Differential diagnosis of usual interstitial pneumonia: When is it truly idiopathic?

Wim A. Wuyts, Alberto Cavazza, Giulio Rossi, Francesco Bonella, Nicola Sverzellati, Paolo Spagnolo

Research output: Contribution to journalArticle

43 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)308-319
Number of pages12
JournalEuropean Respiratory Review
Volume23
Issue number133
DOIs
Publication statusPublished - 2014

Fingerprint

Idiopathic Pulmonary Fibrosis
Differential Diagnosis
Idiopathic Interstitial Pneumonias
Hermanski-Pudlak Syndrome
Asbestosis
Extrinsic Allergic Alveolitis
Collagen Diseases
Interstitial Lung Diseases
Drug-Related Side Effects and Adverse Reactions
Vascular Diseases

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Medicine(all)

Cite this

Differential diagnosis of usual interstitial pneumonia : When is it truly idiopathic? / Wuyts, Wim A.; Cavazza, Alberto; Rossi, Giulio; Bonella, Francesco; Sverzellati, Nicola; Spagnolo, Paolo.

In: European Respiratory Review, Vol. 23, No. 133, 2014, p. 308-319.

Research output: Contribution to journalArticle

Wuyts, Wim A. ; Cavazza, Alberto ; Rossi, Giulio ; Bonella, Francesco ; Sverzellati, Nicola ; Spagnolo, Paolo. / Differential diagnosis of usual interstitial pneumonia : When is it truly idiopathic?. In: European Respiratory Review. 2014 ; Vol. 23, No. 133. pp. 308-319.
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