Amyloidosis Causes

NewsGuard 100/100 Score

Amyloidosis is caused by the accumulation of misfolded proteins in tissues and organs of the body. There are three main types of amyloidosis, each of which have different underlying pathologies.

Primary or AL amyloidosis

Primary amyloidosis is the most common form of the condition. This disease is associated with multiple myeloma. In multiple myeloma, the plasma cells in the bone marrow that form an important part of the immune system are affected by cancer. These plasma cells that usually produce antibodies, become dysfunctional and produce abnormal light chain immunoglobulins. The conditions is named after these amyloid light (AL) chains.

The organs and tissues commonly affected by AL amyloidosis include the heart, kidneys, nervous system, and gastrointestinal tract. The treatments used in primary amyloidosis are the same as those used to treat multiple myeloma and include chemotherapy, corticosteroids and proteasome inhibitors.

The aim of chemotherapy is to suppress the bone marrow plasma cells and prevent production of the amyloid protein. The most commonly prescribed drug regimen includes melphalan and dexamethasone, a well tolerated and effective combination that is associated with a rapid response time.

An agent called bortezomib, one of a relatively new class of drugs called the proteasome inhibitors, has been shown to be effective in the treatment of primary amyloidosis and examples of the corticosteroid medications used include lenalidomide and thalidomide.

These treatments are all aimed at slowing disease progression and prolonging survival, but none can provide a cure. However, research has now started to focus on the effects of autologous stem cell transplantation in combination with high-dose chemotherapy and so far, the results seem to be promising.

Secondary amyloidosis or AA amyloidosis

Secondary amyloidosis is usually associated with conditions of chronic inflammation such as rheumatoid arthritis or inflammatory bowel syndrome. Typically, the deposited protein is the acute phase protein serum amyloid A. Medical or surgical therapy of the underlying condition can be helpful in preventing the progression of this amyloid. For example, an antibiotic may be administered in the case of infection or an anti-inflammatory agent in the case of arthritis.

Familial amyloidosis

This rare form of amyloidosis is caused by inherited mutations in the genes that make the proteins that form fibrils. The main protein involved is the thyroid hormone-binding protein transthyretin. In most cases, the site of transthyretin production is the liver and liver transplant is an effective therapy. Two new agents called diflunisal and tafamidis are being investigated for their effectiveness in stabilizing the transthyretin and preventing it from depositing in organs.

This form of amyloidosis is inherited in an autosomal dominant fashion, meaning that every child born to an affected parent has a 50% chance of inheriting the defective gene that causes the condition.

Further Reading

Last Updated: Dec 31, 2022

Dr. Ananya Mandal

Written by

Dr. Ananya Mandal

Dr. Ananya Mandal is a doctor by profession, lecturer by vocation and a medical writer by passion. She specialized in Clinical Pharmacology after her bachelor's (MBBS). For her, health communication is not just writing complicated reviews for professionals but making medical knowledge understandable and available to the general public as well.


Please use one of the following formats to cite this article in your essay, paper or report:

  • APA

    Mandal, Ananya. (2022, December 31). Amyloidosis Causes. News-Medical. Retrieved on April 16, 2024 from

  • MLA

    Mandal, Ananya. "Amyloidosis Causes". News-Medical. 16 April 2024. <>.

  • Chicago

    Mandal, Ananya. "Amyloidosis Causes". News-Medical. (accessed April 16, 2024).

  • Harvard

    Mandal, Ananya. 2022. Amyloidosis Causes. News-Medical, viewed 16 April 2024,


The opinions expressed here are the views of the writer and do not necessarily reflect the views and opinions of News Medical.
Post a new comment

While we only use edited and approved content for Azthena answers, it may on occasions provide incorrect responses. Please confirm any data provided with the related suppliers or authors. We do not provide medical advice, if you search for medical information you must always consult a medical professional before acting on any information provided.

Your questions, but not your email details will be shared with OpenAI and retained for 30 days in accordance with their privacy principles.

Please do not ask questions that use sensitive or confidential information.

Read the full Terms & Conditions.