Idiopathic Pulmonary Fibrosis

Hiroyuki Nakamura, MD, PhD.Department of Respiratory Medicine,Tokyo Medical University Ibaraki Medical Center, JapanKazutetsu Aoshiba, MD, PhD.Department of Respiratory Medicine,Tokyo Medical University Ibaraki Medical Center, Japan

• ​Part 1.- Definition, epidemiology, and pathogenesis .- Chapter 1: Definition of IPF  (Question: Is the latest classification [ATS/ERS] satisfactory?).- Chapter 2: Epidemiology and risk factors of IPF  (Question: Can we dare to ask, “What are the possible causes?”).- Chapter 3: Acute exacerbation of IPF  (Question: The concept was proposed in Japan, but why was it not recognized in western countries?).- Chapter 4: Pathogenesis   (Question: Is abnormal repair of epithelial damage involved in the basic pathogenesis of this disease?) .- Part 2 Diagnosis    .- Chapter 5: Specific serum markers of IPF  (Question: What are the significances of KL-6, SP-A, and SP-D?).- Chapter 6: High-resolution CT of honeycombing and IPF/UIP  (Question: To what extent can honeycomb lung be diagnosed by imaging? To what extent can IPF diagnosis be made by HRCT?).- Chapter 7: Pathology of IPF  (Question: Why does the pathological classification of IIPs vary among pathologists?).- Chapter 8: Differential diagnosis  (Question: What should we particularly keep in mind in the differential diagnosis?) .- Part 3 Management and prognosis    .- Chapter 9 Pharmacotherapy of IPF (antifibrotic compounds)  (Question: Can antifibrotic agents really inhibit fibrosis? How about the development of new therapeutic agents?).- Chapter 10: Pharmacotherapy of IPF (Corticosteroids, immunosuppressants)  (Question: Are these actually effective? Ineffective? Harmful?).- Chapter 11: Non-pharmacological therapy for IPF  (Question: Is respiratory care actually effective?).- Chapter 12: Pharmacotherapy of acute exacerbation of IPF (Corticosteroids, immunosuppressants and direct hemoperfusion with polymyxin B)  (Question: Are high-dose steroid therapy, other immunosuppressant therapy, and PMX therapy [often used in Japan] really effective?) .- Part 4 Topics    .- Chapter 13 :Combined pulmonary fibrosis and emphysema (CPFE)  (Question:Is it an independent disease entity?).- Chapter 14: Common pathways in IPF and lung cancer  (Question: Why is lung cancer associated with IPF at a high frequency?).- Chapter 15 :Acute exacerbation of IIPs after pulmonary resection for lung cancer   (Question: Can acute exacerbation of IPF be predicted preoperatively?)