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Unfavourable outcome of glucocorticoid treatment in suspected idiopathic pulmonary fibrosis
Author(s) -
Wiertz Ivo A.,
Wuyts Wim A.,
van Moorsel Coline H.M.,
Vorselaars Adriane D.M.,
van Es Hendrik W.,
van Oosterhout Matthijs F.M.,
Grutters Jan C.
Publication year - 2018
Publication title -
respirology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.857
H-Index - 85
eISSN - 1440-1843
pISSN - 1323-7799
DOI - 10.1111/resp.13230
Subject(s) - medicine , idiopathic pulmonary fibrosis , glucocorticoid , pulmonary fibrosis , intensive care medicine , fibrosis , lung
Background and objective The 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. Methods To evaluate outcomes of immunomodulatory treatment, a multicentre cohort of 59 posIPF patients treated with prednisone was analysed retrospectively. Prednisone starting dose was 0.5 mg/kg/day and tapered to 0.15 mg/day/kg over 6 months. Outcome measures were forced vital capacity (FVC) and serious adverse events (SAE), defined as death or hospital admissions. Results The majority of prednisone‐treated posIPF patients were non‐responders (68%) with a decrease in FVC >5% or death within 6 months 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 <5 years 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 3 months on prednisone (at dosage >0.3 mg/kg/day), including five deaths. Conclusion Patients with posIPF demonstrated an accelerated FVC decline and a substantial number of SAE on steroid therapy.