The Heart Stopping Granuloma

When I go to ACR, I’m always looking for “THE” session to provide me with great clinical pearls, hoping it will help with the care of my patients. The Cardiac Sarcoid session was a good overview but it left me wanting more – and confirmed what I already knew – that the understanding and therefore management of this disease with all its particular organ manifestations is still lacking.

Cardiac sarcoidosis is present in ~25% of chronic sarcoidosis patients but mortality may be lower than the often quoted 50%. The prognosis of patients with treatment is better for those with preserved ejection fraction than those with low ejection fraction. Prognosis is better in patients with conduction delays (eg. 50% return to normal with treatment while none will normalize without treatment). Radiofrequency ablation and defibrillators become an important adjunct in treatment but are not without morbidity (eg. unnecessary shocks ~15% of the time). The ultimate survival of patients with treatment remains unclear.

Disappointingly, Dr. Culver did not address recommended treatments because there is insufficient evidence for any specific agent over another. What he did recommend was long-term treatment (eg. several years of corticosteroids) with consideration of steroid-sparing agents during this time. However, he made no recommendations for choosing between methotrexate vs. mycophenolate vs. biologics vs. other drugs.

More work is needed to study this rare disease – and for now, each steroid-sparing agent should be carefully considered. Continued shared care of these patients between cardiology and rheumatology is needed, especially when considering endocardial biopsy, PET or MRI scanning of the heart.

More reports from ACR 2014