Is apheresis a treatment option for cardiac sarcoidosis?

Treatment of Cardiac Sarcoidosis: No Evidence for Apheresis

Apheresis is not a recommended treatment option for cardiac sarcoidosis based on current evidence and guidelines. 1

Established Treatment Options for Cardiac Sarcoidosis

First-Line Therapy

• Corticosteroids are the mainstay of therapy for cardiac sarcoidosis, used to suppress inflammation and granuloma formation 1
• Typically initiated at high doses (prednisone 40-60 mg daily) and tapered slowly over months as clinical and imaging features stabilize or improve 1
• Corticosteroid treatment has been associated with improved survival rates, with studies showing 75% of steroid-treated patients surviving for 5 years 1

Second-Line and Adjunctive Therapies

• Other immunosuppressive therapies are reasonable in patients who cannot tolerate corticosteroids or continue to worsen despite corticosteroid treatment (Level of Evidence C) 1
• These include:
  • Methotrexate 1, 2
  • Azathioprine 1
  • Mycophenolate mofetil 1
  • Cyclophosphamide 1
  • Pentoxifylline 1
  • Thalidomide 1
• Collaboration with pulmonologists or rheumatologists for immune-modulating therapy can be useful (Level of Evidence C) 1

Cardiac-Specific Management

• Standard guideline-directed medical therapy for heart failure is recommended for patients with reduced ejection fraction (Level of Evidence B) 1
• ICD implantation is reasonable for patients with cardiac sarcoidosis (Level of Evidence C) 1
• For advanced heart failure:
  • Referral for cardiac transplantation or mechanical circulatory support should be made for patients with advanced heart failure without significant extracardiac sarcoidosis (Level of Evidence C) 1
  • Cardiac transplantation has shown better short- and intermediate-term survival in sarcoidosis patients compared to those transplanted for other reasons 1

Monitoring and Follow-up

• Cardiac MRI or PET with fluorodeoxyglucose imaging can be useful to diagnose cardiac sarcoidosis and follow response to therapy (Level of Evidence B) 1
• Close monitoring for relapse is essential after discontinuation of corticosteroid treatment 1
• Echocardiography should be performed in patients with signs and symptoms of heart failure to assess left ventricular ejection fraction (Level of Evidence C) 1

Emerging Treatments

• Clinical trials are underway to evaluate alternative treatment strategies, such as low-dose prednisone/methotrexate combinations compared to standard-dose prednisone 3
• Biological agents, particularly infliximab, are being studied as potential steroid-sparing options 2

Important Considerations and Pitfalls

• The diagnosis of cardiac sarcoidosis can be challenging, with endomyocardial biopsy providing diagnostic evidence in only 25-50% of autopsy-confirmed cases 1
• No randomized controlled trials have established optimal dosing, timing, or duration of corticosteroid treatment 1
• Treatment decisions should consider the location, extent, and activity of the disease 3
• Long-term corticosteroid use is associated with significant side effects, highlighting the importance of developing effective steroid-sparing strategies 2, 4

Despite extensive review of the available evidence, apheresis is not mentioned as a treatment option for cardiac sarcoidosis in any of the guidelines or research studies. The American Heart Association's scientific statement on cardiac sarcoidosis provides comprehensive treatment recommendations without including apheresis as a therapeutic option 1.