Chicago Patient Conference

To: My Fellow Sarcoidosis Patients

From: Mark Landiak, FSR Patient Ambassador

Re: Chicago Patient Conference Meeting Notes

Enclosed are my notes from the Foundation for Sarcoidosis Research (FSR) Chicago Patient Conference. While not comprehensive, it will give you a flavor of some of the highlights of the event. I tried my best to keep up with the presentations, but the doctors moved very quickly from slide to slide. I do not certify the accuracy of the items covered, but merely reported the comments to the best of my ability. I hope you will find this helpful.

There are 3 summaries contained on this page.

  • Dr. Wonder Drake, Vanderbilt University (posted)
  • Dr. Nadera Sweiss, Univ. of Chicago (coming this week)
  • Dr. Elliott Crouser, Ohio State University (coming this week)

May 1, 2016: Dr. Wonder Drake – What is Sarcoidosis?

  • Criteria used to make the diagnosis of Sarcoidosis: Why it takes a long time to make a diagnosis.
    • Sarcoidosis does not present itself easily.
      • Doctors don’t look for Sarcoidosis first. Clinical presentation is a process of elimination.
        • The first step in the process is that we have to rule out other things.
        • After we rule everything else out, we call it Sarcoidosis.
    • With Sarcoidosis, sometimes the Chest X-Ray looks like a typical X-Ray.
      • Because Sarcoidosis is rare, it can look like and be interpreted as a fungal infection – especially in areas where fungal infections are common.
    • The type of sickness that you have.
      • What your chest x ray looks like.
      • What your tissue looks like under the microscope and if any microorganisms grow from your tissue.
    • When symptoms occur, the first respiratory interpretations are often Histo fibrosis (fungal infections), Cancer, or TB (accounts for misdiagnosis or long diagnosis).
      • Every time you breathe in air, you could be breathing in bacteria. When this happens, a collection of infection fighting cells can take over the air spaces in between cells and this lessens the right amount of gas that gets exchanged (resulting in patients getting short of oxygen).
      • In Sarcoidosis patients, granulomas form when infection fighting immune cells can’t get out and go away. They band together and more immune cells come to join the fight.
        • Dr. Drake likened this to a bench-clearing brawl with the referee in the middle (oxygen) cannot get out due to all the players (immune fighting cells) surrounding him.
      • Dr. Drake presented data demonstrating the rising incidents of Sarcoidosis mortality and morbidity over the years.
      • She reviewed findings delineating the contribution of genetics to Sarcoidosis’ clinical outcomes.
        • I found this really interesting. There now appears to be a familial link in who can contract Sarcoidosis. That said, the chances are still relatively low – only 4-5% within the same family.
  • She also discussed the results of immunologic inquiry of Sarcoidosis adaptive immunity and how this helps us identify new therapeutics (below) and presented new areas of investigation.
  • Sarcoidosis is characterized by TH-1 Immunophynotype
    • Infection-fighting cells meet up and form granuloma. Then T-Cell comes in.
    • Can’t kill the granuloma, but T cell keeps the disease in check.
      • (See: Baughman RP, et al. Am J Respir Crit Care Med 2011;183:573-581 for more info)
  • There are HLA protiens on infection-fighting cells that can aid healing. 99% w/ this gene can resolve their Sarcoidosis in 2 years w/ no intervention.
  • Race is not the most important factor anymore.
    • How the Immune System resolves the disease.
    • Where the disease started is where the resistance is the strongest. (Scandinavia)

Greatest Per Capita Occurrence of Diagnosed Sarcoidosis

  1. Netherlands
  2. Japan
  3. USA

Additional Information

  • 90% of patient’s have Sarcoidosis indications in the spleen.
  • Sarcoidosis is called “the great masquerader.”
  • Patients who exercise more feel less fatigue.
  • Weight
    • Stay w/in your ideal body weight
  • Exercise
  • The body can adapt, even w/ Sarcoidosis
  • Push yourself gently
  • Do what you can – Even very slow exercise every day is good

Prednisone

  • Needs to be constantly monitored for impact on your body and Immune system
  • Prednisone stops the immune system from working – necessary to avoid granuloma formation

How well does your immune system work?

  • People whose Sarcoidosis is resolving have more normalized immune systems
  • T-Cell exhaustion shows that when Immune system gets tired – PD1 protein on infection fighting cells
  • PD1 stays elevated with Sarcoidosis patients vs patients who are resolving
  • High levels of PD1 are not good

New Areas of Investigation

  • The best news I heard all day was that several clinical trials investigating new treatments for sarcoidosis are underway.
    • Dr. Drake stated: “There is progress being made. I feel like we are going to cure this disease.”
    • Dr. Nadera Sweiss added that it will be at least 2 years before we see published results.
  • Studies mentioned include:
    • CLEAR Study
      • CLEAR: Combined Levathin, Ethambutol, Azithromycin and Rifabutin
    • Genetics of Sarcoidosis
    • GRADS Study
    • Dr.Drake is doing studies on a new drug that appears to work on cancer patients. It is not yet FDA-approved.
      • Research Volunteers are needed for the CLEAR II Study being run by Dr. Drake
      • Phase 1 trial showed improved breathing and got the NIH to fund a Phase 2 study.
      • Purpose of the study is to see if breathing improves with the use of 4 antibiotics
    • If you have an interest in participating, contact:
    • If you want to see all the clinical trials underway, go to ClinicalTrials.Gov and type in Sarcoidosis and you’ll see them all.

Overall, I found Dr. Drake’s presentation to be “Wonder-ful.” I learned a lot and will read up on the CLEAR Phase 1 study. She’s doing amazing work.