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?
- Making the diagnosis
- 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%.
- These are indeed showing benefit in the sarcoid population – just like they have in patients with PHTN + idiopathic pulmonary fibrosis.
- Sarcoid is a multidisciplinary disease. Dr. Baughman runs a Pulmonary HTN clinic alongside a cardiologist
- 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 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?
- 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
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.