and Emphysema: A Meta-Analysis. BMC Pulm Med 2023; 23: 221. [PubMed]
Demirdöğen E, Dilektaşlı AG, Öztürk NAA, et al. Serum Krebs von den Lungen-6: Promising Biomarker to Differentiate CPFE from IPF. Sarcoidosis, Vasc Diffus Lung Dis Off J WASOG 2022; 39: e2022035. [PubMed]
Méthot DB, Leblanc É, Lacasse Y. Meta-Analysis of Gastroesophageal Reflux Disease and Idiopathic Pulmonary Fibrosis. Chest 2019; 155: 33–43. [PubMed]
Wang X, Wright Z, Wang J, et al. Elucidating the Link: Chronic Obstructive Pulmonary Disease and the Complex Interplay of Gastroesophageal Reflux Disease and Reflux-Related Complications. Medicina (Kaunas); 59. Epub ahead of print July 2023. [PubMed]
Global Initiative for Chronic Obstructive Lung Disease (GOLD). Global Strategy for the Diagnosis, Management, and Prevention of Chronic Obstructive Pulmonary Disease (2023 Report), https://goldcopd.org/wp-content/uploads/2023/03/GOLD-2023-ver-1.3-17Feb2023_WMV.pdf (2023).
Fu Y, Chapman EJ, Boland AC, et al. Evidence-Based Management Approaches for Patients with Severe Chronic Obstructive Pulmonary Disease (COPD): A Practice Review. Palliat Med 2022; 36: 770–782. [PubMed]
Janson C, Wiklund F, Telg G, et al. High Use of Short-Acting β(2)-Agonists in COPD is Associated with an Increased Risk of Exacerbations and Mortality. ERJ Open Res; 9. Epub ahead of print May 2023. [PubMed]
Nwaru BI, Ekström M, Hasvold P, et al. Overuse of Short-Acting β(2)-Agonists in Asthma is Associated with Increased Risk of Exacerbation and Mortality: A Nationwide Cohort Study of the Global SABINA Programme. Eur Respir J; 55. Epub ahead of print April 2020. [PubMed]
Ellis AK, Foran V, Kaplan A, et al. Clarifying SABA Overuse: Translating Canadian Thoracic Society Guidelines into Clinical Practice. Allergy, Asthma Clin Immunol 2022; 18: 48. [PubMed]
Maltais F, Naya IP, Vogelmeier CF, et al. Salbutamol Use in Relation to Maintenance Bronchodilator Efficacy in COPD: A Prospective Subgroup Analysis of the EMAX Trial. Respir Res 2020; 21: 280. [PubMed]
López-Campos JL, Gallego EQ, Hernández LC. Status of and Strategies for Improving Adherence to COPD Treatment. Int J Chron Obstruct Pulmon Dis 2019; 14: 1503–1515. [PubMed]
Sandelowsky H, Weinreich UM, Aarli BB, et al. COPD - Do the Right Thing. BMC Fam Pract 2021; 22: 244. [PubMed]
Sculley JA, Corbridge SJ, Prieto-Centurion V, et al. Home Oxygen Therapy for Patients with COPD: Time for a Reboot. Respir Care 2019; 64: 1574–1585. [PubMed]
Jacobs SS, Krishnan JA, Lederer DJ, et al. Home Oxygen Therapy for Adults with Chronic Lung Disease. An Official American Thoracic Society Clinical Practice Guideline. Am J Respir Crit Care Med 2020; 202: e121–e141. [PubMed]
McDonald CF. Home Oxygen Therapy. Aust Prescr 2022; 45: 21–24. [Journal]
Cottin V, Azuma A, Raghu G, et al. Therapeutic Effects of Nintedanib are not Influenced by Emphysema in the INPULSIS Trials. The European Respiratory Journal; 53. Epub ahead of print April 2019. [PubMed]
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