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Identification of Spontaneous Feline Idiopathic Pulmonary Fibrosis^*

Morphology and Ultrastructural Evidence for a Type II Pneumocyte Defect

^* From the Department of Pathobiology and Diagnostic Investigation (Drs. Williams and Patrick), College of Veterinary Medicine, Michigan State University, East Lansing, MI; Department of Microbiology, Pathology, and Parasitology (Dr. Malarkey), College of Veterinary Medicine, North Carolina State University, Raleigh, NC; Department of Veterinary Medicine and Surgery (Dr. Cohn), College of Veterinary Medicine, University of Missouri, Columbia, MO; Experimental Toxicology Division (Dr. Dye), National Health and Environmental Effects Laboratory, United States Environmental Protection Agency, Research Triangle Park, NC; and University of Michigan Department of Internal Medicine (Dr. Toews), Pulmonary and Critical Care Medicine, University of Michigan, Ann Arbor, MI.

Correspondence to: Kurt J. Williams, DVM, PhD, Department of Pathobiology and Diagnostic Investigation, 210 Food Safety and Toxicology Building, Michigan State University, East Lansing, MI 48824; e-mail: williamsk{at}dcpah.msu.edu

Study objectives: Idiopathic pulmonary fibrosis (IPF) is a poorly understood chronic respiratory disease of humans, which has no correlate in other animals. Understanding the role that inflammation, alveolar epithelial cells, and myofibroblasts play in the progression of the disease is controversial, and hampered by the lack of an animal model. We have identified spontaneous IPF in domestic cats and hypothesized that this newly identified disease shares the pathology of human IPF; further, this work provides data suggesting that the disease is related to a defect in type II pneumocyte biology.

Setting and subjects: Chronic respiratory disease with pathology consistent with usual interstitial pneumonia (UIP) spontaneously developed in 16 domestic cats.

Results: The histopathology of feline IPF consisted of the following: (1) interstitial fibrosis with fibroblast/myofibroblast foci, (2) honeycombing with alveolar epithelial metaplasia and type II pneumocyte hyperplasia, and (3) alveolar interstitial smooth-muscle metaplasia. Interstitial inflammation was not a prominent feature of the disease. -Smooth muscle actin-positive myofibroblasts were prominent in myofibroblast foci, beneath honeycomb and hyperplastic epithelium, and in alveolar septa away from the remodeling. Feline IPF type II pneumocyte ultrastructure is similar to a heritable form of human IPF, with abnormal cytoplasmic lamellar body-like inclusions.

Conclusions: We conclude the following: (1) chronic respiratory disease with clinical and pathology features of UIP/IPF occurs in the domestic cat; (2) as in human IPF, the type II pneumocyte and myofibroblasts are important cellular constituents of feline IPF; and (3) type II cell ultrastructure suggests feline IPF is a defect in the type II pneumocyte.

Key Words: animal model • cats • fibrosis • idiopathic pulmonary fibrosis • lung • type II pneumocytes

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