Amyloidosis was first described in the 19th Century. Recently significant advances been made in understanding the disease.
Amyloidosis is now known to be a group of diseases in which one or more organ systems in the body accumulates protein
deposits. There are three major types, all very different from each other.
1. Primary Amyloidosis
Primary amyloidosis is a plasma cell disorder and occasionally occurs with multiple myeloma. This is the most common type
of amyloidosis in the United States and is usually treated (conventionally) with chemotherapy. Primary amyloid is not
associated with any other diseases.
In primary amyloidosis, the organs most often involved include the heart, kidneys, nervous system, and gastrointestinal tract.
Amyloid deposits in these organs cause shortness of breath, fatigue, edema (swelling of ankles and legs), dizziness upon
standing, a feeling of fullness in the stomach (especially after eating), diarrhea, weight loss, enlarged tongue, numbness of the
legs and arms, and protein in the urine.
2. Secondary Amyloidosis
Secondary amyloid is caused by a chronic infection or inflammatory disease. Treatment of the underlying chronic infection or
inflammatory disease can slow down or stop the progression of this type of amyloid.
In secondary amyloidosis, symptoms caused by the underlying chronic infection or inflammatory disease are complicated by
the development of amyloid deposits in the kidney. This may cause protein in the urine, edema, and fatigue
3. Hereditary Amyloidosis
Familial amyloid is the only type of amyloidosis that is inherited. It is rare and is found in families of nearly every ethnic
In hereditary amyloidosis, the nervous system and gastrointestinal tract are often involved. This can cause numbness and
tingling in the arms and legs, dizziness upon standing, and diarrhea. Families have their own pattern of organ involvement and
symptoms. The transmission is autosomal dominant, which means that if you have this type of amyloidosis each of your
children has a 50% chance of inheriting the disease. If your child does not inherit the gene, he/she cannot pass it on to future
4. Other types of amyloidosis include localized amyloid, b2 microglobulin amyloid, and Alzheimer’s disease. Localized
types of amyloidosis are associated with hormones, proteins, aging, and specific areas of the body, and are not found with
systemic involvment. The type of amyloidosis which is due to the b2 microglobulin protein may affect people who have been
on dialysis for a significant length of time. In Alzheimer’s disease, the amyloid protein in the brain is called the b-amyloid
Amyloidosis can only be diagnosed by a positive biopsy; that is, an identification of the amyloid deposits in a piece of tissue.