From the Guidelines
The prognosis for AA amyloidosis is generally poor, with a median survival of approximately 4-5 years from diagnosis, but early diagnosis and aggressive treatment of the underlying inflammatory condition can significantly improve outcomes. The prognosis varies depending on several factors, including the extent of organ involvement and the effectiveness of treatment in suppressing the underlying inflammatory disease. According to the EULAR recommendations for the management of familial Mediterranean fever 1, treatment for amyloidosis comprises measures to support failing organ function, including blood pressure control and dialysis for patients with renal disease.
Key factors that influence the prognosis of AA amyloidosis include:
- The extent of kidney involvement, with patients having advanced renal failure at diagnosis having poorer outcomes, and median survival of about 1-2 years if dialysis is required
- The presence of cardiac involvement, which indicates a worse prognosis when present
- The effectiveness of treatment in suppressing the underlying inflammatory disease, with patients who achieve complete suppression having better survival rates
- The level of serum amyloid A protein, with maintaining levels below 10 mg/L correlating with better outcomes and potential regression of amyloid deposits 1
Treatment for AA amyloidosis focuses on suppressing the underlying inflammatory condition using targeted therapies, and regular monitoring of serum amyloid A protein levels is essential. Organ transplantation may be considered in end-stage organ failure, though recurrence in transplanted organs remains a concern if the underlying inflammation is not controlled. As noted in the EULAR recommendations 1, renal transplantation in selected patients has been encouraging, with long-term graft and patient survival matching that of the age-matched general transplant population.
From the Research
Prognosis for AA Amyloidosis
The prognosis for Amyloid A (AA) amyloidosis is generally poor, especially if left untreated or if diagnosis is delayed 2. Key factors influencing the prognosis include:
- The severity of organ involvement, particularly kidney damage 2, 3
- The effectiveness of controlling the underlying inflammatory process 4, 2
- The presence of multiple organ failure, including heart, autonomic, and adrenal insufficiency 2
Treatment and Management
Current treatment strategies for AA amyloidosis focus on controlling the inflammatory response and reducing serum amyloid A protein levels 4, 2. This can be achieved through:
- Anti-inflammatory biologic agents 4
- Anti-cytokine agents 2
- Colchicine use in selected cases 3
- Kidney transplantation in patients with end-stage kidney disease 4, 2
Outcome and Survival
The long-term prognosis remains poor for patients on chronic dialysis, especially with clinical gut involvement 5. However, effective control of the underlying inflammatory process can halt disease progression and even reverse damage 2. Novel treatments targeting key molecular events in the fibrillogenesis process are under development, offering potential for improved patient outcomes 4, 2.