Serum amyloid A protein (AA) amyloidosis, also known as secondary amyloidosis, is a known consequence of chronic inflammation and results from several conditions including inflammatory arthritis, periodic fever syndromes, and chronic infection. AA amyloidosis can lead to multiorgan dysfunction, including changes in glomerular filtration rate and proteinuria. Definitive diagnosis requires tissue biopsy, and management of AA amyloid kidney disease is primarily focused on treating the underlying inflammatory condition to stabilize glomerular filtration rate, reduce proteinuria, and slow potential progression to kidney failure. In this narrative review, we describe the causes, pathophysiology, presentation, and pathologic diagnosis of AA amyloid kidney disease using an illustrative case of biopsy-proven AA amyloid kidney disease in a patient with long-standing rheumatoid arthritis who had a favorable response to interleukin 6 inhibition. We conclude the review with a description of established and more novel therapies for AA amyloidosis including published cases of use of tocilizumab (an interleukin 6 inhibitor) in biopsy-proven AA amyloid kidney disease.Amyloid A protein (AA) amyloidosis results from chronic inflammation and is characterized by extracellular multiorgan deposition of serum AA protein fibrils leading to organ dysfunction. 

AA amyloid kidney disease is the most common manifestation, typically resulting in proteinuria and progressive decreased kidney function. The management of AA amyloidosis focuses on the treatment of the underlying inflammatory conditions, which include inflammatory arthropathies, familial Mediterranean fever and chronic infections. Herein, we describe the causes, pathophysiology (including biopsy findings) and diagnosis of AA amyloid kidney disease using an illustrative case in a patient with long-standing rheumatoid arthritis who presented with nephrotic syndrome and acute onset chronic kidney injury. We conclude with a review of current literature on established and emerging therapies for AA amyloidosis, including a specific focus on the use of interleukin 6 (IL-6) inhibitors in biopsy-proven AA amyloid kidney disease.

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Mishita
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Journal of Clinical & Experimental Nephrology