PT  - JOURNAL ARTICLE
AU  - Wim A. Wuyts
AU  - Alberto Cavazza
AU  - Giulio Rossi
AU  - Francesco Bonella
AU  - Nicola Sverzellati
AU  - Paolo Spagnolo
TI  - Differential diagnosis of usual interstitial pneumonia: when is it truly idiopathic?
AID  - 10.1183/09059180.00004914
DP  - 2014 Sep 01
TA  - European Respiratory Review
PG  - 308--319
VI  - 23
IP  - 133
4099  - http://err.ersjournals.com/content/23/133/308.short
4100  - http://err.ersjournals.com/content/23/133/308.full
SO  - EUROPEAN RESPIRATORY REVIEW2014 Sep 01; 23
AB  - Idiopathic pulmonary fibrosis (IPF), the most common and lethal of the idiopathic interstitial pneumonias, is defined by a radiological and/or pathological pattern of usual interstitial pneumonia (UIP). However, UIP is not synonymous with IPF as other clinical conditions may be associated with UIP, including chronic hypersensitivity pneumonitis, collagen vascular disease, drug toxicity, asbestosis, familial IPF and Hermansky–Pudlak syndrome. Differentiating IPF (“idiopathic UIP”) from conditions that mimic IPF (“secondary UIP”) has substantial therapeutic and prognostic implications. A number of radiological and histological clues may help distinguish IPF from other conditions with a UIP pattern of fibrosis, but their appreciation requires extensive expertise in interstitial lung disease as well as an integrated multidisciplinary approach involving pulmonologists, radiologists and pathologists. In addition, multidisciplinary discussions may decrease the time to initial IPF diagnosis and, thus, enable more timely management. This concept was strongly emphasised by the 2011 ATS/ERS/JRS/ALAT guidelines. This article highlights, with the aid of a clinical case, the difficulties in making a diagnosis of IPF in clinical practice. Yet, an accurate diagnosis is critical, particularly given the availability of drugs that may reduce the pace of functional decline and disease progression in IPF.