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.

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Critical Care

Compendium

Chris is an Intensivist and ECMO specialist at the Alfred ICU in Melbourne. He is also the Innovation Lead for the Australian Centre for Health Innovation at Alfred Health, a Clinical Adjunct Associate Professor at Monash University, and the Chair of the Australian and New Zealand Intensive Care Society (ANZICS) Education Committee. He is a co-founder of the Australia and New Zealand Clinician Educator Network (ANZCEN) and is the Lead for the ANZCEN Clinician Educator Incubator programme. He is on the Board of Directors for the Intensive Care Foundation and is a First Part Examiner for the College of Intensive Care Medicine. He is an internationally recognised Clinician Educator with a passion for helping clinicians learn and for improving the clinical performance of individuals and collectives.

After finishing his medical degree at the University of Auckland, he continued post-graduate training in New Zealand as well as Australia’s Northern Territory, Perth and Melbourne. He has completed fellowship training in both intensive care medicine and emergency medicine, as well as post-graduate training in biochemistry, clinical toxicology, clinical epidemiology, and health professional education.

He is actively involved in in using translational simulation to improve patient care and the design of processes and systems at Alfred Health. He coordinates the Alfred ICU’s education and simulation programmes and runs the unit’s education website, INTENSIVE.  He created the ‘Critically Ill Airway’ course and teaches on numerous courses around the world. He is one of the founders of the FOAM movement (Free Open-Access Medical education) and is co-creator of litfl.com, the RAGE podcast, the Resuscitology course, and the SMACC conference.

His one great achievement is being the father of two amazing children.

On Twitter, he is @precordialthump.

| INTENSIVE | RAGE | Resuscitology | SMACC

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