What Is Progeria?

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Progeria is a condition in which an affected individual experiences rapid or early aging. There are two main types of progeria; one form is diagnosed in infancy or early childhood, and the other is diagnosed in adolescence or early adulthood.

Progeria is extremely rare, affecting approximately 1 in 20 million children, and its occurrence does not vary worldwide. The other kind of progeria (Werner syndrome) occurs in about 1 in 200,000 live births in the United States but is more common in some other countries, such as Japan.

The condition affects multiple systems of the body, causing physical and metabolic abnormalities, and eventually results in a shortened lifespan.

This article will discuss the types of progeria, symptoms, prognosis, treatment, and more.

Child with progeria playing with interlocking toy

Ilknur Hicret / Getty Images

Types of Progeria

The two main types of progeria are:

In Hutchinson-Gilford progeria syndrome (HGPS), a child has no effects on development in early infancy. But between 9 and 24 months, children with HGPS start having growth delays and develop distinctive characteristics of the condition. This type is the kind most often referred to simply as progeria.

In Werner syndrome, individuals have no effects on development until puberty. Growth stops, and by the time they are in early adulthood (20s and 30s), they have conditions typically found in much older people, as well as an increased risk of cancer.

Progeria Symptoms

In HGPS, symptoms can include:

  • At birth, tight, shiny, and hardened skin over the buttocks, upper legs, and lower abdomen; bluish skin in the middle part of the face
  • Profound growth delay, causing significantly reduced height and low weight for height
  • Underdeveloped facial bones and lower jaw, causing the face to look much smaller than the head, with the front and sides of the skull typically prominent
  • Facial features include small and thin nose, significantly prominent eyes, small ears with no lobes, thin lips
  • Very little scalp hair, usually lost by 2 years of age
  • Skeletal differences
  • High-pitched voice
  • Hearing impairment

Symptoms of Werner syndrome can vary, depending on the systems of the body affected. This can include:

  • Thin build
  • Short height
  • Very little scalp hair
  • A triangular area of white hair above the forehead, called a white forelock
  • Premature aging
  • Decreased gonadal (ovary or testes) activity
  • Type 2 diabetes (about 70% of affected individuals have this at the time of diagnosis)


Progeria is a genetic disease. This means that the disease is caused by changes in genetic material. Whereas HGPS is typically not inherited (passed down from one generation to the next), Werner syndrome is.

In HGPS, there is a tiny change in a gene on chromosome 1 that codes for the protein lamin A. This results in the production of progerin protein instead of the usual lamin A.

HGPS is not typically inherited—it’s nearly always the result of a chance mutation in the gene of a person with the condition. If a person has a child with progeria, there is an increased risk of having another child with progeria because of mosaicism.

Mosaicism is when a person has a genetic mutation in only some of their cells. In HGPS, it would be a small enough fraction that they don't have the effects of the condition. If the mutation is present in the person's sperm or egg cells, it can be passed to their children.

HGPS is autosomal dominant. This means only one copy of the mutated gene (from one parent) results in the child having the condition. As the gene is not on the X or Y chromosome (the sex chromosomes), it occurs equally in people of any sex.

Werner syndrome occurs due to mutations in the WRN gene. More than 80 different mutations of the gene have been found in people with Werner syndrome. This gene normally produces Werner protein, which helps maintain, repair, and replicate DNA in cells throughout the body. Abnormal Werner protein does not work effectively.

Werner syndrome follows an autosomal recessive inheritance pattern. The affected individual inherits an abnormal gene from both parents.

If one parent has an abnormal gene and one doesn’t, the child will be a carrier for the disease but have no symptoms. With each pregnancy, there is a 25% risk of having an affected child if both parents are carriers. As the WRN gene is not on the X or Y chromosome, it occurs equally in people of any sex.


A diagnosis of progeria is confirmed with genetic testing. This often happens once the affected individual starts showing characteristic signs of progeria.

With HGPS, signs of progeria that lead to diagnosis occur early in life. But Werner syndrome may not be diagnosed until a person is in their 20s or 30s when they present with conditions usually seen in much older individuals.


Zokinvy (lonafarnib) was approved in November 2020 by the Food and Drug Administration (FDA) to treat HGPS. It is the first approved treatment for HGPS. Treatment with this drug has been associated with lower mortality rate (rate of death) when compared to those not on the drug.

In addition to this drug, treatment also consists of treating the specific effects of the disease each person has. This can mean treatment for conditions like osteoporosis (thinning of the bones) or heart disease.

For those with Werner syndrome, the treatment is specific to the symptoms in the individual. A team of specialists may be warranted to treat different things like bone and muscle or joint issues, diabetes, cataracts (clouding of the lens of the eye), and atherosclerosis (hardening of the arteries).

Werner syndrome increases cancer risk. Care for the condition includes regular screening tests for hyperlipidemia (high amounts of fatty substances in the blood), cataracts, cancer, and diabetes.


There is no cure for progeria, and those with progeria do have a significantly shorter life expectancy. While there are treatments, nothing stops the progression of the disease.

For those with HGPS, mortality is usually the result of heart failure. A study of 258 people with the condition found an average age at death is 14.6 years of age. The oldest survivor in that study died before age 28.

Individuals with Werner syndrome usually live into their 40s and 50s, with their cause of death most often being cancer or atherosclerosis. But there may be an improvement in life span for those with this condition.

A Japanese survey tracking patients from 2011 to 2020 found the average age of death of people with Werner syndrome was 59 years, and one person in the study survived to age 76. In this group, no deaths were seen from atherosclerosis, while cancer was the leading cause of death.


A diagnosis of progeria, no matter what type, can be emotionally challenging for both caregivers and the individual. It’s important for everyone involved to get support in order to provide care with less risk of burnout and to address emotional needs.

Support groups can provide helpful advice on living with progeria or caring for someone with the condition. It can also be helpful to be around people who have a personal understanding of living with or caring for a person with the condition.

The Progeria Research Foundation has several chapters that can provide information and support. Talk with your treatment team about your support needs, and ask if they are aware of any local resources or support groups.

A therapist can also be helpful and provide much-needed support and coping tools following a diagnosis of progeria.


Progeria is a progressive, life-threatening condition. The two main types of progeria are Hutchinson-Gilford progeria syndrome and Werner syndrome, diagnosed in early childhood and adolescence/early adulthood, respectively.

They are genetic conditions for which there is no cure, but there are treatments available. For those with HGPS, the prognosis is fairly poor, with an average life span of under 15 years. Those with Werner syndrome have a longer life expectancy—into their 40s and 50s—but this is still significantly early mortality.

A Word From Verywell

It can be psychologically stressful to be diagnosed with progeria or to have a child diagnosed with it. Taking care of your mental and emotional health is just as important as your physical health, and professional help may be necessary, in addition to any local lay support groups.

6 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read our editorial process to learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
  1. National Organization for Rare Disorders. Hutchinson-Gilford progeria syndrome.

  2. MedlinePlus. Werner syndrome.

  3. Genetic and Rare Diseases Information Center. Werner syndrome.

  4. Gordon LB, Shappell H, Massaro J, D'Agostino RB, Brazier J, et al. Association of lonafarnib treatment vs no treatment with mortality rate in patients with Hutchinson-Gilford progeria syndrome. JAMA. 2018;319(16):1687-1695. doi:10.1001/jama.2018.3264

  5. American Society for Clinical Oncology. Werner syndrome.

  6. Kato H, Koshizaka M, Kaneko H, Maezawa Y, Yokote K. Lifetime extension and the recent cause of death in Werner syndrome: a retrospective study from 2011 to 2020. Orphanet J Rare Dis. 2022;17(1):226. doi:10.1186/s13023-022-02383-w