Table 1.

Summary of recommendations.

Module	Pico Question		Recommendation	Quality of Evidence	Strength of Recommendation
DIAGNOSIS	1	Can a combination of data indicating an increased severity of clinical symptoms, worsening of respiratory function parameters, and progression of lesions in high-resolution computed tomography (HRCT) serve as a basis for the diagnosis of progressive fibrosing interstitial lung disease (PF-ILD)?	We suggest that the basis for the diagnosis of progressive fibrosing interstitial lung disease (PF-ILD) should be an increased severity of clinical symptoms, worsening of pulmonary function parameters, and progression of lesions in high-resolution computed tomography of the chest.	Very low	Conditional
	2	In each patient with fibrotic interstitial lung disease, should we aim at establishing a precise diagnosis of the underlying disease?	We recommend that in every patient with fibrotic interstitial lung disease, we should aim for establishing a precise diagnosis of the underlying disease.	Very low	Strong
	3	Is it necessary to perform serological tests for autoimmune diseases in every patient with fibrotic interstitial lung disease of unknown etiology?	We recommend that every patient with fibrotic interstitial lung disease of unknown etiology should undergo serological tests for autoimmune diseases.	Very low	Strong
	4	Is an opinion of a multidisciplinary team necessary for establishing a diagnosis of a fibrotic interstitial lung disease?	We recommend that an opinion of a multidisciplinary team should be taken into account in the diagnostics of fibrotic interstitial lung disease.	Very low	Strong
	5	Should a patient with an interstitial lung disease other than IPF who does not meet the criteria for the progressive fibrosis phenotype be monitored for progression?	We recommend that a patient with an interstitial lung disease other than IPF who does not meet the criteria for the progressive fibrosis phenotype should be monitored for progression.	Very low	Strong
	6	Should patients with systemic autoimmune diseases be monitored regularly for signs of interstitial lung disease?	We recommend that patients with systemic autoimmune diseases should be regularly monitored for signs of interstitial lung disease.	Very low	Strong
TREATMENT	1	Should patients with an ILD in the course of systemic autoimmune diseases be managed by a multidisciplinary team?	We recommend that an opinion of a multidisciplinary team should be considered in the management of patients with interstitial lung disease in the course of systemic autoimmune diseases.	Very low	Strong
	2	Should a patient with a progressive fibrosing interstitial lung disease (PF-ILD) other than IPF be treated with first-line therapy dedicated to the diagnosed underlying disease?	We suggest that a patient with a progressive fibrosing interstitial lung disease (PF-ILD) other than IPF should be treated with first-line therapy dedicated to the diagnosed underlying disease.	Very low	Conditional
	3	In a patient with a progressive fibrosing interstitial lung disease (PF-ILD) other than IPF, should anti-fibrotic therapy with nintedanib be used in the event of ineffectiveness of the therapy recommended for the treatment of the underlying disease?	We recommend that in a patient with a progressive fibrosing interstitial lung disease (PF-ILD) other than IPF, anti-fibrotic therapy with nintedanib should be used in the event of ineffectiveness of the therapy recommended for the treatment of the underlying disease.	Low	Strong
	4	In a patient with a progressive fibrosing interstitial lung disease (PF-ILD) other than IPF, should anti-fibrotic therapy with pirfenidone be used in the event of ineffectiveness of the therapy recommended for the treatment of the underlying disease?	No recommendations were made for or against the use of anti-fibrotic therapy with pirfenidone if treatment of the underlying disease has failed in a patient with a progressive fibrosing interstitial lung disease (PF-ILD) other than IPF.	Very low	Not issued
	5	Is it possible to use an anti-fibrotic agent as a first-choice therapy (without the need for previous immunomodulatory treatment) in certain clinical situations (UIP or fibrotic NSIP pattern)?	We suggest using an anti-fibrotic agent as the first-choice treatment in certain clinical situations.	Very low	Conditional
	6	Can a patient with a progressive fibrosing interstitial lung disease (PF-ILD) other than IPF receive simultaneous treatment with a disease-modifying drug and anti-fibrotic therapy?	We suggest that in a patient with a progressive fibrosing interstitial lung disease (PF-ILD) other than IPF, one should consider simultaneous treatment with a disease-modifying drug and anti-fibrotic therapy.	Very low	Conditional
	7	Should progression noted during treatment with an anti-fibrotic agent in a patient with a progressive fibrosing interstitial lung disease (PF-ILD) other than IPF be a reason for discontinuation of anti-fibrotic therapy?	No recommendations were made for or against the termination of anti-fibrotic therapy in the case of noted progression during treatment of a progressive fibrosing interstitial lung disease (PF-ILD) other than IPF.	Very low	Not issued
	8	Should the same principles of non-pharmacological and palliative treatment and eligibility for lung transplantation be applied in a patient with an interstitial lung disease other than IPF with progressive fibrosis as in a patient with IPF?	We recommend that the same principles of non-pharmacological and palliative treatment and eligibility for lung transplantation should be applied in a patient with an interstitial lung disease other than IPF with progressive fibrosis as in a patient with IPF.	Very low	Strong