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

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3 Recommendations Part II. Diagnosis and Treatment of PPF Other Than IPF Pirfenidone ➤ We recommend further research into the efficacy, effectiveness, and safety of pirfenidone in both 1) non-IPF PPF in general and 2) specific types of interstitial lung disease manifesting PPF. Nintedanib ➤ We suggest nintedanib for the treatment of PPF in patients who have failed standard management (conditional recommendation, low- quality evidence). Remarks: Standard management will differ from patient to patient. In many patients it will be immunosuppressive treatment in an attempt to stabilize or reverse initial disease, but this is not a prerequisite since in some patients standard management could be antigen remediation or observation. Besides this, it should be acknowledged that in many ILDs, evidence-based guidance for standard of care is lacking. Hence, standard of care may vary from region to region. ➤ We recommend research into the efficacy, effectiveness, and safety of nintedanib in specific types of non-IPF interstitial lung disease manifesting PPF.

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