Idiopathic Pulmonary Fibrosis

Reviewed and revised 17 September 2014

OVERVIEW

Idiopathic pulmonary fibrosis (IPF) is the most common of the 7 types of idiopathic interstitial pneumonia (IIP)

• it is a dreadful, chronic, and irreversibly progressive fibrosing disease leading to death in all patients affected (median survival time 2 to 4 years)
• By definition, IPF is the term for the clinical syndrome associated with the morphological pattern of usual interstitial pneumonia (UIP)

CLINICAL FEATURES

History

• usually >50 years old
• progressively worsening dyspnea and nonproductive cough
• history of cigarette smoking seems to be a risk factor

Examination

• clubbing
• fine inspiratory crackles

INVESTIGATIONS

CXR

• normal early in the course
• subpleural reticular opacities that increase from the apex to the bases of the lungs

HRCT

• UIP should be considered in patients who present with low lung volumes, subpleural reticular opacities (thickening of intralobular and interlobular septae), macrocystic honeycombing, and traction bronchiectasis, the extent of which increases from the apex to the bases of the lungs
• a characteristic HRCT is diagnostic

Lung biopsy

• biopsy multiple sites if atypical imaging findings, such as extensive ground-glass opacities, nodules, consolidation, or a predominantly peri-bronchovascular distribution

MANAGEMENT

• Admission to ICU is a clinical and ethical challenge because of the extremely poor outcome
• Exacerbation requires rapid decisions about when and whether to initiate mechanical support
• High-dose corticosteroids +/- immunosuppressants are ineffective and represent the coup de grace for these patients
• supportive care and ‘seek and treat’ the ‘underlying cause’ are the only treatment options
• Patients should be considered candidates for lung transplantation early after diagnosis – transplantation in the ICU setting may present insurmountable difficulties

References and Links

Journal articles

• Arakawa H, Honma K. Honeycomb lung: history and current concepts. AJR Am J Roentgenol. 2011 Apr;196(4):773-82. doi: 10.2214/AJR.10.4873. PMID: 21427324. [Free Full Text]
• Mueller-Mang C, Grosse C, Schmid K, Stiebellehner L, Bankier AA. What every radiologist should know about idiopathic interstitial pneumonias. Radiographics. 2007 May-Jun;27(3):595-615. PMID: 17495281. [Free Full Text]
• Palmucci S, Roccasalva F, Puglisi S, Torrisi SE, Vindigni V, Mauro LA, Ettorre GC, Piccoli M, Vancheri C. Clinical and radiological features of idiopathic interstitial pneumonias (IIPs): a pictorial review. Insights Imaging. 2014 Jun;5(3):347-64. PMC4035488.
• Papiris SA, Manali ED, Kolilekas L, Kagouridis K, Triantafillidou C, Tsangaris I, Roussos C. Clinical review: idiopathic pulmonary fibrosis acute exacerbations–unravelling Ariadne’s thread. Crit Care. 2010;14(6):246. MC3220036.