OVERVIEW

What is fulminant type 1 diabetes?

Fulminant type 1 diabetes was first reported in 2000 by Japanese scholar Imagawa A et al. It is an acute-onset form of type 1 diabetes and a critical emergency condition. Without prompt treatment, it can often be life-threatening.

The hallmark of this disease is severe destruction of insulin-producing pancreatic β-cells, leading to rapid hyperglycemia and diabetic ketoacidosis. Diabetes-related antibodies are often negative, while pancreatic enzymes like amylase are significantly elevated. Pregnant women are a high-risk group for this condition, and contracting it during pregnancy may result in miscarriage or fetal death.

The exact pathogenesis of fulminant type 1 diabetes remains unclear but may be associated with HLA genotypes, autoimmunity, and viral infections. In women, pregnancy may also be a contributing factor.

Due to its severe and rapid progression, suspected or confirmed cases require immediate hospitalization for insulin therapy and intravenous fluids to control blood sugar and correct ketoacidosis.

It should be noted that individuals previously diagnosed with type 2 diabetes are not exempt from the possibility of developing fulminant type 1 diabetes later.

Is fulminant type 1 diabetes common?

Fulminant type 1 diabetes is not a common disease. A survey of hospitalized diabetic patients by China's Xiangya Hospital of Central South University found its incidence to be approximately 1.24‰. Among newly diagnosed type 1 diabetes cases, about 10% are fulminant type 1 diabetes.

Is fulminant type 1 diabetes the same as type 1 diabetes?

Fulminant type 1 diabetes is a distinct subtype of type 1 diabetes. Beyond fulminant type 1 diabetes, type 1 diabetes includes other variants.

According to the WHO classification, type 1 diabetes is divided into immune-mediated (type 1A) and idiopathic (type 1B). Fulminant type 1 diabetes falls under the type 1B category.

SYMPTOMS

How does fulminant type 1 diabetes develop?

Fulminant type 1 diabetes is a severe condition characterized by rapid and aggressive progression.

Most patients experience respiratory or gastrointestinal infection symptoms within one week before onset, followed by rapid development of hyperglycemia and diabetic ketoacidosis.

Patients with fulminant type 1 diabetes often have no prior history of diabetes, making early diagnosis challenging. Therefore, checking blood glucose levels during severe upper respiratory infections or acute gastroenteritis is an effective way to identify this condition promptly.

What are the common symptoms and manifestations of fulminant type 1 diabetes?

• Early warning signs: Some patients may experience flu-like symptoms such as fever, sore throat, cough, or headache before onset, with fever being the most common. Others may exhibit gastrointestinal symptoms like nausea, vomiting, abdominal pain, or loss of appetite, with nausea and vomiting being the most frequent. Some patients may only show fatigue or weakness.

• Blood glucose symptoms: After onset, blood sugar rises rapidly, leading to pronounced symptoms like extreme thirst, excessive drinking, frequent urination, and weight loss.

• Severe ketoacidosis symptoms: Extremely high blood sugar levels can trigger diabetic ketoacidosis, presenting as loss of appetite, nausea, vomiting, headache, irritability, drowsiness, deep rapid breathing, and a fruity (acetone) odor on the breath. As the condition worsens, severe dehydration occurs, leading to reduced urine output, dry skin, sunken eyes, rapid heartbeat, low blood pressure, and cold limbs. Without intervention, coma may follow.

If onset occurs during pregnancy, symptoms are typically more severe and may include frequent uterine contractions, reduced fetal movement, and vaginal bleeding.

What serious complications can fulminant type 1 diabetes cause?

Severe or untreated cases can be life-threatening.

If the disease occurs during pregnancy, the risk of miscarriage and fetal mortality is significantly higher.

CAUSES

What is the cause of fulminant type 1 diabetes?

The cause of fulminant type 1 diabetes remains unclear, but it may be related to genetic factors, viral infections, and immune factors.

• Genetic factors: Human leukocyte antigen (HLA) gene phenotypes are closely associated with the onset of this disease, and reported gene phenotypes vary among different countries and ethnic groups.

• Viral infections: Existing reports suggest that infections with Coxsackie virus, mumps virus, hepatitis A virus, parvovirus, or parainfluenza virus may trigger the disease.

• Immune factors: Both congenital and acquired immune disorders may contribute to the development of fulminant type 1 diabetes.

Who is most susceptible to fulminant type 1 diabetes?

Fulminant type 1 diabetes shows significant ethnic differences, with the highest incidence in East Asian populations. Japan reports the most cases, followed by China, South Korea, and the Philippines, while it is rare in Western countries.

The disease can occur in individuals aged 1 to 80, with no gender difference, though it is more common in adults and rare in children and adolescents.

In males, the incidence increases with age. In females, the onset age tends to be younger, often related to pregnancy, with most cases occurring during the second or third trimester or within two weeks postpartum. Nearly all new-onset type 1 diabetes cases during pregnancy are fulminant type 1 diabetes, making pregnant women a high-risk group.

Is fulminant type 1 diabetes contagious?

Fulminant type 1 diabetes is not contagious.

Is fulminant type 1 diabetes hereditary?

The exact cause of fulminant type 1 diabetes remains unknown, but genetic factors play a role. However, having a parent with the disease does not guarantee that the child will develop it.

DIAGNOSIS

How is fulminant type 1 diabetes diagnosed?

When diagnosing fulminant type 1 diabetes, doctors primarily refer to the diagnostic criteria established by the Japan Diabetes Society in 2012. A diagnosis is confirmed when all three of the following conditions are met:

1. Rapid onset (within approximately 7 days) of diabetic ketosis or diabetic ketoacidosis after the appearance of hyperglycemic symptoms (detected through urine and/or blood tests showing elevated ketones);

2. Blood glucose level ≥ 16.0 mmol/L (≥ 288 mg/dL) and glycated hemoglobin (HbA1c) < 8.5%;

3. Urinary C-peptide excretion < 10 μg/day or fasting C-peptide level < 0.3 ng/mL (< 0.1 nmol/L), with post-glucagon (or postprandial) C-peptide level < 0.5 ng/mL (< 0.17 nmol/L).

What tests are needed for fulminant type 1 diabetes?

• Viral infection screening: Tests for viruses such as EBV, CMV, HSV, and rubella to identify potential causes.

• Blood glucose: Confirms hyperglycemia and guides treatment.

• Glycated hemoglobin (HbA1c): Assesses the duration of hyperglycemia; a lack of significant elevation suggests acute onset, consistent with fulminant type 1 diabetes.

• Fasting and postprandial C-peptide: Evaluates pancreatic β-cell function.

• Urinalysis, blood ketones, and arterial blood gas analysis: Determines the presence and severity of diabetic ketoacidosis.

• Liver and kidney function, lipid profile, pancreatic enzymes, and electrolytes: Assesses overall health and guides treatment.

• Diabetes-related autoantibodies: Includes GAD antibodies, IA-2 antibodies, and islet cell antibodies to aid diagnosis and classification.

• Pancreatic CT scan: Rules out acute pancreatitis.

Which diseases are easily confused with fulminant type 1 diabetes? How to differentiate them?

Fulminant type 1 diabetes is often accompanied by elevated pancreatic enzymes and gastrointestinal symptoms like nausea, vomiting, and abdominal pain, making it easily confused with acute pancreatitis.

Acute pancreatitis may present with diabetic ketoacidosis and elevated pancreatic enzymes, but patients usually have predisposing factors such as gallbladder disease, binge eating, or alcohol consumption. Severe pancreatitis also exhibits specific clinical signs, and imaging (ultrasound or CT) may reveal hemorrhage or necrosis. Unlike fulminant type 1 diabetes, pancreatic function often recovers after treatment in acute pancreatitis.

TREATMENT

Which department should be consulted for fulminant type 1 diabetes?

Fulminant type 1 diabetes should be treated by an endocrinologist, but in emergencies, patients can go directly to the emergency department. In critical conditions, intensive care unit (ICU) admission may be required.

Can fulminant type 1 diabetes resolve on its own?

Fulminant type 1 diabetes does not resolve on its own. Without prompt treatment, it can be life-threatening.

How is fulminant type 1 diabetes treated?

Fulminant type 1 diabetes is a severe condition requiring immediate hospitalization upon suspicion or diagnosis. Treatment involves insulin administration and intravenous fluids to control blood sugar and correct ketoacidosis.

As the condition stabilizes, treatment transitions to subcutaneous insulin injections. A "three short-acting, one long-acting" regimen is recommended—short-acting or rapid-acting insulin before meals and long-acting insulin (e.g., insulin glargine) at bedtime. Alternatively, insulin pump therapy may be used.

Oral hypoglycemic drugs and premixed insulin are not recommended for fulminant type 1 diabetes.

Is hospitalization necessary for fulminant type 1 diabetes?

Yes, fulminant type 1 diabetes is life-threatening and requires immediate hospitalization upon suspicion or diagnosis. ICU admission may be needed in severe cases.

Can fulminant type 1 diabetes be cured?

Fulminant type 1 diabetes cannot be cured, but it can be managed. The primary treatment is long-term insulin therapy.

DIET & LIFESTYLE

What should patients with fulminant type 1 diabetes pay attention to in their diet after their condition stabilizes?

Dietary precautions are the same as for diabetes. Search for "diabetes" on the Dingxiang Doctor App for further details.

What should patients with fulminant type 1 diabetes pay attention to in daily life after their condition stabilizes?

Lifestyle precautions are the same as for diabetes. Search for "diabetes" on the Dingxiang Doctor App for further details.

PREVENTION

Can Fulminant Type 1 Diabetes Be Prevented?

The exact cause of fulminant type 1 diabetes remains unclear, and there are currently no effective preventive measures.

How Can Patients with Fulminant Type 1 Diabetes Prevent Other Diseases?

Upon diagnosis, active treatment is essential. After stabilizing the condition, maintain a balanced diet, follow a regular routine, adhere to insulin therapy as prescribed, and monitor blood sugar levels consistently.