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AA amyloidosis
Other Resources UpToDate PubMed

AA amyloidosis

Contributors: Amirah Khan MD, Catherine Moore MD, Paritosh Prasad MD
Other Resources UpToDate PubMed


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 or uvula, 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


E85.3 – Secondary systemic amyloidosis

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

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • AL amyloidosis
  • Hereditary amyloidosis
  • Dialysis-related amyloidosis
  • Age-related systemic amyloidosis
  • Diabetic nephropathy
  • Membranoproliferative glomerulonephritis
  • Membranous nephropathy
  • Focal segmental glomerulosclerosis
  • Fibrillary Glomerulonephritis
  • Idiopathic nodular glomerulosclerosis
  • Sarcoidosis
  • Ischemic cardiomyopathy (see Dilated cardiomyopathy)
  • Systemic lupus erythematosus
  • Multiple myeloma

Best Tests

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Management Pearls

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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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Last Reviewed:01/31/2019
Last Updated:03/24/2022
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AA amyloidosis
A medical illustration showing key findings of AA amyloidosis : Easy bruising, Numbness, Proteinuria, Weakness
Copyright © 2024 VisualDx®. All rights reserved.