Pulmonary hypertension, high pressure in the blood vessels of the lungs, is a common and serious complication of chronic lung diseases, including idiopathic pulmonary fibrosis (IPF).

IPF is a type of interstitial lung disease, which causes progressive scarring, leading to the lung tissue thickening and stiffening. In particular, it affects the interstitium, or the area between the alveoli where gas exchange takes place in the lungs, and the blood vessels. The scar tissue reduces lung capacity and restricts gas exchange, resulting in less oxygen being transferred to the blood.

The exact mechanism through which IPF can cause pulmonary hypertension is not well understood, but it is thought that the scarring restricts blood vessels, impeding blood flow and making it more difficult for the heart to pump blood through the lungs. Another theory is that the damage response and resulting scar tissue reactivates pathways involved in lung development and triggers vascular remodeling. This can change the shape of the blood vessels, such as causing them to narrow and restrict blood flow.

IPF is a debilitating and fatal condition, and while current therapies can help to improve survival and quality of life, there is  no known cure. However, research into promising approaches like stem cell therapy is ongoing.

What is stem cell therapy?

The body is made up of many different types of specialized cells that fulfill specific functions.

Once a cell is specialized, it generally cannot change and will only divide to produce similar cells. Stem cells are different in that they have the potential to develop and specialize into multiple different types of cells. Depending on where the stem cell originates, the type and range of cells it can develop into will differ.

By administering stem cells to IPF patients, it may be possible to generate the new lung tissue necessary for effective gas exchange.

Mesenchymal stem cells (MSCs) are now being investigated to treat IPF due to their ability to potentially reduce inflammation in the lungs. Damage caused by inflammation can lead to scarring in the lungs, so reducing lung inflammation may be able to lessen further scarring.

MSCs can be obtained from various tissues, including the adult bone marrow, umbilical cord blood, and the placenta.

Stem cell therapy in clinical trials for IPF

Both ongoing and completed clinical trials have investigated stem cell therapy in IPF patients.

A proof-of-concept, open-label Phase 1 clinical trial (NCT01385644), carried out at The Prince Charles Hospital in Brisbane, Australia, aimed to determine whether MSC therapy was safe and feasible. The study enrolled eight IPF patients, who received either a high or low concentration of MSCs. Trial results, published in the journal Respirology, suggested that the therapy is feasible and both doses were well-tolerated, with only minor and short-term adverse effects. At six months after treatment, the patients showed no worsening in their condition.

A Phase 1 randomized and blinded, placebo-controlled clinical trial, called AETHER (NCT02013700), enrolled 25 IPF patients at the Interdisciplinary Stem Cell Institute at the University of Miami. The trial aimed to assess the safety of MSC therapy, and gain a preliminary idea of its efficacy over a 60-week period. Participants were randomly assigned a single dose of one of three concentrations of MSCs or to a placebo. The trial is ongoing, but no longer recruiting participants.

Results from nine patients, who were treated and monitored for the full 60 weeks, have been published in the scientific journal, Chest. These results did not include a placebo arm. The treatment appeared to be well-tolerated and no serious side effects due to the therapy were recorded. However two patients died for reasons attributed to disease progression.

The only study currently recruiting patients is a Phase 1/2 clinical trial (NCT02745184) taking place at two sites in China. Researchers intend to isolate lung stem cells from the patient’s own bronchi (the large tubes of the lungs) and expand them in the laboratory. About 20 enrolled patients will then receive a single injection of their cultured lung stem cells directly into the area affected by IPF.

To assess the safety of the treatment, patients will be monitored for side effects for up to one year. Efficacy will be measured by  changes in several lung function and exercise ability tests. The primary test, at 48 weeks post-treatment, will be the change in forced vital capacity (FVC), or the volume of air that can be quickly exhaled after a deep breath. The trial is expected to finish in December 2018.

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Pulmonary Hypertension News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.

30 comments

  1. William ("Bill") Skewes says:

    I also have been diagnosed (March 9, 2017) with IPF and would gladly participate in any clinical trials. I will so advise my pulmonologist when I meet with her on March 23. I turned 72 years of age in February. I am waiting for approval to take pirfenidone which I am told will be approved this week, subject to some required tests. I live in the metro Denver, Colorado area

  2. Lee Seidman says:

    I have NSIP and would like to participate in clinical trials for stem cell research….South Florida resident

  3. Dora Guzman says:

    I am 68 yrs old living with oxygen walking around in the house with a breathing tube, sleeping with oxygen 24/7 totally dependent on oxygen. My quality of life has diminished. All I do is sit around the house read, watch television and that’s about it. I used to work and had hobbies. Will you please help me. I feel that my time on earth is near.

  4. Jamie Robinson says:

    Does a biopsy for this condition, to rule out other possibilities, worsen inflammation? My brother is scheduled for one this month. He’s in good health otherwise – still very active – went skiing out west the day after receiving diagnosis. (Breckenridge probably not the ideal spot for those with this condition – such high altitude affected nearly everyone in his group of 15 the 1st day.) If poor wound healing can lead to this condition, is a biopsy likely to worsen his condition? Also, can they eliminate cancer while he’s having the biopsy, then insert stem cells?

    • David Patterson says:

      My IPF Doctor at U of Mich. does not recommend or do biopsies. The probability of further damage outweighs any clinical information. I am on Ofev.

      • austin norman says:

        I agree…I’ve had stems (mesenchymal cells) 40 million transplanted by I.V. in the arm. The cells collect in the lungs very easily. I couldn’t walk 100 ft. now I routinely walk & hike 2-3 miles in an open space park with hills every day with my dog. I still use Advair inhaler twice a day. A side effect is that my allergies have disappeared and my appetite & taste buds & olfactory sense of smell & taste have improved greatly. I will need several more I.V.’s for 95% efficacy. It’s only been 112 days. What’s 6 months going to be?

        • Marilyn says:

          Hello Norman🤗
          It is sooo exciting to know that someone actually has made it through whatever therapy that is available to date!!! Congratulations on your longevity and quality of life!
          You have to be ecstatic!
          My question to you is what path did you take to land in the right place of research and where are you located today AND the hospital and Surgeons that extended your life. I would gladly move to wherever to have my life restored!
          Just left Rheumotologists on yesterday and he wants me to start keeping the oxygen on ALL DAY EVERY DAY!!! Before I started having these frequent bouts of shortness of breath, I was ONLY on the oxygen at night.
          My ONLY DAUGHTER is getting married next year and I would love to be able to walk down the isle without a tank on my side!!!!
          Any direction for help would be GREATLY APPRECIATED🙏🏽
          Marilyn in Dallas Texas 😇

  5. Marcus says:

    46 yo male. No current complications. Just short of breath Looking for stem cell treatment for familieral IPF.
    Have read recently published research from University of North Carolina. Looking for similar in Australia

  6. Earl Robinson says:

    I have been diagnosed with IPF after having a persistent dry cough for a couple of years. My age, 79, still active but have cough and shortness of breath with exertion. Human clinical trials or stem cell treatment are appealing, currently no oxygen, on OFEV at this time.

  7. James A Ryals says:

    I’m 67 years old was diagnosed with IPF in November 2016 I was taking ESBREIT for a year I would love totry stem cells I love to play golf but dont have enought energey or Breath to play. I get short of breath minor excertion I live in jacksonville Fl 32223

    • Owen Wadleigh says:

      Mr. Ryals, God bless you that you have the resources to afford the $27,000 + a month for Esbreit. I have just been diagnosed with IPF and I am 57. After being told the price for the drug to extend life a little it just made me mad. Then I was told I couldn’t be considered for a lung transplant because I couldn’t afford my share of the hospital bill for surgery after my insurance paid their part and I couldn’t afford the anti-rejection meds for the rest of my life. Living in the USA is just for the healthy and wealthy it seems. I live in Ocala Florida 34472

  8. PATRICIA FAY ROGNER says:

    ”DEAR SIR/MADAM: I AM A RETIRED FNP AGE 84 DIAGNOISED WITH IPF JUNE 2017. HISTORY OF DRY, NONPRODUCTIVE COUGH AND SHORTNESS OF BREATH WITH EXERTION. CT SCAN CONFIRMED
    FIBROSIS CHANGES AS NOTED ON CHEST FILMS STARTED IN 2013. I AM TAKING OFEV 100 MG BID STARTED JUNE 2017.DOING WELL BUT WOULD CONSIDER STEM CELL CLINICAL STUDY IN VIRGINIA. I AM ON CPAP NO OXYGEN YET.
    PLEASE LET ME KNOW IF I MAY BE A CANDIDATE FOR A STEM CELL STUDY. P. FAY

  9. Mahmoud A Shaalan;MD says:

    I am 83 Y old MD with pulmonary fibrosis confirmed with multiple CT scans & is on 24 hour O2 (for 6 months). Had open lung biopsy at Cleveland clinic. One week ago (4/22/18) I was given 4 cc IV stem cells . So far doing OK. What is your openion & what to expect ANY clinical changes (Good or bad). THANKS.

  10. BR wagoner says:

    I had weaknesss and an ongoing nonproductive coughing approx 3-4 months with episodes of weakness and no energy well before. Finally got the shocking diagnosis of pulmonary fibrosis and am still reeling from it. No pulmonologist appt yet im supposed to get a call. I would really like to be in a stem cell trial. There are two clinics offering treatment with stem cells; i’ll try anything but cost is not listed

  11. W. Adams says:

    I was diagnosed with IPF two months ago. I am 73 and not using O2. Starting Esbriet next week.

    I am very interested in participating in a stem cell Clinical Trial. Will travel.

  12. Kaye Morrison says:

    I have pulmonary fibrosis secondary to interstitial pneumonitis with autoimmune factors. Would my diagnosis qualify for these studies.

  13. I just got to the point where I’m now dormant after having Valley Fever for 18 months. I was then immediately diagnosed with IPF which was a couple of months ago. I just started using Esbriet a couple weeks ago with few side effects so far. I would also be interested in a stem cell clinical trial which I’m sure my pulmonary doctor would agree to my participation. Please let me know of any clinical trials for which I may qualify and also updated on the results of and existing or new clinical trials.

    • Wayne says:

      I have a friend who was went on a 1 month vacation earlier this year to southern Nevada. She was fine for about 2 weeks then developed a cough and flu like symptoms. The prompt care service here gave her antibiotics which seemed to help for about a week. After visiting her regular physician and doing more tests she was diagnosed with IPF. Was there any concerns as to whether Valley Fever may have caused the Pulmonary Fibrosis?

  14. Neelam says:

    My mother is 62. She just got diagnosed with ILD. After biopsy we got to know that it was pulmonary fibrosis. She is on 18 hrs of oxygen and housebound. She finds it difficult to walk and breathe………. If she can be a candidate for clinical trial do let me know. Thanks.

  15. ROBERT MCCREARY says:

    I was diagnosis about 18 months (current age 67) or so ago and I immediately went on ESBREIT. I was doing Pulmonary Rehab 3 to 4 days a week and progressing well. Only 2 liters of oxygen when I worked out or would need a oxygen tune-up as I called it if I overdid. Basically go into house and put on the oxygen for a few minutes. Felt like I was getting stronger and could almost do anything just a bit slower. After being on the ESBREIT for about 8 months there was no increase in the lung scarring. All looked good. Then the mistake I was getting hip shots because my right hips was bad and the shots stopped working so I agreed to have hip surgery. Either (and they are not sure which) I got pneumonia in the hospital or had a pulmonary flareup does it really matter which put me back in the hospital. Now on steroids and oxygen 24/7 and talking lung transplant. So the lesson is NO SURGERY; MAKE SURE YOU ASK EVEREY QUESTION IF YOU MUST GO INTO SURGERY LIKE “CAN MY LUNG CONDITION GET WORST”. Had I known my lung would take such a hit I would never have had the hip surgery screw the hip. Remember no one cares about you but you so you best be your won advocate.

  16. Gary L Short says:

    I was diagnosed with I PF in July this year would gladly participate in in stem cell research. I a 66
    years old and I am on oxygen 24/7. If one comes open in the Phoenix,Az please let me know!

  17. Marilyn says:

    59 diagnosis began with Lupus
    Raynauds; Crest Syndrome; PAH; and the fatal diagnosis of Scleroderma along with Interstitial Pulmonary Fibrosis.!
    I was blessed to vegan my treatment with Oxygen only at night. Lost my baby brother at the end of July of this year and have been experiencing more shortness of breath and fatigue.
    My rheumatologist just prescribed me to continue oxygen 24/7 due to oxygen exhaustion which of course causes fatigue and escalates shortness of breath and anxiety!
    I am still 59yrs young with a daughter who is planning a wedding for this time next year!!
    By the Grace of God I am praying someone will respond with good news and additional information needed🙏🏽

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