INSIDE ▶ Introduction ▶ Recommendations ▶ Tables ▶ Figures Idiopathic Pulmonary Fibrosis and Progressive Pulmonary Fibrosis

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DISCLAIMER This pocket guide attempts to define principles of practice that should produce high-quality patient care. It is applicable to specialists, primary care, and providers at all levels. This pocket guide should not be considered exclusive of other methods of care reasonably directed at obtaining the same results. The ultimate judgment concerning the propriety of any course of conduct must be made by the clinician after consideration of each individual patient situation. Neither IGC, the medical associations, nor the authors endorse any product or service associated with the distributor of this clinical reference tool. CONSULTANTS Ganesh Raghu, MD | Martine Remy-Jardin, MD | Luca Richeldi, MD | Carey C. Thomson, MD | Yoshikazu Inoue, MD | Takeshi Johkoh, MD | Michael Kreuter, MD | David A. Lynch, MD | Toby M. Maher, MD | Fernando J. Martinez, MD | Maria Molina- Molina, MD | Jeffrey L. Myers, MD | Andrew G. Nicholson, MD | Christopher J. Ryerson, MD | Mary E. Strek, MD | Lauren K. Troy, MD | Marlies Wijsenbeek, MD ABBREVIATIONS AEF, airspace enlargement with fibrosis; AFOP, acute fibrinous and organizing pneumonia; AIP, acute interstitial pneumonia; BAL, bronchoalveolar lavage fluid; COP, cryptogenic organizing pneumonia; DIP, desquamative interstitial pneumonia; D L CO, diffusing capacity of the lungs for CO; dx, diagnosis; CT, computed tomography; CTD, connective tissue disease; CTD-ILD, connective tissue disease-associated ILD; F-LCH, fibrotic Langerhan's cell histiocytosis; F-NSIP, fibrotic non-specific interstitial pneumonia; FOP, fibrosing organizing pneumonia; FVC, forced vital capacity; GCT, genomic classifier testing; GER, gastroesophageal reflux; GERD, gastroesophageal reflux disease; GGO, ground-glass opacity; Hb, hemoglobin; HP, hypersensitivity pneumonitis; HRCT, high-resolution computed tomography; IAFE, intraalveolar fibrosis and elastosis; iDIP, idiopathic desquamative interstitial pneumonia; IIP, Idiopathic interstitial pneumonia; ILA, interstitial lung abnormalities; ILD, interstitial lung disease; iLIP, idiopathic lymphoid interstitial pneumonia; iNSIP, idiopathic non-specific interstitial pneumonia; IP, interstitial pneumonia; IPF, idiopathic pulmonary fibrosis; iPPFE, idiopathic pleuroparenchymal fibroelastosis; LAM, lymphangioleiomyomatosis; LCH, Langerhan's cell histiocytosis; LIP, lymphocytic interstitial pneumonia; MCTD, mixed connective tissue disease; MDD, multidisciplinary discussion; NSIP, non-specific interstitial pneumonia; PAP, pulmonary alveolar proteinosis; PLCH, pulmonary Langerhan's cell histiocytosis; PM/DM, polymyositis/dermatomyositis; PPF, progressive pulmonary fibrosis; PPFE, pleuroparenchymal fibroelastosis; RA, rheumatoid arthritis; RB-ILD, respiratory bronchiolitis interstitial lung disease; SLB, surgical lung biopsy; SLE, systemic lupus erythematosus; SSc, systemic sclerosis; TBLC, transbronchial lung cryobiopsy; UIP, usual interstitial pneumonia SOURCE Raghu G, Remy-Jardin M, Richeldi L, et al. Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. 2022;205(9):e18-e47. doi:10.1164/rccm.202202-0399st 106 Commerce Street, Suite 105 Lake Mary, FL 32746 TEL: 407.878.7606 ⋅ FAX: 407.878.7611 Order additional copies at Copyright © 2022 All rights reserved ATSIPF03223

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