Written by Private MD Labs Medical Writing Team • Last Updated: September 8, 2025
Hyper IgD syndrome (HIDS) is a rare inherited autoinflammatory disorder characterized by recurrent episodes of fever, abdominal pain, joint pain, and other inflammatory symptoms. The condition is caused by genetic mutations that affect the immune system's regulation, leading to distinctive changes in blood markers.
This test measures the level of immunoglobulin D in the blood, which is characteristically elevated in HIDS patients. The elevated IgD levels result from genetic mutations that disrupt normal immune system regulation, making this test a crucial component in confirming the diagnosis of this rare inherited condition.
HIDS diagnosis relies on a combination of clinical symptoms, family history, and specific laboratory findings. The hallmark laboratory feature is elevated levels of immunoglobulin D (IgD) in the blood, which occurs due to the underlying genetic mutation affecting immune system regulation.
In HIDS, genetic mutations affect cellular processes that normally regulate immune responses. This disruption leads to chronic low-level inflammation and the characteristic elevation of IgD levels. While not all patients with HIDS have extremely high IgD levels, the test remains an important diagnostic marker when combined with clinical presentation and genetic testing.
The information here is for educational purposes only and is not a substitute for professional medical advice. HIDS diagnosis typically requires evaluation by specialists familiar with autoinflammatory disorders. Always consult with a healthcare provider for proper diagnosis and treatment.