IgD disease, more accurately known as Hyperimmunoglobulin D syndrome (HIDS), is a rare genetic disorder characterized by recurrent fevers and associated symptoms.
Understanding Hyperimmunoglobulin D Syndrome (HIDS)
HIDS is an autosomal recessive condition, meaning that an individual must inherit two copies of the mutated gene to develop the disorder. Here's a breakdown of key aspects:
Symptoms
- Recurrent Febrile Episodes: The most prominent feature is repeated episodes of high fever.
- Lymphadenopathy: Swelling of the lymph nodes is common during these episodes.
- Abdominal Pain: Many individuals experience discomfort or pain in the abdomen.
- Elevated Serum IgD: A key diagnostic marker is a high level of immunoglobulin D (IgD) in the blood.
Genetic Basis
HIDS is a genetic condition caused by mutations in a specific gene. This affects the immune system, resulting in the characteristic symptoms. The condition is inherited in an autosomal recessive manner, meaning that both parents must carry the mutation for their child to develop the disease.
Diagnosis
Diagnosis typically involves recognizing the characteristic symptoms and confirming an elevated level of IgD in the blood. Genetic testing can also help verify the diagnosis.
Key Features of HIDS
| Feature | Description |
|---|---|
| Type | Rare, autosomal recessive genetic disorder |
| Primary Symptom | Recurrent fevers |
| Associated Symptoms | Lymphadenopathy, abdominal pain, |
| Diagnostic Marker | Elevated serum IgD levels |
Practical Insights
- HIDS is not contagious.
- It is important to seek medical advice if you suspect you or someone you know has HIDS.
- Management typically focuses on controlling symptoms during fever episodes.
Conclusion
HIDS is a rare inherited disease with recurring fever and other symptoms such as swollen lymph nodes and abdominal pain. It can be diagnosed by testing for high IgD levels.
