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Hirata disease
ICD-10 Codes
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Description
Hirata Disease, also known as Insulin Autoimmune Syndrome (IAS)
Hirata disease is a rare condition characterized by episodes of spontaneous hypoglycemia due to the presence of high titers of autoantibodies to insulin. This disorder is also known as Insulin Autoimmune Syndrome (IAS).
Key Features:
- Hyperinsulinemic Hypoglycemia: The primary symptom of Hirata disease is recurrent episodes of low blood sugar (hypoglycemia) caused by excessive production of insulin.
- Elevated Insulin Autoantibody Titters: High levels of autoantibodies to endogenous insulin are present in the body, which contributes to the development of hypoglycemia.
- No Prior Pancreatitis or Diabetes: Unlike other forms of autoimmune diabetes, Hirata disease is not preceded by pancreatitis or a history of diabetes.
Causes and Risk Factors:
The exact cause of Hirata disease is unknown. However, it is believed to be an autoimmune disorder triggered by the presence of autoantibodies to insulin.
Symptoms and Diagnosis:
- Spontaneous Episodes of Hypoglycemia: Patients experience recurring episodes of low blood sugar without any apparent reason.
- Positive Insulin Autoantibody Test: A blood test can detect high levels of autoantibodies to insulin, confirming the diagnosis.
Treatment and Prognosis:
Hirata disease is a reversible condition, and treatment typically involves addressing the underlying autoimmune response. In some cases, patients may require medication or lifestyle changes to manage their symptoms.
References:
- [1] by D Cappellani · 2020 · Cited by 128 — Insulin autoimmune syndrome (IAS), also named Hirata's disease, is a rare condition characterized by hypoglycemic episodes due to the presence of high titers ...
- [2] Oct 2, 2020 — Insulin autoimmune syndrome, or Hirata's disease, is a rare cause of hypoglycaemia. It is characterised by spontaneous episodes of hypoglycaemia, without any ...
- [3] by M Yukina · 2020 · Cited by 18 — Insulin autoimmune syndrome (Hirata's disease) is a disorder caused by development of autoantibodies to insulin and manifested by hypoglycaemic syndrome.
- [4] by D Cappellani · 2020 · Cited by 128 — Insulin autoimmune syndrome (IAS), also named Hirata's disease, is a rare condition characterized by hypoglycemic episodes due to the presence of high titers ...
- [5] A rare endocrine disease characterized by hyperinsulinemic hypoglycemia associated with the presence of autoantibodies to endogenous insulin without previous ...
- [6] by S Censi · 2018 · Cited by 132 — Insulin autoimmune syndrome (IAS) is characterized by: (I) hyperinsulinemic hypoglycemia; (II) elevated insulin autoantibody (IAA) titers; (III) no prior ...
- [7] Insulin autoimmune syndrome (IAS), a rare cause of reversible autoimmune hypoglycemia also known as Hirata's disease, was first described by Hirata in Japan ...
- [8] by H Al-Bahadili · 2022 — Hirata's Disease is a rare condition characterized by episodes of spontaneous hypoglycemia secondary to hyperinsulinemia in the setting of positive insulin ...
Additional Characteristics
- Hirata disease
- Insulin Autoimmune Syndrome (IAS)
Signs and Symptoms
Hirata disease, also known as insulin autoimmune syndrome (IAS), is a rare condition characterized by episodes of spontaneous hypoglycemia secondary to hyperinsulinemia in the setting of positive insulin autoantibodies. The clinical hallmark of IAS is hypoglycemia, which manifests with autonomic and neuroglycopenic symptoms.
Autonomic Symptoms:
- Hunger
- Sweating
- Tremor
- Anxiety
These symptoms occur due to the body's attempt to compensate for the low blood sugar levels by releasing stress hormones such as adrenaline. [9]
Neuroglycopenic Symptoms:
- Confusion
- Drowsiness
- Seizures (in severe cases)
These symptoms occur due to the lack of glucose in the brain, which can impair cognitive function and lead to seizures if left untreated. [9]
In addition to these symptoms, patients with Hirata disease may also experience other complications such as:
- Weight gain or loss
- Fatigue
- Muscle weakness
It's worth noting that the presentation of IAS can vary widely among individuals, and some cases may be atypical or mild. [10]
Additional Symptoms
- Hunger
- Sweating
- Seizures (in severe cases)
- Weight gain or loss
- muscle weakness
- tremor
- confusion
- drowsiness
- fatigue
- anxiety
Diagnostic Tests
Diagnostic Tests for Hirata Disease
Hirata disease, also known as insulin autoimmune syndrome (IAS), is a rare condition characterized by recurrent hypoglycemia due to insulin autoimmune disease. The diagnosis of this condition requires careful analysis of clinical findings and laboratory test results.
- Complete Blood Count: A complete blood count (CBC) may be performed to rule out other conditions that can cause hypoglycemia.
- Liver Function Tests: Liver function tests, such as alanine transaminase (ALT) and aspartate transaminase (AST), may be elevated in patients with IAS.
- Insulin Autoantibody (IAA) Titer: The measurement of IAA titer is mandatory for the diagnosis of IAS. Elevated levels of IAA are a hallmark of this condition [5].
- Laboratory Tests: Laboratory tests show elevated levels of serum insulin, C-peptide, and proinsulin in patients with IAS [3].
It's essential to note that the diagnosis of Hirata disease requires a detailed interview on the use of implicated drugs and careful analysis of clinical findings and laboratory test results [7]. A blood test for polyclonal autoantibodies against insulin (IAA) may also be reported positive in patients with this condition [8].
References:
[1] by R Galván · 2023 — Diagnosis of this condition requires careful analysis of clinical findings and laboratory test results. We present below the procedure followed by our clinical ...
[2] Oct 2, 2020 — These included: complete blood count, liver function tests ... Recurrent hypoglycaemia due to insulin autoimmune disease (Hirata disease) ...
[3] Laboratory tests show elevated levels of serum insulin, C-peptide, and proinsulin. The condition may be linked to other autoimmune diseases, monoclonal ...
[5] by D Cappellani · 2020 · Cited by 128 — The measurement of IAA titer is mandatory for the diagnosis of IAS since it is the only lab test that allows a reliable diagnosis of IAS.
[7] by S Censi · 2018 · Cited by 132 — This review covers all aspects of the autoimmune forms of hypoglycemia, from diagnosis to treatment.
[8] by M Yukina · 2020 · Cited by 18 — Diagnosis of this condition requires detailed interview on the use of implicated drugs and careful analysis of the clinical findings and laboratory test results ...
[9] by K Kitsios · 2024 — A week later, a blood test for polyclonal autoantibodies against insulin (IAA) was reported positive (IAA level, 175 IU/mL; reference range, <20 ...
Additional Diagnostic Tests
- Laboratory Tests
- Liver Function Tests
- Complete Blood Count
- Insulin Autoantibody (IAA) Titer
Treatment
Treatment Options for Hirata Disease
Hirata disease, also known as Insulin Autoimmune Syndrome (IAS), is a rare condition characterized by hypoglycemic episodes due to the presence of high titers of insulin autoantibodies (IAA). The treatment options for Hirata disease are aimed at managing symptoms and preventing complications.
Pharmacological Therapy
Several medications have been used
Recommended Medications
- Insulin sensitizers
- glucagon-like peptide-1 receptor agonist
- metformin
- Metformin
💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.
Differential Diagnosis
Differential Diagnosis of Hirata Disease
Hirata disease, also known as Insulin Autoimmune Syndrome (IAS), is a rare disorder characterized by the development of autoantibodies to insulin, leading to hypoglycemic episodes. When considering differential diagnosis for Hirata disease, several other conditions must be ruled out.
- Type B Insulin Resistance Syndrome (TBIRS): This is another rare autoimmune disorder that results in broad insulin resistance and hyperinsulinemia. TBIRS can present with similar symptoms to Hirata disease, making it a potential differential diagnosis [6].
- Other forms of hypoglycemia: Table 3 from the study by D Cappellani (2020) provides a scheme for differential diagnosis with other forms of hypoglycemia, including:
- Factitious hypoglycemia
- Factitious hyperinsulinism
- Insulinoma
- Non-insulinoma pancreatogenous hypoglycemia syndrome (NIPHS)
- Other rare causes of hypoglycemia [1].
- Hyperinsulinemic hypoglycemia: This condition is characterized by excessive insulin secretion, which can lead to hypoglycemic episodes. Differential diagnosis from Hirata disease requires careful evaluation of the patient's symptoms and laboratory results [8].
Key Points
- Hirata disease must be differentiated from other rare autoimmune disorders, such as TBIRS.
- Other forms of hypoglycemia, including factitious and non-insulinoma-related causes, should also be ruled out.
- Careful evaluation of the patient's symptoms and laboratory results is essential for accurate differential diagnosis.
References
[1] D Cappellani (2020) - A scheme for differential diagnosis with other forms of hypoglycemia is reported in Table 3. [6] S Censi (2018) - This condition is also known as “Hirata's disease”. The other is type B insulin resistance syndrome (TBIRS), a rare autoimmune disorder resulting in a broad ... [8] Insulin autoimmune syndrome (Hirata disease) as differential diagnosis in patients with hyperinsulinemic hypoglycemia. ... Search GEO for disease gene expression ...
Additional Differential Diagnoses
- Type B Insulin Resistance Syndrome (TBIRS)
- Other rare causes of hypoglycemia
- Factitious hypoglycemia
- Factitious hyperinsulinism
- hyperinsulinemic hypoglycemia
- hypoglycemia
- insulinoma
Additional Information
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