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Amyloidosis

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Core Concepts in Parenchymal Kidney Disease

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Abstract

Amyloidosis is a group of diseases characterized by deposition of amyloid fibrils in soft tissues and organs. These fibrils all have the similar characteristics that distinguish them as amyloid. More than 24 proteins have been identified to form amyloid via several mechanisms such as genetic mutation, accumulation, age associated, and multifactorial. The kidney is one of the most commonly affected organs, but affinity to different organs may be predetermined by the native protein. The diagnosis of amyloidosis required demonstration of amyloid deposits in the tissues. In kidney tissue, amyloid appears as pale amorphous extracellular deposits that are periodic acid Schiff (PAS) and silver negative. Congo red staining will produce a green birefringence when viewed by polarized light. Amyloid fibrils are characteristically 7–12 nm in diameter and are randomly arranged on electron microscopy. Once amyloid is detected, typing must be performed to identify the native protein as treatment is type specific. The most common method of amyloid typing is with immunofluorescence or immunohistochemistry. If antibodies are not available, genetic testing may be helpful in identifying hereditary cases. The most advanced method of amyloid typing is liquid chromatography–tandem mass spectrometry. Once amyloid is identified, serum amyloid P (SAP) scintigraphy can be used to locate amyloid in the body. Treatment of amyloidosis depends on the type. Anti-myeloma therapy is used to treat immunoglobulin light chain amyloidosis (AL), antimicrobials or anti-inflammatory medications are used for serum amyloid A (AA), and organ transplantation may be used for some hereditary amyloidoses.

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Authors and Affiliations

  1. Division of Nephrology and Hypertension, Mayo Clinic, Rochester, MN, USA

    Nelson Leung M.D.

  2. Division of Hematology, Mayo Clinic, Rochester, MN, USA

    Nelson Leung M.D.

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  1. Nelson Leung M.D.
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Editors and Affiliations

  1. Div. Nephrology & Hypertension, Mayo Clinic Dept. Internal Medicine, Rochester, Minnesota, USA

    Fernando C. Fervenza

  2. Renal Division, Brigham and Women's Hospital, Boston, Massachusetts, USA

    Julie Lin

  3. Mayo Clinic, Rochester, Minnesota, USA

    Sanjeev Sethi

  4. Renal Division, Brigham and Women's Hospital Harvard Medical School, Boston, Massachusetts, USA

    Ajay K. Singh

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Leung, N. (2014). Amyloidosis. In: Fervenza, F., Lin, J., Sethi, S., Singh, A. (eds) Core Concepts in Parenchymal Kidney Disease. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-8166-9_21

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