What is Amyloidosis?

Amyloidosis is a variety of conditions in which the body produces bad proteins called amyloids, gets deposit up in the organs and tissues and cause harm to the organs. The buildup may occur in a single organ or throughout the body.

Condition frequently affects liver, heart, spleen, the gastrointestinal tract and nervous system. Major symptoms include weakness, leg and ankle swelling, shortness of breath, tingling in hands or feet and easy bruising.
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It is a very rare disease and its exact origin is unknown. The treatment options available are used to manage the signs and symptoms and can stop the progression of the condition by reducing the production of amyloid protein

Symptoms:

Symptoms depend on the organ of the body affected. Common signs and symptoms may include:

• Swelling of your ankles and legs
• Weakness
• Significant weight loss
• Shortness of breath
• Numbness or tingling in your hands or feet
• Diarrhea or constipation
• Feeling full quickly
• Severe fatigue
• An enlarged tongue (macroglossia)
• Skin changes, such as thickening or easy bruising
• Purplish patches (purpura) around the eyes
• An irregular heartbeat
• Difficulty swallowing
• Protein in the urine (detected by urine tests)

Causes:

Amyloidosis is broadly classified into three categories. These are:

• Primary amyloidosis: This is the most common form of amyloidosis, which can affect many areas, including heart, kidneys, liver, spleen, nerves, intestines, skin, tongue and blood vessels. Primary amyloidosis isn't associated with other diseases, except for multiple myeloma, a form of bone marrow cancer.

Though the exact cause of primary amyloidosis id not known yet, it is known that the disease begins in the bone marrow. One marrow produces red blood cells, white blood cells, platelets and antibodies, proteins that protect the body against infection and disease. The body recycles these antibodies Amyloidosis occurs when cells in the bone marrow produce antibodies that can't be broken down. These antibodies then build up in the bloodstream. Gradually, they leave the bloodstream and gets deposit in the tissues as amyloid, interfering with normal function.

• Secondary amyloidosis: This occurs in association with chronic infectious or inflammatory diseases, such as tuberculosis, inflammatory arthritis or osteomyelitis, a bone infection. Though other organs may be involved, it primarily affects the kidneys, spleen, liver and lymph nodes. Progression of this form of amyloidosis may be stopped by treating the underlying disease.
• Hereditary (familial) amyloidosis: As the name implies, this form of amyloidosis is inherited. This type often affects the liver, nerves, heart and kidneys.
• Dialysis-related amyloidosis (DRA): This form of amyloidosis develops when proteins in blood are deposited in joints and tendons, causing pain, stiffness and fluid in the joints, as well as carpal tunnel syndrome. The condition is most likely to affect people on long-term hemodialysis.

Risk factors:

Anyone can get amyloidosis, but several factors may increase the risk of developing the condition. Risk factors may include: Consult to Endocrinologist in Gurgaon and Endocrinologist in Faridabad for best treatment.

• The risk of amyloidosis increases as the age increase. The majority of people who develop the disease are older than 60 years of age.
• Chronic infectious or inflammatory disease may put the person at a greater risk.
• People who have multiple myeloma a form of bone marrow cancer are also at increased risk of amyloidosis.
• Often cases of amyloidosis are inherited.
• Kidney dialysis for kidney diseases increases the risk of dialysis associated amyloidosis because dialysis can't remove large proteins from the blood, so large, abnormal proteins may build up and deposit in surrounding tissues

Diagnosis:

The doctor may need to conduct various tests to rule out other condition. The diagnosis may begin by conducting a physical exam. He may evaluate the symptoms and the medical history of the patient. The doctor may also take a look of the family history of health conditions. Consult to Endocrinologist in Noida and Endocrinologist in Ghaziabad for best treatment.

Tests:

The doctor may order certain tests to confirm the diagnosis and rule out various health conditions. The tests may include:

• Blood test
• Urine test

If blood or urine tests detect an abnormal protein which could indicate amyloidosis, the doctor may order a tissue biopsy to make a definitive diagnosis.

• Biopsy

In a tissue biopsy, the doctor uses a needle to remove a small sample of tissue and then examined under microscope in a laboratory to check for signs of amyloid.

If the doctor suspects that the patient may have systemic amyloidosis or it affects several parts of the body rather than one organ, the sample may be taken from the abdominal fat, bone marrow, gums, salivary glands, skin or rectum.

Occasionally, tissue samples may be taken from other parts of the body, such as heart, liver or kidney, to help diagnose the specific organ affected by amyloidosis. These procedures may require hospitalization.

Treatment:

There is no specific way to cure amyloidosis. The treatment focuses on minimizing the symptoms and reducing the production of amyloid proteins through dietary changes and medications.

Treatment involves decreasing the proteins that can make up amyloid. Chemotherapy is used for the treatment of primary amyloidosis.

Depending on the organs that are affected, the specialist may ask the patient to follow a special diet.

There is no cure for secondary amyloidosis, but medication can improve survival. The treatment involves curing the underlying condition. Stem cell transplants are also a treatment for secondary amyloidosis.

A liver transplant may be a necessity for hereditary amyloidosis.

Anti-inflammatory/immune suppressive therapy to reduce amyloid precursor load may be recommended for managing the symptoms.

Drugs:

Drugs which can be prescribed by the doctor may include:

For secondary amyloidosis a combination of prednisone (a corticosteroid) and melphalan (Alkeran, also used to treat some kinds of cancer) is used.

To provide relief from the symptoms the doctor may prescribe:

• Diuretics to relieve swelling caused by fluid retention
• Anti-arrhythmics to control heart rhythm
• Metoclopramide to help empty food from the stomach
• Antibiotics to control bacteria that may cause diarrhea or prevent the body from absorbing nutrients

Alternatives:

Dietary choices, supplements, and herbs that reduce inflammation may help prevent amyloidosis. Damage from oxidation may play a role in the development of amyloidosis so add antioxidants to the diet to help slow the disease.

Though herbal and natural treatment options are available, Amyloidosis should never be treated with complementary and alternative therapies alone. Consult the doctor about any medications, herbs, or supplements before taking

Herbs which may be useful as a supportive therapy to standard medical care in treating amyloidosis:

• Pycnogenol (Pinus pinaster)
• Gingko (Gingko biloba)

Complication:

Complications depend on which organ the amyloid deposits disease affects. It can be life threatening.

• Kidney damage
• Heart damage
• Nervous system damage

If amyloid deposits affect the nerves that control your bowel function, you may experience periods of alternating constipation and diarrhea. Sometimes the condition affects nerves that control blood pressure, and you may experience dizziness or near fainting when standing too quickly as a result of a drop in your blood pressure.

Prevention:

There is no specific way to prevent amyloidosis however measures can be taken to manage the signs and symptoms and provide relief.

• The patient should have proper knowledge about the disease so that he knows his limitations and can pace his life according to that.
• Follow a balanced diet to provide body with an adequate energy supply.
• Avoid caffeine, refined sugars, dairy products and processed foods and should
• Eat more fresh fruits, vegetables, nuts and cold water fish.