Primary systemic amyloidosis is a rare, progressive, and incurable disease caused by the buildup of abnormal proteins called amyloid in the organs and tissues of the body. It is a type of amyloidosis, a group of diseases caused by the buildup of abnormal proteins in the body. Symptoms of primary systemic amyloidosis can vary depending on the organs affected, but may include fatigue, weight loss, an enlarged liver or spleen, and swelling in the legs and feet. Treatment typically involves medications to reduce symptoms and slow the progression of the disease.

The most common symptoms of primary systemic amyloidosis include:

-Fatigue

-Weight loss

-Enlarged tongue
-Shortness of breath
-Swelling in the legs and abdomen
-Numbness or Tingling in the hands and feet
-Heart problems, such as an irregular heartbeat or heart failure
-Kidney problems, such as protein in the urine or kidney failure
-Gastrointestinal problems, such as diarrhea, constipation, or abdominal pain
-Skin changes, such as thickening or reddening of the skin
-Neurological problems, such as confusion, memory loss, or difficulty speaking

Primary systemic amyloidosis is caused by a genetic mutation that leads to the production of an abnormal protein called amyloid. This protein accumulates in the organs and tissues of the body, leading to organ dysfunction and other symptoms. In some cases, the cause of the mutation is unknown. In other cases, the mutation is caused by a genetic disorder such as familial Mediterranean fever, multiple myeloma, or rheumatoid arthritis.

The main treatment for primary systemic amyloidosis is chemotherapy. This is usually done with a combination of drugs, such as melphalan and dexamethasone. Other treatments may include stem cell transplantation, radiation therapy, and monoclonal antibody therapy. In some cases, medications such as colchicine or thalidomide may be used to reduce the amount of amyloid in the body. In addition, supportive care such as blood transfusions, dialysis, and medications to treat symptoms can be used to help manage the condition.

The exact cause of primary systemic amyloidosis is unknown, but there are several risk factors that may increase a person’s risk of developing the condition. These include:

• Age: Primary systemic amyloidosis is more common in people over the age of 60.

• Gender: Men are more likely to develop primary systemic amyloidosis than women.

• Family history: People with a family history of the condition are more likely to develop it.

• Certain medical conditions: People with certain medical conditions, such as rheumatoid arthritis, multiple myeloma, and certain types of cancer, are more likely to develop primary systemic amyloidosis.

• Exposure to certain toxins: Exposure to certain toxins, such as asbestos, may increase the risk of developing primary systemic amyloidosis.

Primary systemic amyloidosis is a rare and serious condition that is caused by abnormal proteins called amyloid proteins building up in the body's organs and tissues. There is currently no cure for primary systemic amyloidosis, but treatments are available to help manage the symptoms and slow the progression of the disease. Medications such as chemotherapy, corticosteroids, and immunosuppressants may be used to reduce inflammation and slow the progression of the disease. Other treatments such as stem cell transplants, plasmapheresis, and monoclonal antibody therapy may also be used to help manage the symptoms.