Unfavourable outcome of glucocorticoid treatment in suspected idiopathic pulmonary fibrosis.

Background and objectiveThe diagnostic classification of possible idiopathic pulmonary fibrosis (posIPF)' is characterized by a radiological pattern of inconsistent usual interstitial pneumonia (UIP) on high-resolution computed tomography (HRCT) scan and a UIP pattern in surgical lung biopsy (SLB). The evidence base to guide treatment for patients with posIPF is lacking; the clinician must choose between observation, treatment with immunomodulatory agents or anti-fibrotic agents. MethodsTo evaluate outcomes of immunomodulatory treatment, a multicentre cohort of 59 posIPF patients treated with prednisone was analysed retrospectively. Prednisone starting dose was 0.5mg/kg/day and tapered to 0.15mg/day/kg over 6months. Outcome measures were forced vital capacity (FVC) and serious adverse events (SAE), defined as death or hospital admissions. ResultsThe majority of prednisone-treated posIPF patients were non-responders (68%) with a decrease in FVC >5% or death within 6months from baseline; 90% of patients with radiographical presence of honeycombing were non-responders. In contrast, six out of seven patients with focal desquamative interstitial pneumonia-like reaction in the SLB who had stopped smoking for <5years ago were responders to prednisone, demonstrating <5% FVC decline. The mean decline of FVC was 8.7% (95% CI: 3.1-14.3%) before treatment and 20% (95% CI: 9.4-31.1%) after treatment (P=0.018) in the 32 patients with available FVC data. Twelve SAE occurred within the first 3months on prednisone (at dosage >0.3mg/kg/day), including five deaths. ConclusionPatients with posIPF demonstrated an accelerated FVC decline and a substantial number of SAE on steroid therapy.