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Journal of Radiological Review 2021 June;8(2):132-43

DOI: 10.23736/S2723-9284.21.00136-0


language: English

Idiopathic pulmonary fibrosis complications: what a radiologist should know

Manuela MEREU 1 , Michela SCUTTI 1, Daniele VERI 1, Sabrina CONTE 1, Rosa L. PATEA 1, Lucio CALANDRIELLO 2, Stefano PALMUCCI 3, Adele VALENTINI 4, Alessandra FARCHIONE 2, Anna Rita LARICI 2, 5

1 Department of Radiology, Santissima Annunziata Hospital, Chieti, Italy; 2 Department of Diagnostic Imaging, Oncological Radiotherapy and Hematology, “A. Gemelli” University Polyclinic Foundation IRCCS, Rome, Italy; 3 Department of Medical Surgical Sciences and Advanced Technologies GF Ingrassia, Radiology Unit 1, “G. Rodolico-San Marco” University Polyclinic Hospital, University of Catania, Catania, Italy; 4 Department of Radiology, “San Matteo” Polyclinic Foundation IRCCS, Pavia, Italy; 5 Department of Radiological and Hematological Sciences, Section of Radiology, Catholic University of the Sacred Heart of Rome, Rome, Italy

Idiopathic pulmonary fibrosis (IPF) is a progressive chronic fibrosing lung disease of unknown causes. It has a variable clinical course, ranging from slow to rapid deterioration. Several are the IPF complications that may worse its course with a negative prognostic effect, such as infections, acute exacerbation, lung cancer, pulmonary hypertension, spontaneous pneumothorax and pneumomediastinum. The underlying fibrosis can modify the typical HRCT (high-resolution computed tomography) presentation of these complications. IPF patients are more susceptible to pulmonary infections, especially those caused by aspergillus species, opportunistic infections and mycobacterium tuberculosis. IPF is also recognized as an independent risk factor for lung cancer; squamous cell carcinoma is the most frequent histotype followed by adenocarcinoma. Acute exacerbation is defined as an acute, clinically significant respiratory deterioration characterized by the occurrence of new widespread alveolar abnormalities on HRCT. Three patterns of lung abnormalities have been suggested: peripheral, multifocal, and diffuse ground glass opacities. Multifocal and diffuse patterns have been associated with worse prognosis. Pulmonary hypertension impacts adversely on morbidity and mortality with a two to three-fold increase in IPF patients. Spontaneous pneumothorax and pneumomediastinum are frequently encountered in IPF patients and are not well tolerated. This review article aimed to describe the main possible complications occurring in patients with IPF, and to highlight the relevant contribution of HRCT in the diagnosis and management of these potentially life-threatening lung complications in this specific population.

KEY WORDS: Idiopathic pulmonary fibrosis; Infections; Lung neoplasms; Complications

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