This non-contagious disease is characterized by the formation of protein deposits that alter the normal functioning of organs. It most often progresses to a severe form and can affect all organs. We do not know precisely the total number of people affected in France by this rare disease, but it is estimated that it affects 1 in 100 people. (000) Each year in France, around 1 new cases of AL amyloidosis are diagnosed. the less rare form of the disease.

Amyloidosis deposits can potentially appear in almost any tissue and organ in the body. Amyloidosis most often progresses to a severe form by gradually destroying the affected organs: the kidney, heart, digestive tract and liver are the organs most often affected. But amyloidosis can also affect bones, joints, skin, eyes, tongue …

Therefore, the range of symptoms is very wide: discomfort and shortness of breath when the heart is involved, edema of the legs with regard to the kidney, drop in muscle tone when peripheral nerves are affected, diarrhea / constipation and obstruction in the case of the kidney. digestive tract, etc. Acute symptoms such as shortness of breath, diarrhea and bleeding should alert and lead to screening for amyloidosis.

Amyloidosis is caused by changing the molecular structure of a protein, making it insoluble in the body. The protein then forms molecular deposits: the amyloid substance. It is more correct to speak of amyloidosis than of amyloidosis, because although this disease has the same cause, its expressions are very diverse. In fact, around twenty proteins are responsible for so many specific forms. However, three types of amyloidosis predominate: AL (immunoglobulinic), AA (inflammatory) and ATTR (transthyretin).

• AL amyloidosis results from the multiplication of certain white blood cells (plasma cells). They produce antibodies (immunoglobulins) which will aggregate and create deposits.
• AA amyloidosis occurs when chronic inflammation causes a high production of SAA proteins which will form amyloidosis in the tissues.
• The transthyretin protein is involved in ATTR amyloidosis. This form of the disease is genetic. An affected parent has a 50% risk of passing the mutation on to their child.

Amyloidosis is not contagious. Epidemiological data clearly indicate that the risk increases with age (amyloidosis is often diagnosed around 60-70 years). People with infectious or inflammatory disease, as well as those who have undergone prolonged dialysis, are more vulnerable. As some forms of amyloidosis, such as ATTR amyloidosis, are linked to a hereditary genetic mutation, families in which one or more members have been affected must be the subject of special monitoring.

To date, there is no means of prevention to fight against amyloidosis. The treatments all consist in reducing the production of the toxic protein which is deposited and aggregated in the tissues, but they vary according to the type of amyloidosis:

• by chemotherapy in the case of AL amyloidosis. The drugs used in his treatment show ” of real efficiency », Affirms the National Society of Internal Medicine (SNFMI), while specifying that the cardiac attack remains serious.
• using powerful anti-inflammatory drugs to fight the inflammation that caused AA amyloidosis.
• For ATTR amyloidosis, a transplant may be necessary to replace a liver colonized with the toxic protein.

The French Association against Amyloidosis publishes a lot of information about this disease and its treatments, intended for patients and their relatives.