Abstract
Idiopathic pulmonary fibrosis (IPF), the most common of the idiopathic interstitial pneumonias, is a devastating condition that carries a prognosis worse than that of many cancers. As such, it represents one of the most challenging diseases for chest physicians. The diagnostic process is complex and relies on the clinician integrating clinical, laboratory, radiologic, and/or pathologic data. Therefore, a close collaboration between chest physicians, radiologists, and pathologists experienced in the diagnosis of interstitial lung diseases (ILDs) is necessary in order to minimize diagnostic uncertainty. Similarly, the management of IPF continues to pose major difficulties. However, while there are no proven effective therapies for IPF beyond lung transplantation, recent trials of novel agents suggest that pharmacological treatment may retard the progression of the disease. In this regard, enrolment of patients into clinical trials is considered the “best current practice”by the most recent guidelines as it offers IPF patients the chance to receive new agents that may be more effective than current therapies. A more recent trend focusing on improving quality of life in IPF patients has also been gaining ground.The diagnosis and management of IPF remains a constant challenge for even the most experienced of clinicians. However, a multidisciplinary approach to this complex disease is steadily improving diagnostic accuracy, while recent advances in the pharmacological therapy offer the genuine promise of future treatments for this devastating disease.
Highlights
Idiopathic pulmonary fibrosis (IPF) is a progressive fibrosing interstitial pneumonia of unknown cause, limited to the lung and associated with the histopathological and/or radiological pattern of usual interstitial pneumonia (UIP) [1]
The diagnosis of IPF is established in the presence of a UIP pattern on highresolution computed tomography (HRCT) of the chest and/or in the surgical lung biopsy (SLB) specimen in the
Less than one-third of a large patient cohort evaluated at a center with extensive expertise in interstitial lung diseases (ILDs) could realize these currently recommended treatment options, highlighting the need for other management approaches [11]
Summary
Idiopathic pulmonary fibrosis (IPF) is a progressive fibrosing interstitial pneumonia of unknown cause, limited to the lung and associated with the histopathological (evidence of patchy involvement of lung parenchyma by fibrosis/architectural distortion, honeycombing in a predominantly subpleural/paraseptal distribution, presence of fibroblast foci) and/or radiological (subpleural, basalpredominant honeycombing and reticular abnormality, with or without traction bronchiectasis) pattern of usual interstitial pneumonia (UIP) [1]. IPF may present, though rarely, acutely in patients without a known history of chronic lung disease [39] In such cases, lung biopsy (often difficult to obtain due to patient clinical conditions) shows diffuse alveolar damage (DAD) superimposed on a UIP pattern, similar to what is seen in acute exacerbations of IPF (AE-IPF). Referral to a tertiary care center IPF is often initially misdiagnosed - at least until physiological and imaging data suggest the presence of an interstitial lung disease - leading to delay in accessing appropriate care This point is not trivial in a disease with a median survival of 3 years after diagnosis.
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