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.
