Amyloidosis

5 mins read

Introduction:
Amyloidosis is a rare condition that involves build up of amyloid, an abnormal protein. It is usually produced in bone marrow and is capable of being present in any organ of a patient. The type of amyloidosis is dependent of the production of a particular amyloid. It can damage any particular organ or entire body. It is usually known to affect the heart, kidneys, liver, spleen, nervous system and digestive tract. Severe amyloidosis can lead to life-threatening organ failure. If untreated, amyloidosis can prove to be fatal to health.

Causes & Types Of Amyloidosis
Several proteins can cause formation of amyloid deposits, but only a few have been linked to significant health problems. The type of protein and its location helps in ascertaining the type of amyloidosis in a patient. Amyloidosis is defined by the type of protein associated with it. Several types of amyloidosis are known to exist:

Primary (Systemic AL) Amyloidosis: This occurs without a known cause, but it has been seen in people with a blood cancer called multiple myeloma. This is the most common type of amyloidosis.

Secondary (Systemic AA) Amyloidosis: This is the result of a chronic inflammatory disease, such as lupus, rheumatoid arthritis, tuberculosis, inflammatory bowel disease (Crohn’s disease and ulcerative colitis), and certain cancers. It usually affects the spleen, kidneys, liver, adrenal gland, and lymph nodes. AA denotes the amyloid type A protein that causes this type of amyloidosis.

Dialysis-Related Amyloidosis (DRA):  This is more common in older adults and people undergoing dialysis for more than 5 years. This form of amyloidosis occurs due to deposits of beta-2 microglobulin produced in the blood. Deposits can occur in many different tissues, but generally affects bones, joints, and tendons.

Familial, Or Hereditary, Amyloidosis (AF): As the name suggests, it is an inherited form, caused by production of abnormal amyloid transthyretin (TTR) protein, made in the liver. This protein is responsible for most common forms of hereditary amyloidosis.

Senile Systemic Amyloidosis (SSA): It is commonly observed in older men and is caused due to deposits of TTR protein in heart and other tissues.

Organ-Specific Amyloidosis: This is caused due to deposition of amyloid protein in single organs, including the skin (cutaneous amyloidosis).

Few types of amyloid deposits are also associated with Alzheimer’s disease.

Diagnosis
A methodical physical examination and an elaborate account of one’s medical history play a key role in diagnosis of amyloidosis. One cannot detect Amyloidosis through blood. State-of-art laboratory devices including electrophoresis or free light chain assays help in revealing onset of amyloid proteins.

Confirmatory test for Amyloidosis calls for a biopsy to find specific protein involved in producing the disease. The sample of tissue for biopsy can be taken from the abdominal fat pad, mouth, rectal, or other involved organs. It is not mandatory to conduct biopsy on body part damaged by the amyloid deposits.

Genetic testing is recommended when the disease is speculated to be inherited. Like diagnosis, the treatment for hereditary amyloidosis also differs from that required for other forms of disease.

Functioning of organ is done through blood, urine, and imaging tests.

Treatment
So far, no surefire cure exists for amyloidosis. Health care specialist may prescribe treatments to curb the development of the amyloid-forming protein, and to control the symptoms. If amyloidosis is a result of another condition, then treatment will include treating that underlying condition. A combination of prednisone (a type of corticosteroid) and cancer treating drugs such as melphalan are used for treatment.

Life saving treatment includes high-dose chemotherapy, prescription of anti-inflammatory medicines, organ transplant, dialysis or implantation of pacemaker.

To relieve intensity of symptoms, diuretic medicines are advised for removal of excess water from the body. Compression stockings are used to heal swelling in the legs or feet. Changes in diet are prescribed to those with gastrointestinal amyloidosis.

Alternative Medicine
Dietary restrictions include restriction of meat, as amyloidosis incidence is likely to increase from amyloid enhancing factor (AEF) present in the diseased animal. Fish oil supplements help in reducing painful rheumatoid arthritis. It is advisable to avoid refined foods, sugar and dairy to avoid inflammation. Instead diet rich in whole grains, fresh fruits, vegetables, seeds, nuts and fish is recommended. Bromelain an anti-inflammatory compound found in pineapple is a naturally occurring pain killer. Resveratrol found in grape skin is a powerful antioxidant, which reduces free radical production. Quercetin and Glutathione are two types of antioxidants helpful for this condition.

Among herbs, Pinus pinaster (Maritime French Pine) derived Pycnogenol protect cells from radical damage. Extracts of Ginkgo (Ginkgo biloba) also contain flavonoids. It has been suggested as a treatment for Alzheimer’s disease. Considering the connection between Alzheimer and amyloid deposits, gingko may help treat amyloidosis. However, Gingko may be unsuitable for people with certain conditions, such as diabetes, seizures, bleeding disorders, or those actively trying to get pregnant.

 

References
1. National Amyloidosis Centre: “Amyloidosis Overview.”

2. MedLine Plus: “Amyloidosis.”

3. National Kidney and Urologic Diseases Clearinghouse: “Amyloidosis and Kidney Disease.”

4. American Society of Clinical Oncology: “Amyloidosis.”

5. Amyloidosis Foundation: “Amyloidosis Information: A General Overview for Patients.”

6. Stankovic K, Grateau G. Amyloidosis AA. Nephrol Ther. 2008;4(4):281-7.

7. Taal. Brenner and Rector’s The Kidney, 9th ed. Philadelphia, PA. Saunders. An Imprint of Elsevier; 2011.

8. Beers MH, Porter R, eds. The Merck Manual of Diagnosis and Therapy. 18th ed. Whitehouse Station, NJ: Merck Research Laboratories; 2006:1310-12.

9. Okuda Y, Yamada T, Matsuura M, Takasugi K, Goto M. aging: a risk factor for amyloid A amyloidosis in rheumatoid arthritis. Amyloid. 2011:18(3):108-11.

10. Lebrazi H, Hachulla E, Saile R. Treatments for amyloidosis beyond symptomatic care [in French].Rev Med Interne. 2000: 21(3):247-255.

 

 

 

 

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