Integration and Application of Clinical Practice Guidelines for the Diagnosis of Idiopathic Pulmonary Fibrosis and Fibrotic Hypersensitivity Pneumonitis

• Published in: Chest Vol. 162; no. 3; pp. 614 - 629
• Main Authors: Marinescu, Daniel-Costin, Raghu, Ganesh, Remy-Jardin, Martine, Travis, William D., Adegunsoye, Ayodeji, Beasley, Mary Beth, Chung, Jonathan H., Churg, Andrew, Cottin, Vincent, Egashira, Ryoko, Fernández Pérez, Evans R., Inoue, Yoshikazu, Johannson, Kerri A., Kazerooni, Ella A., Khor, Yet H., Lynch, David A., Müller, Nestor L., Myers, Jeffrey L., Nicholson, Andrew G., Rajan, Sujeet, Saito-Koyama, Ryoko, Troy, Lauren, Walsh, Simon L.F., Wells, Athol U., Wijsenbeek, Marlies S., Wright, Joanne L., Ryerson, Christopher J.
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01-09-2022; American College of Chest Physicians
• Series: Chest
• Subjects: Alveolitis, Extrinsic Allergic - diagnosis; Alveolitis, Extrinsic Allergic - pathology; clinical practice guidelines; Humans; hypersensitivity pneumonitis; idiopathic pulmonary fibrosis; Idiopathic Pulmonary Fibrosis - diagnosis; Idiopathic Pulmonary Fibrosis - pathology; Life Sciences; Lung - diagnostic imaging; Lung - pathology; Lung Diseases, Interstitial - diagnosis; Lung Diseases, Interstitial - pathology; multidisciplinary discussion; Tomography, X-Ray Computed; usual interstitial pneumonia; ALAT; ATS; ILD; usual interstitial pneumonia; hypersensitivity pneumonitis; NSIP; multidisciplinary discussion; JRS; idiopathic pulmonary fibrosis; CTD-ILD; MDD; fHP; clinical practice guidelines; IA; CPG; FPF; UIP; IPF
• ISSN: 0012-3692; 1931-3543
• DOI: 10.1016/j.chest.2022.06.013

Recent clinical practice guidelines have addressed the diagnosis of idiopathic pulmonary fibrosis (IPF) and fibrotic hypersensitivity pneumonitis (fHP). These disease-specific guidelines were developed independently, without clear direction on how to apply their respective recommendations concurrently within a single patient, where discrimination between these two fibrotic interstitial lung diseases represents a frequent diagnostic challenge. The objective of this review, created by an international group of experts, was to suggest a pragmatic approach on how to apply existing guidelines to distinguish IPF and fHP. Key clinical, radiologic, and pathologic features described in previous guidelines are integrated in a set of diagnostic algorithms, which then are placed in the broader context of multidisciplinary discussion to guide the generation of a consensus diagnosis. Although these algorithms necessarily reflect some uncertainty wherever strong evidence is lacking, they provide insight into the current approach favored by experts in the field based on currently available knowledge. The authors further identify priorities for future research to clarify ongoing uncertainties in the diagnosis of fibrotic interstitial lung diseases.