Types of Amyloidosis

The types of amyloidosis are:

Primary (AL) Amyloidosis

• Primary amyloidosis is caused by the abnormal production of immunoglobulin light chains (amyloid L or AL protein) by plasma cells in the bone marrow.
• It primarily affects the heart, kidneys, liver, nerves, and gastrointestinal tract.
• Symptoms may include heart failure, kidney dysfunction, neuropathy, and gastrointestinal disturbances.

Secondary (AA) Amyloidosis

• Secondary amyloidosis occurs as a complication of chronic inflammatory or infectious diseases, such as rheumatoid arthritis, tuberculosis, or inflammatory bowel disease.
• It is characterized by the deposition of serum amyloid A protein (SAA) derived from acute-phase reactants produced in response to inflammation.
• Secondary amyloidosis commonly affects the kidneys, liver, spleen, and adrenal glands.
• Treatment involves managing the underlying inflammatory condition.

Hereditary (ATTR) Amyloidosis

• Hereditary amyloidosis results from mutations in the transthyretin (TTR) gene, leading to the production of abnormal transthyretin protein.
• It can manifest as familial amyloid polyneuropathy (FAP), familial amyloid cardiomyopathy (FAC), or familial leptomeningeal amyloidosis (FLA).
• Symptoms vary depending on the specific mutation but commonly involve peripheral neuropathy, cardiomyopathy, and gastrointestinal dysfunction.
• Treatment may include liver transplantation, gene-silencing therapies, or TTR stabilizers.

Dialysis-Related Amyloidosis

• Dialysis-related amyloidosis occurs in individuals undergoing long-term hemodialysis or peritoneal dialysis.
• It results from the accumulation of beta-2 microglobulin protein (β2M) in joints, tendons, and bones, leading to musculoskeletal symptoms such as joint pain and stiffness.
• Treatment may involve optimizing dialysis techniques and kidney transplantation to reduce β2M levels.

Senile Systemic Amyloidosis

• Senile systemic amyloidosis primarily affects older individuals, over the age of 70.
• It is characterized by the deposition of wild-type transthyretin protein, particularly in the heart.
• Senile systemic amyloidosis may lead to heart failure with preserved ejection fraction (HFpEF) and other cardiovascular complications.

Localized Amyloidosis

• Localized amyloidosis involves the deposition of amyloid protein in specific organs or tissues, without systemic involvement.
• Examples include cerebral amyloid angiopathy (amyloid deposition in cerebral blood vessels), cutaneous amyloidosis (amyloid deposits in the skin), and laryngeal amyloidosis (amyloid accumulation in the larynx).
• Treatment varies depending on the affected organ and may include surgical excision or localized radiotherapy.

Amyloidosis is a rare disease characterized by the abnormal accumulation of amyloid proteins in organs and tissues. This buildup can disrupt normal function and lead to organ damage. Amyloidosis symptoms may include weight loss, swelling, and organ dysfunction.

Amyloidosis treatment helps to manage symptoms, slow the progression of the disease, and address underlying conditions that may contribute to amyloidosis. In this article, we will study the meaning of amyloidosis, its types, stages of development, causes, symptoms, and treatment in detail.