Introduction Usual Interstitial Pneumonitis (UIP) may be caused by asbestos exposure (asbestosis), collagen vascular diseases (CVD) or agents causing hypersensitivity pneumonitis (HP), or may be idiopathic (IPF). Referrals to our ILD MDT have increased since the limited availability of pirfenidone which is only prescribable for IPF with a FVC 50–80% predicted. We have reviewed presentation in 2014 to identify patients suitable for Pirfenidone prescription.
Methods Our hospital provides a regional service for Pirfenidone prescription. All patients with a MDT diagnosis of definite UIP were included. A standard proforma requested information on exposures, CVD and antibodies relevant to CVD and HP. If there were no relevant exposures and CVD was excluded, a diagnosis of IPF was made. Asbestosis, HP and CVD associated UIP were diagnosed when the relevant information supported this, unspecified UIP was diagnosed in the absence of specific information.
Results 202/546 referrals in 2014 were judged to have definite UIP after consideration by a fully constituted ILD MDT including histopathologists, radiologists, clinicians and CNS’. After exclusion of 22 with asbestosis, 11 with CVD associated UIP, 17 with CPFE and 17 with HP associated UIP, 51 with IPF and 84 with unspecified UIP remained for consideration of Pirfenidone treatment. Only 64/135 suitable patients had a FVC 50–80% predicted (Figure 1).
Conclusions The decisions of an ILD MDT are limited by the completeness of investigation. We found causes for 50/200 patients with UIP. Pirfenidone was not prescribable for 53% of otherwise suitable patients. If the FVC limit was raised to 90% 32% would still be excluded, including 2 who died of their disease within 12 months. The FVC is often preserved even in terminal IPF.