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AA amyloidosis
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AA amyloidosis

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Contributors: Amirah Khan MD, Catherine Moore MD, Paritosh Prasad MD
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Synopsis

AA amyloidosis (previously known as secondary amyloidosis) is characterized by the extracellular deposition of serum amyloid A containing fibrils, which is synthesized in the liver. Serum amyloid A is an acute phase reactant and is most strongly associated with chronic inflammatory diseases such as rheumatoid arthritis, ankylosing spondylitis, juvenile idiopathic arthritis, inflammatory bowel disease, psoriatic arthritis, familial Mediterranean fever, malignancies, chronic infections, and intravenous drug abuse. AA amyloidosis has become less common in the past century due to improvement in diagnosis and therapy of chronic inflammatory diseases and infections.

The clinical presentation of AA amyloidosis is dependent on which organ systems are affected. The kidneys are affected the majority of the time (90% of the time), and untreated AA amyloidosis can lead to end-stage renal disease. The liver and heart are other major sites of amyloid deposition. Gastrointestinal (GI) amyloidosis is also more common in AA amyloidosis, present in 10%-70% of patients depending on series.

Signs and symptoms of AA amyloidosis include arrhythmia, dysphagia, dyspnea, anasarca, fatigue, numbness in hands and feet, rash, easy bruising, swollen tongue, weak grip, weight loss, diarrhea, GI tract bleeding, proteinuria, hepatomegaly, and splenomegaly.

The hallmark of treatment for AA amyloidosis is treating the underlying inflammatory disease process.

Related topic: AL amyloidosis

Codes

ICD10CM:
E85.3 – Secondary systemic amyloidosis

SNOMEDCT:
402457007 – Secondary systemic amyloidosis affecting skin (AA fibril type)

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed: 02/01/2019
Last Updated: 02/11/2019
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AA amyloidosis
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AA amyloidosis : Easy bruising, Numbness, Proteinuria, Weakness
Copyright © 2019 VisualDx®. All rights reserved.