Background: Fibrosing, non-idiopathic pulmonary fibrosis (non-IPF) interstitial lung diseases (fILDs) are a\nheterogeneous group of diseases characterized by a different amount of inflammation and fibrosis. Therapy is\ncurrently based on corticosteroids and/or immunomodulators. However, response to these therapies is highly\nvariable, sometimes without meaningful improvement, especially in more fibrosing forms. Pirfenidone and\nnintedanib have recently demonstrated to reduce functional decline in patients with IPF. However, their antifibrotic\nmechanism makes these two drugs an interesting approach for treatment of fibrosing ILDs other than IPF.\nObjectives: We here report our experience with antifibrotic drugs in fibrosing non-IPF ILDs patients having a\nprogressive phenotype during immunosuppressive therapy.\nMethods: Patients with a multidisciplinary team diagnosis of fibrosing non-IPF ILDs experiencing a progressive\nphenotype during treatment with corticosteroids and/or immunomodulators between October-2014 and January-\n2018 at our tertiary referral Center for ILDs were retrospectively analyzed. Antifibrotic therapy was administered after\napplication with the respective health insurance company and after consent by the patient. Pulmonary-functiontests\nand follow-up visits were performed every ..........................
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