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Trial Objectives


Progressive Pulmonary Fibrosis, or PPF, is a rare lung disease that causes scarring in the lung over time. This causes difficulty breathing and worsened symptoms over time, including cough, fatigue or shortness of breath. Current treatments for PPF are in pill form. While they can slow the disease in some people, the high doses needed can cause side effects. Researchers in the MIST study want to know if the investigational study medication, an inhaled form of pirfenidone called AP01, can improve lung function and have fewer side effects.

Volunteers will be randomly assigned to receive oral pirfenidone or a placebo (a medication containing no active ingredient). At clinic visits, volunteers will receive physical examinations, have their lung function evaluated, and will receive questionnaires about their symptoms in the clinic and at home.

Who Can Participate


Adults with a diagnosis of progressive pulmonary fibrosis (not idiopathic pulmonary fibrosis), who meet spirometry test requirements and are not taking oral Esbriet or Pirfenidone.  

Age: 18-80 Gender: Any

Estimated Time Commitment


12 in person visits and 1 phone visit over 56 weeks (13 months), with twice a day nebulizer treatments.

Payment & Reimbursement


Payment: Provided

Travel Reimbursement: Available

Trial Contact


For more information, contact:

Elizabeth Del Real
303.398.1172

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Trial Location


National Jewish Main Campus, Denver, CO

Trial Sponsors


Avalyn Pharma, Inc.

Principal Investigators

Jeff Swigris

Jeff Swigris, DO, MS

Co-Investigators

  • Evans Fernández

    Evans Fernández, MD, MS

  • Tristan J. Huie

    Tristan J. Huie, MD

  • Michael P. Mohning

    Michael P. Mohning, MD

  • Joshua J. Solomon

    Joshua J. Solomon, MD

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