Statistics of Amyloidosis

Executive Summary:

The exact statistics of amyloidosis among the affected individuals are not predicted as amyloidosis is unusual. Approximately, 4000 individuals have Amyloidosis in the United States every year. Amyloid light-chain amyloidosis is the most common type of amyloidosis, and in addition, the disease is prevalent among individuals who are between 50-65 years of age. On the contrary, 20 years old people have cases too. The amyloidosis is common among men and women. It affects approximately 60% of the population. 5% of the population have AA or secondary amyloidosis. Also, Familial amyloidosis is prevalent among the Caucasians in the United States and African American population. 

Amyloidosis statistics

Based on the statistics of Amyloidosis, It is a rare disease condition. The disease is seen as a result of amyloid protein deposits in tissues and organs. Even though it is rare, it is a severe disorder. The deposits affect the normal functioning of the affected organs, which eventually results in organ failure. 

Since the disease condition is rare and not widely prevalent, studies and research are still learning more about the disease condition. Obtaining exact statistics of people affected with amyloidosis is impossible due to the unusual nature of the disorder. In the United States, Amyloidosis is commonly diagnosed in around 4000 people in every year. And the most common type diagnosed among patients is the AL or Amyloid Light chain amyloidosis. The disease is prevalent among people belonging to the age group of 50 to 65. While there are some cases also diagnosed among young people around 20 years of age.

Prevalence: Amyloidosis is considered a rare disease. The exact prevalence rates vary depending on the specific type of amyloidosis and geographic region. Estimates suggest that amyloidosis affects 2 to 12 people per 100,000 individuals in the general population.

Primary Systemic Light-chain (AL) Amyloidosis: AL amyloidosis is the most common type of systemic amyloidosis. It is estimated to occur in approximately 8 to 12 individuals per million people each year.

Transthyretin (ATTR) Amyloidosis: ATTR amyloidosis has two forms – hereditary (hATTR) and wild-type (wtATTR). hATTR amyloidosis is more prevalent in certain geographic regions, such as Portugal, Sweden, and Japan, where specific mutations are more common. The prevalence of hATTR amyloidosis varies across populations, ranging from 1 in 1,000 to 1 in 100,000 individuals. wtATTR amyloidosis typically affects older individuals and is more prevalent in males.

Dialysis-Related Amyloidosis: Dialysis-related amyloidosis, primarily associated with beta-2 microglobulin (β2M) amyloid deposits, occurs in a significant proportion of individuals undergoing long-term hemodialysis. The prevalence of dialysis-related amyloidosis has decreased over the years due to improvements in dialysis techniques.

Amyloidosis Probability

Although the incidence of amyloidosis cases among men and women are somewhat equal, around 60% of patients who seek treatment for amyloidosis are men. Although, AL amyloidosis is the most prevalent type of amyloidosis ​1​ , it is quite rare and people with chronic inflammatory diseases like juvenile arthritis, psoriatic arthritis, rheumatic arthritis, familial Mediterranean fever, inflammatory bowel disease or ankylosing spondylitis (found in children) are at a high risk of developing AA amyloidosis. People with other infectious diseases like leprosy, tuberculosis, bronchiectasis, chronic pyelonephritis and chronic osteomyelitis are also at risk. Around 5% of individuals are have AA and secondary amyloidosis.

The probability of Familial amyloidosis caused by the protein transthyretin is approximately around one in 100,000 among the Caucasians in the United States. And around 4% among the African American population. People have familial amyloidosis in Sweden, Portugal, Japan, Spain, Ireland, France, Germany, Greece and Finland ​2,3​ too. The disease symptoms usually appear around 40-65 years of age.

AL amyloidosis is more common when compared to AA and Familial Amyloidosis. But ATTR amyloidosis is more prevalent, but most cases remain undiagnosed. 

The rate of AL amyloidosis (being the most prevalent form) depends on various factors like how sooner or later the disease is diagnosed, how it has affected the heart, what treatment strategy has been implemented, and how it is working.

It is essential to note that the figures mentioned in this article concerning amyloidosis are an estimate. It can vary as time progresses. Patients should talk with their doctor and healthcare team to get the latest disease, diagnosis, and treatment information.    

References

  1. 1.
    Kyle RA, Larson DR, Kurtin PJ, et al. Incidence of AL Amyloidosis in Olmsted County, Minnesota, 1990 through 2015. Mayo Clinic Proceedings. Published online January 2019. doi:10.1016/j.mayocp.2018.08.041
  2. 2.
    Saatçi Ü, Ozen S, Özdemir S, et al. Familial Mediterranean fever in children: report of a large series and discussion of the risk and prognostic factors of amyloidosis. European Journal of Pediatrics. Published online July 25, 1997:619-623. doi:10.1007/s004310050677
  3. 3.
    Mukhin NA, Kozlovskaya LV, Bogdanova MV, Rameev VV, Moiseev SV, Simonyan AKh. Predictors of AA amyloidosis in familial Mediterranean fever. Rheumatol Int. Published online January 14, 2015:1257-1261. doi:10.1007/s00296-014-3205-x