Management of AA Amyloidosis
The primary management approach for AA amyloidosis is aggressive treatment of the underlying inflammatory condition to suppress serum amyloid A (SAA) protein production, with the goal of maintaining SAA levels below 10 mg/L to prevent progression and potentially reverse established amyloid deposits. 1
Underlying Disease Management
Step 1: Identify and Treat the Underlying Inflammatory Condition
- Common causes requiring targeted therapy:
- Rheumatic disorders (rheumatoid arthritis, ankylosing spondylitis)
- Autoinflammatory diseases (especially Familial Mediterranean Fever)
- Chronic infections (tuberculosis, osteomyelitis, bronchiectasis)
- Inflammatory bowel disease (particularly Crohn's disease)
- Primary sclerosing cholangitis 2
Step 2: Disease-Specific Treatments
For Familial Mediterranean Fever (FMF):
- Colchicine is the cornerstone therapy
- Starting dose: 0.5-1.5 mg/day (can be divided to improve tolerance)
- Dose should be increased by 0.5 mg/day if attacks persist or inflammatory markers remain elevated
- Target: Complete suppression of inflammatory activity 1
For other inflammatory conditions:
- Appropriate disease-modifying therapies based on the underlying condition
- Biological agents may be required for refractory cases
Monitoring Disease Activity
Laboratory Monitoring
- Serum Amyloid A (SAA) protein: Target < 10 mg/L
- C-reactive protein (CRP): Used as surrogate when SAA unavailable
- Complete blood count
- Renal function tests
- Liver enzymes
- Proteinuria assessment 1
Frequency of Monitoring
- Initially: Monthly monitoring of inflammatory markers
- Once stable: Every 3-6 months
Organ Support and Management of Complications
Renal Management
- Proteinuria control:
- Blood pressure management
- Avoid nephrotoxic drugs
- Prevent hypoperfusion
- Renal replacement therapy when needed:
- Dialysis for end-stage renal disease
- Renal transplantation in selected patients (outcomes comparable to non-diabetic end-stage renal failure) 1
Other Organ Support
- Manage symptoms of affected organs (liver, spleen, GI tract)
- Avoid factors that may trigger inflammation or worsen amyloidosis
Advanced Therapeutic Options
Biological Agents for Refractory Cases
- IL-6 inhibitors (tocilizumab):
Transplantation Considerations
- Renal transplantation can be considered in appropriate candidates
- Long-term graft and patient survival matches age-matched general transplant population
- Living donor transplants increasingly used 1
Special Considerations
Amyloidotic Kidney Protection
- Amyloidotic kidneys are unusually sensitive to additional injury:
- Avoid hypoperfusion
- Maintain strict blood pressure control
- Avoid nephrotoxic drugs
- Take special precautions during surgery 1
Trigger Avoidance
- Identify and avoid triggers that may exacerbate the underlying inflammatory condition:
- Physical or emotional stress
- Physical trauma
- Exposure to cold
- Infections
- High fat intake
- Starvation
- Sleeplessness and tiredness 1
Colchicine Dosing Considerations
- Consider temporarily increasing colchicine dose during periods of stress or trigger exposure
- For gastrointestinal side effects:
- Split doses
- Temporary dietary modifications (reduce dairy products)
- Gradual dose escalation
- Antidiarrheal agents if needed 1
Treatment Algorithm
- Diagnose AA amyloidosis (tissue biopsy with Congo Red staining and immunohistochemical confirmation)
- Identify and aggressively treat underlying inflammatory condition
- Monitor SAA levels (target < 10 mg/L)
- Provide organ support as needed
- Consider advanced therapies for refractory cases
- Monitor for disease progression and treatment response
The prognosis of AA amyloidosis has improved significantly with modern management approaches, particularly when the underlying inflammatory condition can be effectively controlled.