Amyloidosis

Amyloidosis

Pervez Musharraf dies of Amyloidosis

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For Prelims

Amyloidosis

  • Former Pakistan president Pervez Musharraf passed away on Sunday (05-02-23) from amyloidosis for which he had been undergoing treatment since March 2016.
  • Amyloidosis is a rare disease that occurs when a protein called amyloid builds up in organs which results in the organs not work properly.
  • It can affect the heart, kidneys, liver, spleen, nervous system and digestive tract.
  • While some types of amyloidosis may improve with treatment, some may lead to life-threatening organ failure.

Types of Amyloidosis

The most common types of amyloidosis are:

AL (Primary) Amyloidosis.
  • AL refers to “amyloid light chains,” which is the kind of protein responsible for causing the ailment.
  • This is the most common type of amyloidosis and used to be called primary amyloidosis.
  • It happens when your bone marrow makes abnormal antibodies that can’t be broken down however there is no known cause for this.
AA (Secondary) Amyloidosis.
  • This condition is the result of another chronic infectious or inflammatory disease such as rheumatoid arthritis, Crohn’s disease, or ulcerative colitis and used to be known as secondary amyloidosis.
  • Even though it mostly affects your kidneys, it can also affect your digestive tract, liver, and heart.
  • AA refers to the amyloid type A protein which causes this disease.
Dialysis-related amyloidosis (DRA).
  • This form of amyloidosis is caused by deposits of beta-2 microglobulin that build up in the blood and is more common in older adults and people who have been on dialysis for more than 5 years.
  • It most commonly affects bones, joints, and tendons but deposits can build up in many different tissues.
Familial, or hereditary, amyloidosis.
  • This is a rare form passed down through families and often affects the liver, nerves, heart, and kidneys.
  • Many genetic defects are linked to a higher chance of amyloid disease however, an abnormal protein like transthyretin (TTR) is usually the cause.
Age-related (senile) systemic amyloidosis.
  • This is caused by deposits of normal TTR in the heart and other tissues and happens most commonly in older men.

Symptoms

Symptoms of amyloidosis may include:

  • Severe fatigue and weakness
  • Shortness of breath
  • Numbness, tingling, or pain in the hands or feet
  • Swelling of the ankles and legs
  • Diarrhea, possibly with blood, or constipation
  • An enlarged tongue, which sometimes looks rippled around its edge
  • Skin changes, such as thickening or easy bruising, and purplish patches around the eyes

Treatment

  • Treatments include chemotherapy with strong drugs used to treat cancer or medications can reduce amyloid production and control symptoms.
  • While some may benefit from organ or stem cell transplants.

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