Do steroids help treat cardiac amyloidosis?

Steroids for Cardiac Amyloidosis Treatment

Corticosteroids alone are not recommended as primary treatment for cardiac amyloidosis, but they are used as part of combination chemotherapy regimens for AL amyloidosis. 1, 2

Treatment Based on Amyloidosis Type

Treatment for cardiac amyloidosis must be tailored to the specific type:

AL (Light Chain) Amyloidosis

• First-line treatment: Daratumumab combined with cyclophosphamide, bortezomib, and dexamethasone (Dara-CyBorD) 1, 2
  • Corticosteroids (dexamethasone) are included in this regimen
  • Achieves high rates of deep hematologic responses
• Alternative options:
  • CyBorD alone (cyclophosphamide, bortezomib, dexamethasone) for patients who cannot tolerate daratumumab 2
  • High-dose melphalan with autologous stem cell transplantation (HDM/SCT) for eligible patients without significant cardiac involvement 1, 2

ATTR (Transthyretin) Amyloidosis

• FDA-approved treatment: Tafamidis 2
• Steroids have no role in ATTR amyloidosis treatment

Corticosteroid Use and Considerations

When used as part of combination therapy for AL amyloidosis, corticosteroids come with significant cardiac risks:

• Peripheral edema
• Pulmonary edema
• Fluid overload 1

These side effects can be particularly problematic in patients with pre-existing cardiac involvement, requiring careful monitoring and dose adjustments.

Monitoring During Treatment

For patients receiving treatment that includes corticosteroids:

• Monthly monitoring of:
  • Complete blood count
  • Biochemistry
  • NT-proBNP
  • Troponin
  • Serum-free light chain quantification 2
• Echocardiography with strain measurements every 6 months
• Holter ECG every 6 months 2

Multidisciplinary Approach

Management of cardiac amyloidosis requires collaboration between:

• Cardiologists
• Hematologists
• Neurologists (for ATTR with neuropathy)
• Nephrologists (for renal involvement)
• Palliative care specialists 1, 2

Important Caveats

• Cardiac toxicity risk: Treatment-related mortality is a significant concern, particularly in patients with advanced cardiac involvement 1, 3
• Eligibility assessment: Patients must be carefully selected for aggressive therapies based on cardiac staging and overall performance status
• Response monitoring: Hematologic response (goal: very good partial response or better) must be regularly assessed 2
• Advanced options: For end-stage disease, heart transplantation may be considered, sometimes combined with liver transplantation for hereditary ATTR 2, 3

The treatment landscape for cardiac amyloidosis has evolved significantly in recent years, with targeted therapies showing improved outcomes compared to traditional approaches. While corticosteroids remain an important component of AL amyloidosis treatment regimens, they are never used as monotherapy and must be administered with careful attention to their potential cardiac complications.