treatment and prognosis of systemic AA amyloidosis
Mobilization and clearance of amyloid deposits are possible and are best recognized for patients with AA. A basic tenet is to control the underlying inflammatory disease. Potent biologic agents are available to control the inflammatory arthritides (RA, JIA, and AS) and the autoinflammatory syndromes. Prophylactic colchicine (1.2–1.8 mg/day) is effective in suppressing the inflammatory episodes, and subsequent amyloidosis seen in familial Mediterranean fever. Surgical treatment of osteomyelitis with amputation and aggressive surgical therapy for Crohn’s disease has been reported to reverse or resolve nephrotic syndrome. Measurement of SAA levels can monitor the success of therapy. Median survival in patients with AA amyloidosis whose underlying inflammatory disease is not suppressed is 5 to 10 years with 40% to 60% patients dying of renal failure. Other therapies being tested include antiamyloid immunotherapy as well as a molecule which competitively binds to the GAG-binding sites on SAA and inhibits fibril polymerization and amyloid deposition in tissues such as the kidneys.