Rapidly non-ipf progressive fibrosing interstitial lung disease: a phenotype with an ipf-like behavior.

BACKGROUND: A subgroup of patients with fibrotic ILD experience progression and several risk factors for ILD progression have been reported, such as male sex, older age, lower baseline pulmonary function, and a radiological or pathological pattern of usual interstitial pneumonia. OBJECTIVE: To describe a possible new phenotype of rapidly non IPF progressive fibrosing with an IPF-like outcome. METHODS: Three previously fit and well patients who developed a rapidly progressive ILD and died within 6 to 7 months from the initial development of respiratory symptoms. RESULTS: Unlike what is currently known, our patients developed a severe fibrosing ILD with an IPF-like outcome despite a) being younger than the average patient with IPF, b) having received a non-IPF MDT diagnosis, c) having a non-UIP pattern on HRCT. Moreover and similarly to IPF, they failed to respond to immunosuppressive treatment which is the preferred treatment option in these cases. CONCLUSION: .