Japanese guideline for the treatment of idiopathic pulmonary fibrosis 2017

Abstract

Objective: To improve clinical outcomes for idiopathic pulmonary fibrosis (IPF), the Japanese Guideline for Treatment of Idiopathic Pulmonary Fibrosis 2017 was developed to standardize treatment and management of IPF. Methods: The Japan Ministry of Health, Labour and Welfare Research Committee on Diffuse Pulmonary Disorders used the GRADE system to develop the first edition of a clinical practice guideline that includes clinical questions (CQs) and recommendations. Results: The nine sub-CQs addressing treatment and management recommendations for progressive IPF include corticosteroids alone, immunosuppressants combined with corticosteroids, inhaled N-acetylcysteine monotherapy, pirfenidone, nintedanib, pirfenidone combined with inhaled N-acetylcysteine, pirfenidone combined with nintedanib, long-term oxygen therapy, and pulmonary rehabilitation. The five sub-CQs for acute exacerbation of IPF include corticosteroid pulse therapy, immunosuppressants, sivelestat sodium hydrate, direct hemoperfusion with a polymyxin-B–immobilized fiber column, and recombinant human soluble thrombomodulin. The three sub-CQs for coexisting IPF and lung cancer include surgical therapy, perioperative prophylactic treatment, and chemotherapy. For all CQs except pirfenidone and nintedanib, the certainty/quality of evidence was low or very low and the strength of the recommendations was weak. In addition to recommendations for CQs, the guidelines include a summary of evidence, a discussion by the guideline development group, and a list of studies evaluated. Conclusion: The new clinical practice guideline for refractory IPF is expected to standardize IPF treatment and management and improve IPF outcomes among Japanese.