Idiopathic pulmonary fibrosis in West Highland white terriers: An update.

Canine idiopathic pulmonary fibrosis (CIPF) affects middle-aged to older dogs of a single breed, mainly the West Highland white terrier (WHWT), which is suggestive of a genetic predisposition. CIPF causes exercise intolerance, restrictive dyspnoea and coughing. Coarse crackles are heard on thoracic auscultation. Abnormal blood gas parameters and a shortened '6-min-walking test' distance are common; secondarily induced pulmonary hypertension and/or airway collapse are frequent. These features of CIPF mimic those of idiopathic pulmonary fibrosis (IPF) in humans and therefore identify CIPF as a possible spontaneously arising model for study of human IPF. However, computed tomographic and histopathological findings of CIPF are not identical to those of human IPF. As in human IPF, the aetiology of CIPF is not yet fully elucidated. There are no curative treatments and the prognosis is poor. This paper reviews advances in understanding of the clinical description and natural history of CIPF, the investigation of biomarkers and the exploration of possible aetiologies and mechanistic hypotheses.