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Keywords:

  • Idiopathic pulmonary fibrosis;
  • Interstitial lung disease;
  • Respiratory disease;
  • SP-C;
  • West Highland White Terrier

Background: Canine pulmonary fibrosis (CPF) occurs most commonly in West Highland White Terriers. The differing incidences of CPF among dog breeds suggest that genetic factors contribute to its pathophysiology. Pulmonary fibrosis in humans is associated with mutations in the gene coding for lung surfactant protein C (SP-C) (SFTPC).

Hypothesis/Objectives: To investigate the histopathologic changes and SP-C composition and genetic structure in dogs with CPF.

Animals: Five dogs with PF, 2 dogs with other lung diseases, and 3 healthy dogs.

Methods: Lung tissue from dogs with clinically suspected CPF and 5 control cases was analyzed histopathologically. Bronchoalveolar lavage fluid (BALF) collected postmortem from 3 terriers with histopathologically confirmed pulmonary fibrosis and the 5 controls were analyzed by Western blots, and the exons of SFTPC were sequenced for 2 dogs with PF and 1 dog with other lung disease.

Results: SP-C could not be detected in BALF of 1 dog with PF, although SP-B was present. A mutation was detected in SFTPC exon 5 of this dog. From 2 dogs with PF and in all 5 control dogs SP-B and SP-C were detected in BALF.

Conclusions: Taken together, the results indicate that canine and human lung fibrosis share histopathologic features and that analysis of SP-C and its gene in a larger set of dogs with PF is warranted.