Secondary amyloidosis medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Roukoz A. Karam, M.D.[2]

Overview

Medical or surgical treatment of the underlying chronic infection or inflammatory disease is recommended among all patients who develop AA amyloidosis.

Medical Therapy

• Medical or surgical treatment of the underlying chronic infection or inflammatory disease is recommended among all patients who develop AA amyloidosis.
• Aggressively treating the disease that is causing the excess amyloid protein can improve symptoms and slow down or halt the progression of the disease.
• Complications such as heart failure, renal failure, and other problems can sometimes be treated, when needed.
• Although the choice of therapy depends on the underlying cause of chronic inflammation, the aim is always to suppress production of SAA to within the normal range.
• Examples of treatments for the commonest disorders underlying AA amyloidosis, adapted from Rheumatic Diseases Clinics of North America:

Underlying Condition	Treatment Options	Examples
Inflammatory arthritis	Conventional disease-modifying agents	Gold Hydroxychloroquine sulfasalazine Azathioprine Methotrexate
	Other immunosuppressant agents	Cyclosporine Cyclophosphamide Mycophenolate Leflunomide
	Biologic agents	Infliximab Etanercept Adalimumab Tocilizumab Rituximab
Periodic fevers	On-demand agents	Nonsteroidal anti-inflammatory drugs Prednisone
	Colchicine (for familial mediterranean fever)	Colchicine
	Biologic agents	Anakinra Canakinumab
Inflammatory bowel disease	Conventional disease-modifying agents	Sulfasalazine Mesalazine Azathioprine Methotrexate
	Biologic agents	Infliximab Adalimumab
	Antibiotics	Metronidazole Ciprofloxacin Azithromycin
	Biologic agents	Infliximab Adalimumab
	Surgery	ileo-cecal resection and primary reconstruction
Immunodeficiency	Immunoglobulins
	Antibiotics	Cotrimoxazole Miconazole
Chronic infections	Antibiotics and surgery
	Physiotherapy (in case of bronchiectasis)
Immunodeficiency	Immunoglobulins
	Antibiotics	Cotrimoxazole Miconazole
Neoplasia	Chemotherapy and surgery	Varies according to type of cancer
	Biologic agents (in Castleman disease)	Tocilizumab

• Long-term inflammatory control can be accompanied by gradual regression of amyloid deposits and improvement in renal function.
• Currently a second clinical trial is in progress in order to evaluate a targeted inhibitor molecule, Kiacta, in the management of secondary amyloidosis.
• Novel therapies aimed at promoting clearance of existing amyloid deposits soon may be an effective treatment approach.