Interstitial lung diseases (ILDs) refer to a broad category of more than 200 lung diseases encompassing a variety of illnesses with diverse causes, treatments, and prognoses. These disorders can be separated in those with an underlying disorder, including connective tissue diseases (CTDs) and Hermansky-Pudlak syndrome (HPS), or known exposure, such as hypersensitivity pneumonitis, asbestosis, drug-induced ILD, and many others. In the absence of exposure or underlying cause, the disease belongs to the group of idiopathic interstitial cases of pneumonia (IIPs) [1]. In this group, idiopathic pulmonary fibrosis (IPF) is the most frequent and most aggressive form of IIP [2]. ILDs might have several similarities, including radiological and pathological presentation which makes it the main challenge to make a confident diagnosis and an appropriate treatment approach. Particularly, a number of these disorders show usual interstitial pneumonia (UIP) pattern, suggesting that UIP is not itself diagnostic of IPF. For these reasons, the diagnosis of ILDs-including those with a UIP pattern-needs a full work-up to exclude prior exposures or an underlying disease.
Luppi, F., Richeldi, L. (2023). Idiopathic Pulmonary Fibrosis and the Many Faces of UIP. In V. Cottin, L. Richeldi, K. Brown, F.X. McCormack (a cura di), Orphan Lung Diseases A Clinical Guide to Rare Lung Disease (pp. 549-560). Springer [10.1007/978-3-031-12950-6_32].
Idiopathic Pulmonary Fibrosis and the Many Faces of UIP
Luppi F.;
2023
Abstract
Interstitial lung diseases (ILDs) refer to a broad category of more than 200 lung diseases encompassing a variety of illnesses with diverse causes, treatments, and prognoses. These disorders can be separated in those with an underlying disorder, including connective tissue diseases (CTDs) and Hermansky-Pudlak syndrome (HPS), or known exposure, such as hypersensitivity pneumonitis, asbestosis, drug-induced ILD, and many others. In the absence of exposure or underlying cause, the disease belongs to the group of idiopathic interstitial cases of pneumonia (IIPs) [1]. In this group, idiopathic pulmonary fibrosis (IPF) is the most frequent and most aggressive form of IIP [2]. ILDs might have several similarities, including radiological and pathological presentation which makes it the main challenge to make a confident diagnosis and an appropriate treatment approach. Particularly, a number of these disorders show usual interstitial pneumonia (UIP) pattern, suggesting that UIP is not itself diagnostic of IPF. For these reasons, the diagnosis of ILDs-including those with a UIP pattern-needs a full work-up to exclude prior exposures or an underlying disease.
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