SAT0593 Progressive fibrosing interstitial lung disease (PF-ILD) in patients with autoimmune rheumatic diseases
Authors: A. Fischer, M. Wijsenbeek, M. Kreuter, B. Mounir, L. Zouad-Lejour, V. Acciai, C.D. Wells, M. Quaresma, C. Denton
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Interstitial lung disease (ILD) associated with autoimmune conditions is among the most challenging aspect of care for patients with rheumatic diseases. While idiopathic pulmonary fibrosis (IPF) is the classic fibrosing ILD, some patients with differing clinical ILD diagnoses including autoimmune associated-ILD can develop a progressive fibrosing phenotype. This phenotype is characterised by progressive pulmonary fibrosis, worsening respiratory symptoms, declining lung function, resistance to immunomodulatory therapies and early mortality. There are limited data available on current practice in diagnosis, management and treatment of PF-ILD in patients with autoimmune rheumatic diseases. <h3>Objectives</h3> To investigate the patient journey in patients with autoimmune rheumatic diseases and PF-ILD. <h3>Methods</h3> Twenty-two ILD experts from Germany, Japan, UK and the US participated in a 1 hour interview. Physicians who spend ≥75% of their professional time managing patients and in whose caseload ≥10 patients had PF-ILD in the past year completed an online survey. Physicians (243 pulmonologists, 203 rheumatologists, 40 internal medicine physicians) from the US, Japan, Germany, France, Italy, Spain and UK participated. ILD prevalence and treatment patterns in the US were analysed based on insurance claims from patients with ≥2 claims with an ILD diagnosis between 2014 and 2016 (ICD-9/10 codes) and ≥1 visit to a pulmonologist in the 3 years. <h3>Results</h3> Analysis of US claims identified 21 592 patients with autoimmune ILD. Rheumatoid arthritis ILD (RA-ILD) and systemic sclerosis ILD (SSc-ILD) were the most prevalent ILDs across all autoimmune rheumatic diseases. The course of the patient journey is summarised (figure 1). Most patients with autoimmune-associated ILD initially present to a rheumatologist or a primary care doctor. Both pulmonologists and rheumatologists play a key role in detection and diagnosis of ILD; however US claims data suggest that the former are more likely to make the diagnosis. Although there is significant inter-patient variation, diagnosis of ILD is estimated to take approximately 9–12 months after symptoms develop. In general ILD is diagnosed earlier in SSc. Management of patients with autoimmune-associated ILD is typically multidisciplinary, involving both a rheumatologist and a pulmonologist. The physician survey suggested that 24%&gt;31% of patients with autoimmune-associated ILD develop PF-ILD; detection can take up to 1 year. The majority of physicians use corticosteroids as a first line treatment across autoimmune rheumatic diseases; cyclophosphamide and mycophenolate mofetil were the second and third first line option for SSc-ILD. Physicians estimate the total disease course in patients with autoimmune-associated PF-ILD to be approximately 5–7 years. <h3>Conclusions</h3> Physicians who manage patients with autoimmune diseases estimate that 24%&gt;31% develop PF-ILD. Delayed referral to a pulmonologist or rheumatologist is likely to delay diagnosis and management of PF-ILD. Life expectancy for these patients is believed to be similar to patients with IPF. There is an unmet need for treatments that slow or stabilise disease progression of PF-ILD. <h3>Disclosure of Interest</h3> A. Fischer Consultant for: Boehringer Ingelheim, M. Wijsenbeek: None declared, M. Kreuter: None declared, B. Mounir: None declared, L. Zouad-Lejour: None declared, V. Acciai: None declared, C. Wells: None declared, M. Quaresma: None declared, C. Denton: None declared