Rare types of diabetes are less common forms of diabetes caused by genetic changes, autoimmune conditions, pancreatic disorders, medications or other underlying health factors. Rare types of diabetes affect an estimated 1.5–2% of people living with diabetes worldwide.
Unlike type 1 diabetes, type 2 diabetes and gestational diabetes, rare forms of the condition often require specialised diagnosis and treatment. Some develop during infancy or early adulthood, while others result from medical conditions, rare genetic disorders or medications.
What are rare types of diabetes?
Rare forms of diabetes include a diverse group of conditions that affect how the body produces or uses insulin. They have characteristics that distinguish them from type 1 and type 2 diabetes.
Unlike type 1 diabetes, not all rare diabetes types are autoimmune conditions. Unlike type 2 diabetes, many are not primarily caused by insulin resistance or lifestyle factors.
Rare types of diabetes may develop because of:
- Genetic mutations
- Autoimmune conditions
- Pancreatic disorders
- Hormonal conditions
- Medications
- Other underlying health conditions
Some rare forms of diabetes are inherited and can run in families, while others develop because of illness, treatment or environmental factors.
Rare forms of diabetes affect an estimated 1.5–2% of people living with diabetes worldwide. They are often under-recognised or misdiagnosed.
Why rare types of diabetes matter
Although less common, rare forms of diabetes can have a significant impact on health and quality of life.
Many people living with rare forms of diabetes experience:
- Delayed diagnosis
- Misdiagnosis as type 1 or type 2 diabetes
- Limited access to specialised care
- Inappropriate treatment plans
- Increased risk of complications
Improving awareness of rare diabetes types is important to ensure earlier diagnosis, more personalised care and better long-term outcomes.
Types of rare diabetes
Alström Syndrome
Alström Syndrome is a rare genetic condition associated with:
- Insulin resistance
- Type 2 diabetes
- Obesity
- Vision loss
- Hearing impairment
Symptoms often begin in childhood and can affect multiple organs and body systems.
Diabetes insipidus
Diabetes insipidus is a rare condition that affects the body’s ability to regulate fluid balance, leading to:
- Excessive thirst
- Frequent urination
- Dehydration risk
LADA (Latent Autoimmune Diabetes in Adults)
LADA is a form of autoimmune diabetes that develops in adulthood and progresses more slowly than type 1 diabetes.
MODY (Maturity-Onset Diabetes of the Young)
MODY is a rare inherited form of diabetes caused by mutations in a single gene affecting insulin production. It usually develops around age 30 and younger. It usually runs in families across several generations.
Unlike type 1 diabetes, MODY is not usually an autoimmune condition. Unlike type 2 diabetes, it is not primarily linked to insulin resistance or lifestyle factors.
Depending on the form of MODY, some can be managed without insulin, while others may require tablets or insulin.
Neonatal diabetes
Neonatal diabetes develops within the first six months of life and is usually caused by genetic mutations that affect insulin production.
It differs from type 1 diabetes and may be temporary or permanent depending on the underlying genetic cause.
Genetic testing is usually recommended for infants diagnosed with diabetes before six months of age.
Secondary diabetes
Secondary diabetes develops due to other medical conditions, such as cystic fibrosis, pancreatitis or corticosteroid use,hormornal disorders or medications.
Steroid-induced diabetes
Steroid-induced diabetes can develop in people taking corticosteroid medications, particularly over long periods or at high doses.
Steroids can increase blood glucose levels and reduce how effectively insulin works in the body.
Some people are at greater risk, especially those with:
- Prediabetes
- Obesity
- Family history of diabetes
- Existing insulin resistance or other type 2 diabetes risk factors
Monitoring blood glucose levels during steroid treatment is important.
Wolfram Syndrome
Wolfram Syndrome is a rare genetic disorder associated with diabetes, diabetes insipidus, vision loss and hearing impairment.
The condition is also known as DIDMOAD (Diabetes Insipidus Diabetes Mellitus Optic Atrophy Deafness)
Type 3c diabetes
Type 3c diabetes is also known as pancreatogenic diabetes. It is a form of diabetes caused by structural or physical damage to the pancreas, which impairs the body’s ability to produce insulin.
Diagnosing rare forms of diabetes
Diagnosing rare forms of diabetes can be challenging because symptoms often resemble those of type 1 or type 2 diabetes.
Accurate diagnosis may require:
- Blood glucose testing
- Autoantibody testing
- Genetic testing
- Family history assessment
- Pancreatic function testing
- Specialist medical review
Early and accurate diagnosis is important because treatment plans for rare diabetes types may differ significantly from those used for type 1 or type 2 diabetes.
Managing rare forms of diabetes
Managing rare forms of diabetes requires personalised care based on the specific condition and individual needs.
Treatment approaches may include:
- Insulin therapy
- Oral diabetes medications
- Nutritional support
- Blood glucose monitoring
- Treatment of underlying medical conditions
- Specialist healthcare support
Some rare diabetes types can be managed without insulin, while others require lifelong insulin therapy.
