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4. Idiopathic Pulmonary Fibrosis (IPF)

                  Recruiting patients

           IPF is an important disease in humans and it occurs with a higher than normal frequency in West Highland White terriers (20). The cause of the disease is unknown. The increased fibrosis (similar to that seen in the middle panel below) “clogs” the lungs so that although the dog breathes with difficulty, there is very little air exchanged. Substantial evidence in laboratory animals document that MSCs are beneficial in treating IPF models (21). In the figures below, the left panel is a cross section of normal lungs. Notice how “open” it appears with lots of white air-exchange spaces between the pink/purple lung tissue. In the center panel, the animal has pulmonary fibrosis-much less “white” and a lot more pink/purple-due to treatment with a specific chemical that induces fibrosis. Note how solid looking and dense

the lung tissue is. In the right panel, an animal that has been treated with the same chemical and then treated with MSCs shortly after receiving the lung fibrosing chemical. Note the lungs are much less dense than the center panel but not quite as good as the control panel on the left. This suggests that treatment with MSCs may delay or inhibit the fibrosis that occurs following chemical insult. It is not known what the effects of MSCs would be on naturally occurring IPF but it is an intriguing concept that justifies further clinical trials.

Ortiz et al. Proc. Natl. Acad. Sci. 100: 8407-11, 2003

Ongoing Clinical Trials 9