Negative outcome of prednisone in possible idiopathic pulmonary fibrosis

Background The diagnostic classification “possible idiopathic pulmonary fibrosis (IPF)” is characterised by an inconsistent usual interstitial pneumonia (UIP) pattern on HRCT-scan and a UIP pattern in surgical lung biopsy. Therapeutic management in patients with “possible IPF” is challenging. The clinician must choose between either immunomodulatory agents or anti-fibrotic agents, but evidence is lacking. Methods A multi-centre cohort of 59 patients with “possible IPF” treated with prednisone were retrospectively analysed. Prednisone starting dose was 0.5 mg/kg/day and tapered to 0.15 mg/day/kg in six months. Patient demographics and serious adverse events (SAEs), defined as death and hospital admissions, were collected. Forced vital capacity (FVC) before start of therapy, baseline (start of therapy) and six months after start of therapy were evaluated. Results In 59 prednisone treated “possible IPF” patients, 22% had a SAE: twelve in the first three months on prednisone >0.3mg/kg/day and two during the last three months on 5% decrease or death within six months from baseline. Six former smoking patients with an additional histopathological desquamative interstitial pneumonia (DIP) component besides UIP, were responders with a mean increase of 6% FVC. Conclusions Patients with “possible IPF” demonstrated accelerated FVC decline and high incidence of SAEs during high dosed prednisone treatment. Presence of concomitant DIP histopathological pattern is associated with responsiveness to prednisone treatment.