Sarcoidosis: Beyond 40mg of Prednisone with Dr. Baughman

What is sarcoidosis?

  • It’s more of an inflammatory, rather than autoimmune, disease (one of the key features being noncaseating granulomas).
  • It often begins in the lungs, skin or lymph nodes. However, we also know that sarcoidosis can involve the brain, heart, liver, eyes or really – any organ.
  • The cause is unknown and the outcome is highly variable – ranging from benign disease to devastating organ infiltration and dysfunction.

What are most challenging aspects of caring for patients with sarcoidosis?

  1. Making the diagnosis
  2. Determining the prognosis

Pulmonary Hypertension & Sarcoidosis

Who has it?

Consider concomitant pulmonary HTN in patients treated for sarcoid who still have persistent shortness of breath or desaturations. Workup to consider:

  • Six minute walk test
  • PFTs (with a careful eye toward the diffusion capacity of the lungs for carbon monoxide)
  • Echocardiogram (looking for more than just elevated RVSP)
  • Right heart cath (almost always)

Prevalence of PHTN + Sarcoid

  • In the literature, lowest number (all-comers) 5% (Japan); highest number 20% (Saudi Arabia).
  • In patients specifically with persistent shortness of breath (mild, moderate OR severe), these numbers jump up to 50%.

Vasoactive Agents

  • These are indeed showing benefit in the sarcoid population – just like they have in patients with PHTN + idiopathic pulmonary fibrosis.

Multidisciplinary approach

  • Sarcoid is a multidisciplinary disease. Dr. Baughman runs a Pulmonary HTN clinic alongside a cardiologist

 Cardiac sarcoid

  • Have to have high index of suspicion in order to diagnose cardiac sarcoid. Make sure to ask about palpitations.
  • EKG and echo are often a part of the workup, but 24 hour holter monitors are much more helpful. Looking for a high burden of PVCs or any other arrhythmia.

Cardiac MRI vs PET

  • This is institution specific (they do PET)
  • MRI – useful to determine if the patient has cardiac involvement
  • PET – useful to determine if the patient has active inflammatory disease

Management of cardiac sarcoid

  • Determine if they need a defibrillator or not
  • Remember that defibrillator is just safety net, not a treatment
  • Anti-inflammatory therapy (methotrexate +/- prednisone)
  • Monitor for arrhythmias/PVCs and tailor treatment to reduce their occurrence
  • Serial PET scans – study in progress, unclear benefit
  • Treatment is also likely to benefit patients with very low EF%
  • Treat for at least two years; decision to withdraw therapy is complex. Remember there is no cure, so we’re only treating based on symptoms.

De novo sarcoidosis

  • Often refers to isolated cardiac or isolated neural sarcoidosis
  • Roughly 5-10% prevalence, but it depends on how hard you look.
  • 50% of patients diagnosed with ‘de novo’ sarcoidosis will actually develop a granuloma elsewhere
  • Further – some patients with isolated pulmonary sarcoidosis will develop other manifestations of the disease (e.g. iritis).

Antimicrobial Therapy: CLEAR trial

Levofloxacin ethambutol, azithro, rifampin

The real question is – is it the anti-inflammatory effect of these agents or is it the antimicrobial effect? Is it a component of both?

The CLEAR trial isn’t necessarily standard of care, but if you use it, therapy is for a total of 16 weeks:

  • First 8 weeks – all 4 agents
  • Second 8 weeks – ethambutol rifampin second 8 weeks

Funding

Funding for sarcoidosis research is limited. Dr. Baughman attributes this to:

  • There is currently no good model for sarcoidosis as a disease entity
  • Sarcoidosis is a very diverse disease with heterogeneous patients, a wide variety of clinical outcomes and no clear endpoints for therapy.

HP Acthar Gel

3rd-4th line agent. Best for patients who are failing other agents

 

What can patients with sarcoidosis do?

FSR_logo2.jpg
  • Get plugged in
  • Check out The Foundation for Sarcoidosis Research at https://www.stopsarcoidosis.org/
  • Understand fatigue is common
  • Look out for signs of sleep apnea
  • Do NOT take too much vitamin D
  • Gluten free diet? Jury is out on this one

 

Marshall Protocol

The Marshall Protocol involves two things:

  • Antimicrobial regimen (similar to CLEAR)
  • Avoid vitamin D
    • Both of these things seem reasonable
  • Olmesartan therapy – nothing in literature to support its use
    • Keep in mind this will lower your measured ACE levels

 

Future of sarcoid

  • We probably need to start doing what the rheumatologists do – Being therapy with other widely available drugs and ONLY use prednisone when the patient is absolutely miserable or failing conventional therapy.
  • At this point, we need to find out WHY patient isn’t responding.
  • Do they have PHTN? Fibrosis? – we have treatment for these! Are they fatigued – neurostimulants may be beneficial
  • Bottom line: we have options other than just 40mg prednisone. This is where the future of sarcoid is headed.

 

Jeremy AmayoComment