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What is it?
Very little is known at this time about Pulmonary Fibrosis. It is mostly seen in Westies, although Scotties and Cairns do develop it. Idiopathic Pulmonary Fibrosis, also known as Westie Lung Disease, is a scarring and fibrosing of the alveoli and interstitium (air sacs and connective tissue) of the lungs. The scarring may be the result of chronic inflammation of the alveoli, and can replace much of the normal structure of the lungs. It is suspected that this disease is similar to the same disease in people.
What causes it?
At this writing, we do not know, because research has yet to be done. Some veterinary researchers feel that there may be a link between Pulmonary Fibrosis, the immune systems and allergies. Most researchers agree that Westies are predisposed to Pulmonary Fibrosis, relative to other dog breeds.
What are the symptoms?
Symptoms may be caused by other factors or diseases, so caution should be exercised when diagnosing Pulmonary Fibrosis in the Westie. In a recent talk, Dr. Elizabeth Rozanski DVM from Tufts Veterinary School reported a number of signs to look for in diagnosing Pulmonary Fibrosis. They included the following, although not all symptoms need be present:
Rapid Shallow Breathing Pattern or Labored Breathing
Loss of Exercise Tolerance
Build up of Scar Tissue in the Lungs
Crackles in the Lungs
Dry Cough
These dogs can also develop pulmonary hypertension or can have an enlargement of the heart, due to breathing patterns.
How is it diagnosed?
Diseases of the interstitium of the lungs are difficult to identify because they usually require a lung biopsy for correct diagnosis. Lung biopsy is not simple in normal patients, and may be quite risky in affected dogs. Furthermore, many different lung diseases exist, and differentiation is difficult. Not enough samples have been taken, and pathologists vary in expertise in reading these samples. Radiographic changes in the lungs can be seen in X-rays, and the blood gases are abnormal, showing hypoxia, or lack of oxygen to the tissues. Tests that will easily diagnose Pulmonary Fibrosis and indicate the prognosis are needed. A specialized machine exists that can effectively screen people for the similar disease, and it is presently being tested on a control group of Westies.
What is the treatment?
Very few treatment options exist at this time. Once scarring occurs, there is little that can be done. Preventing respiratory tract infections, limiting exercise, and planned weight loss for overweight patients are all important. Drugs such as bronchial dilators (theophylline and terbutaline) may strengthen the respiratory muscles, but tend to lose effectiveness with time. Some dogs may benefit from controlled use of steroids, such as Prednisone and Interferon. Experimental usage of inhaled steroids has been tried in some cases. Cough suppressants can be helpful.
What is the prognosis?
The prognosis for affected dogs is very poor. Lung disease appears to affect older Westies, with the average age of onset being about nine years of age. Patients live 17-24 months after diagnosis, although some live less and some live longer. Our very limited data shows the average survival time after diagnosis to be about eighteen months, although some dogs have survived for more than three years. Very recently an international study group has been formed among veterinary respiratory specialists, which should help to speed research.
Is Westie Lung Disease?
We honestly do not know. It appears to be breed-specific, which could indicate a genetic basis. Pedigree studies have shown that some affected individuals have been related, but so little data has been collected for the general population that these observations should not be taken as proof that Pulmonary Fibrosis is inherited.
What can Westie owners do?
Owners of affected dogs can contribute significantly to the data collection and research programs. Make your veterinarian aware of the research being done. Monitor your own dogs for signs of respiratory disease. The earlier a diagnosis is made, the more likely treatment may be beneficial. | 
Pulmonary fibrosis/Westie Lung Disease 
