Treatment Recommended Strength of recommendation Quality of evidence Pharmacologic therapies Corticosteroid monotherapy No Strong ⊕○○○ Colchicine No Strong ⊕○○○ Cyclosporine A No Strong ⊕○○○ Corticosteroid + immunomodulatory No Strong ⊕○○○ Corticosteroid + azathioprine + acetylcysteine Majority…
Study Number of patients with CPFE/with IIPs Prevalence (%) Akira et al. [8] 15/80 18.8 Choi et al. [9] 66/254 26.0 Copley et al. [10] 76/212 35.8 Doherty et al….
Fig. 11.1 Oxygen time courses in pulmonary capillary when diffusion is normal and abnormal (e.g., because of thickening of the blood–gas barrier by disease) (Reprinted with permission of Wolters Kluwer…
Fig. 5.1 Structures of SP-A and SP-D. Monomeric and oligomeric structures of SP-A and SP-D. Monomeric structures can be conceptually divided into four major structural domains: a short N-terminal segment…
Fig. 3.1 Clinical disease courses in patients with IPF. The typical natural history of IPF is the slow progression of the disease with a median survival period of approximately 3 years…
© Springer Japan 2016Hiroyuki Nakamura and Kazutetsu Aoshiba (eds.)Idiopathic Pulmonary Fibrosis10.1007/978-4-431-55582-7_12 12. Pharmacotherapy of Acute Exacerbation of IPF (Corticosteroids, Immunosuppressants, and Direct Hemoperfusion with Polymyxin) Are High-Dose Steroid Therapy, Other Immunosuppressant Therapy, and PMX Therapy…
Fig. 1.1 Diagnostic algorithm for idiopathic pulmonary fibrosis (IPF). Patients with suspected IPF should be carefully evaluated for identifiable causes of interstitial lung disease (ILD). In the absence of an…
Fig. 9.1 Pathogenesis of IPF and the mechanism of action of antifibrotic agents 9.3 Pirfenidone 9.3.1 Mechanism of Action Animal studies have shown that pirfenidone suppresses accumulation of inflammatory cells…
Fig. 4.1 Schematic drawing of the proposed molecular pathogenesis of IPF. AEC, alveolar epithelial cell; EMT, epithelial-mesenchymal transition; ECM, extracellular matrix 4.2 Epithelial Injury and Genetic Background Studies of the…
Fig. 2.1 Alveolar epithelial cells are thought to be the initial lesion of lung injury caused by environmental factors, such as smoking, inhalational agents, drugs, oxygen radicals, toxins, and viruses,…
