The Syndrome of Combined Pulmonary Fibrosis and Emphysema
| Autor: | Vincent Cottin, Jean-François Cordier |
|---|---|
| Přispěvatelé: | Rétrovirus et Pathologie Comparée (RPC), Institut National de la Recherche Agronomique (INRA)-École pratique des hautes études (EPHE), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Ecole Nationale Vétérinaire de Lyon (ENVL) |
| Rok vydání: | 2009 |
| Předmět: |
Pulmonary and Respiratory Medicine
Spirometry medicine.medical_specialty [SDV]Life Sciences [q-bio] medicine.medical_treatment Critical Care and Intensive Care Medicine 03 medical and health sciences Idiopathic pulmonary fibrosis 0302 clinical medicine Usual interstitial pneumonia Internal medicine Pulmonary fibrosis medicine Lung transplantation ComputingMilieux_MISCELLANEOUS Lung medicine.diagnostic_test business.industry respiratory system medicine.disease Combined pulmonary fibrosis and emphysema Pulmonary hypertension respiratory tract diseases 3. Good health medicine.anatomical_structure 030228 respiratory system 030220 oncology & carcinogenesis Cardiology Cardiology and Cardiovascular Medicine business |
| Zdroj: | Chest Chest, American College of Chest Physicians, 2009, 136 (1), pp.1-2. ⟨10.1378/chest.09-0538⟩ |
| ISSN: | 0012-3692 |
| Popis: | Combined pulmonary fibrosis and emphysema (CPFE) is a recently identified syndrome occurring almost exclusively in smokers or ex-smokers, characterized by severe dyspnea and limitation to exercise capacity. Tobacco smoking is the main etiologic factor. However, it may occur in the context of connective tissue disease especially rheumatoid arthritis and systemic sclerosis. Velcro-crackles are present at lung auscultation. CPFE may be overlooked due to preserved lung volumes and subnormal spirometry, however gas exchange is severely impaired, with hypoxemia at exercise. The diagnosis is based on the presence at HRCT of both emphysema predominating in the upper lobes and frequently paraseptal, and interstitial abnormalities suggesting pulmonary fibrosis in the lower zones of the lungs, with especially reticulation, traction bronchiectasis, honeycombing, and possible ground glass opacities. Thick-walled large cysts are another distinctive feature at imaging. Pathologic radiological correlations are difficult owing to various pathology and difficulties in identifying honeycombing at chest HRCT in the setting of coexistent emphysema. Lung pathology when available may show a pattern of usual interstitial pneumonia, however other patterns can also be found. Survival is generally comparable to that of idiopathic pulmonary fibrosis (without emphysema), however with an increased risk of precapillary pulmonary hypertension associated with a dismal prognosis. Lung cancer and acute exacerbation of pulmonary fibrosis may further hamper the prognosis. The CPFE syndrome impacts modalities of follow-up with a relative lack of restrictive physiology despite progression of disease, and the need to monitor for pulmonary hypertension. Management is mostly supportive, with smoking cessation, inhaled bronchodilators, and long-term oxygen therapy or lung transplantation when appropriate. Drug therapy with pirfenidone or nintedanib, which reduce the decline in lung function in idiopathic pulmonary fibrosis, might be used pending further evaluation. There are no data to support treatment specific for pulmonary hypertension in CPFE outside clinical studies or prospective registries. |
| Databáze: | OpenAIRE |
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