AA Amyloidosis

Overview

What is AA amyloidosis?

AA amyloidosis is one type of the rare disorder amyloidosis (pronounced “am-uh-loy-doh-sis”). Amyloidosis happens when proteins in your body lose their three-dimensional (3D) structure and become twisted clumps of misshapen fibrils (amyloid deposits) that gather on your organs and tissues.

AA amyloidosis is also known as secondary amyloidosis or amyloid serum A protein. This amyloidosis type happens when you have high levels of inflammation in your body that boost the serum A protein levels in your bloodstream. You may have high serum A protein levels if you have a long-lasting infection or inflammatory disease. In a sense, AA amyloidosis is a serious complication of inflammatory diseases and conditions. Healthcare providers treat AA amyloidosis by controlling the underlying disease or condition.

How does AA amyloidosis affect my body?

AA amyloidosis can damage several of your organs, including your kidneys (most common), liver, spleen, stomach and intestines. Rarely, AA amyloidosis damages your heart. AA amyloidosis may also affect the amount of protein in your pee and your cholesterol levels.

Is AA amyloidosis terminal?

AA amyloidosis can be a terminal illness if it isn’t treated. You may develop AA amyloidosis if you have a chronic illness that causes inflammation. When healthcare providers treat the underlying condition, they’re also treating AA amyloidosis.

Who is affected by AA amyloidosis?

Anyone who has a chronic inflammatory condition can develop AA amyloidosis.

Symptoms and Causes

What causes AA amyloidosis?

AA amyloidosis is a type of amyloidosis. Healthcare providers call amyloidosis a “protein misfolding disorder.” Your proteins are long molecules that form 3D structures. They’re multitaskers that do everything from providing energy, balancing fluids and helping with immunity to providing structure, carrying material and regulating your body’s processes.

Normally, proteins leave your bloodstream once they finish their assigned tasks. In protein misfolding disorders, proteins take on abnormal shapes that can’t be removed and your body can’t use. In AA amyloidosis, the abnormal proteins clump together, making amyloid deposits or fibrils that collect in your organs.

Recent studies have identified dozens of medical conditions that cause AA amyloidosis. Those conditions include certain blood cancers, bacterial infections from diseases like chronic ulcers to rheumatic diseases such as rheumatoid arthritis, or inflammatory bowel diseases like Crohn’s disease or ulcerative colitis. People may inherit conditions that cause AA amyloidosis, including periodic fever syndromes such as Familial Mediterranean Fever.

What are AA amyloidosis symptoms?

AA amyloidosis often affects your kidneys. It may also affect your liver, spleen and stomach. Some common symptoms and conditions that may be signs of AA amyloidosis include:

Diagnosis and Tests

How do healthcare providers diagnose AA amyloidosis?

Healthcare providers may do a series of biopsies to diagnose AA amyloidosis:

  • “Fat pad” biopsy: Providers take a small sample of abdominal fat just under your skin.
  • Organ biopsy: Providers take a small sample of tissue from the affected organ.

Providers may do other tests to rule out AL amyloidosis, which is another amyloidosis type. AL amyloidosis and AA amyloidosis have similar symptoms, but AL amyloidosis is a more aggressive form of amyloidosis.

Management and Treatment

How do healthcare providers treat AA amyloidosis?

AA amyloidosis happens because you have a chronic inflammatory condition that caused abnormal proteins to gather in clumps on your organs. Healthcare providers treat AA amyloidosis by treating the underlying inflammatory condition. By treating the underlying condition, providers reduce the inflammation that caused AA amyloidosis. AA amyloidosis symptoms ease as inflammation levels drop.

They may also use medications that treat active AA amyloidosis. Providers and researchers are developing additional AA amyloidosis treatments that may clear amyloid fibrils from your bloodstream before the fibril can build on your organs. They’re also developing lab-made antibodies to target and clear amyloid fibrils already lodged in organs.

Prevention

Can I prevent AA amyloidosis?

You may develop AA amyloidosis if you have a chronic inflammatory disease like rheumatoid arthritis that increases the amount of a certain protein in your bloodstream. As such, the only way you can prevent AA amyloidosis is to seek timely treatment for underlying chronic inflammatory diseases.

Outlook / Prognosis

Is AA amyloidosis curable?

Yes, healthcare providers treat AA amyloidosis by treating the underlying inflammatory condition that causes AA amyloidosis. Like many illnesses, the key to managing AA amyloidosis is to diagnose and treat early on.

Living With

How do I take care of myself?

If you have AA amyloidosis, you can take care of yourself by working with your healthcare provider to treat the underlying inflammatory condition that caused AA amyloidosis. Researchers have identified dozens of diseases and conditions that cause long-lasting inflammation linked to AA amyloidosis. Ask your healthcare provider to explain how your condition caused AA amyloidosis and how they’ll treat your inflammatory condition.

A note from Cleveland Clinic

In a sense, AA amyloidosis is a very rare and serious complication to dozens of common inflammatory diseases or conditions. Fortunately, healthcare providers can treat AA amyloidosis by treating the underlying condition. Many times, AA amyloidosis symptoms may seem like common problems you’re used to managing as part of your specific inflammatory disease or condition. But like many serious illnesses, healthcare providers can do more to treat AA amyloidosis when they catch the condition in its early stages. Talk to your provider if you notice changes in your inflammatory disease or condition. They’ll be glad to evaluate your symptoms and explain what they mean.

Last reviewed by a Cleveland Clinic medical professional on 06/23/2022.

References

  • Brunger AF, Nienhuis HLA, Bijzet J, et al. Causes of AA amyloidosis: a systematic review. (https://pubmed.ncbi.nlm.nih.gov/31766892/) Amyloid. 2020 Mar;27(1):1-12. Accessed 6/23/2022.
  • Bustamante JG, Zaidi SRH. Amyloidosis. (https://www.ncbi.nlm.nih.gov/books/NBK470285/) [Updated 2022 Feb 9]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-. Accessed 6/23/2022.
  • National Organization for Rare Disorders. Amyloidosis. (https://rarediseases.org/rare-diseases/amyloidosis/) Accessed 6/23/2022.
  • Nuvolone M, Merlini G. Systemic amyloidosis: novel therapies and role of biomarkers. (https://academic.oup.com/ndt/article/32/5/770/3059474) Nephrol Dial Transplant. 2017 May 1;32(5):770-780. Accessed 6/23/2022.
  • Westermark GT, Fändrich M, Westermark P. AA Amyloidosis: Pathogenesis and Targeted Therapy. (https://www.annualreviews.org/doi/full/10.1146/annurev-pathol-020712-163913?url_ver=Z39.88-2003&rfr_id=ori%253Arid%253Acrossref.org&rfr_dat=cr_pub%253Dpubmed) Annu Rev Pathol. 2015;10:321-44. Accessed 6/23/2022.

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