Canis ISSN: 2398-2942

Idiopathic pulmonary fibrosis

Synonym(s): Chronic fibrosing alveolitis, Cryptogenic pulmonary fibrosis, Chronic pulmonary disease of West Highland White Terriers, Interstitial lung disease in West Highland Terriers, Chronic idiopathic pulmonary fibrosis, IPF

Contributor(s): Chris Little, Rachel Blake

Introduction

  • Uncommon condition which has only briefly been described in the veterinary literature. Almost certainly under-reported.
  • Cause: unknown, apparently genetic.
  • Signs: dyspnea, cyanosis, hypoxia.
  • Diagnosis: lung biopsy. Presumptive diagnosis by breed, radiography/thoracic ultrasonography, blood gas analysis, and exclusion of other causes of chronic thoracic pathology.
  • Treatment: symptomatic only.
  • Prognosis: poor.

Pathogenesis

Pathophysiology

  • Poorly understood. Suspect genetic and environmental factors cause repeated insult to lung parenchyma followed by abnormal healing resulting in migration and proliferation of fibroblasts, activation to myofibroblasts and extracellular matrix (ECM) production.
  • Expansion of the interstitial space of alveolar septae by ECM consisting of a mixture of type-1 and type-111 collagens resulting in impaired gas exchange.
  • Can develop secondary infection, pulmonary hypertension and airway collapse.

Timecourse

  • Slowly progressive over months.

Diagnosis

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Treatment

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Outcomes

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Further Reading

Publications

Refereed papers

  • Recent references from PubMed and VetMedResource.
  • Clercx C, Fastres A, Roles E (2018) Idiopathic pulmonary fibrosis in West Highland white terriers: An update. Vet J 242, 53-58 PubMed.
  • Thierry F, Handel I, Hammond G, King L G, Corcoran B M, Schwarz T (2017) Further characterization of computed tomographic and clinical features for staging and prognosis of idiopathic pulmonary fibrosis in West Highland white terriers. Vet Radiol Ultrasound 58, 381-388 PubMed.
  • Heikkilä-Laurila H P, Rajamäki M M (2014) Idiopathic pulmonary fibrosis in West Highland White Terriers. Vet Clin North Am Small Anim Pract 44, 129-142 PubMed
  • Thierry F, Handel I, Hammond G, King L G, Corcoran B M, Schwarz T (2017) Further characterization of computed tomographic and clinical features for staging and prognosis of idiopathic pulmonary fibrosis in West Highland white terriers. Vet Radiol Ultrasound 58, 381-388 PubMed.
  • Norris A J, Naydan D K, Wilson D W (2005) Interstitial lung disease in West Highland White Terriers. Vet Pathol 42 (1), 35-41 PubMed.
  • Corcoran B M, Cobb M, Martin W S, Dukes-McEwan et al (1999) Chronic pulmonary disease in West Highland White Terriers. Vet Rec 144 (22), 611-616 PubMed.
  • Corcoran B M, Dukes-McEwan J, Rhind S & French A (1999) Idiopathic pulmonary fibrosis in a Staffordshire bullterrier with hypothyroidism. JSAP 40 (4), 185-188 PubMed.

Other sources of information

  • Corcoran B M (2004) Idiopathic Pulmonary Fibrosis. In: Textbook of Respiratory Disease in Dogs and Cats. Ed Lesley G King, Saunders/Elsevier. Chapter 77, pp 581-586.


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