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[Hyper-IgD syndrome (HIDS)]

R Scolozzi

    Recenti Progressi in Medicina
    |June 1, 1995
    PubMed
    Summary
    This summary is machine-generated.

    Hyper-IgD syndrome (HIDS) is a rare periodic fever disorder characterized by recurrent fevers and elevated IgD levels. While its cause remains unknown, HIDS generally has a benign prognosis with supportive care.

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    Area of Science:

    • Immunology
    • Genetics
    • Rheumatology

    Background:

    • Hyper-IgD syndrome (HIDS) is a rare autoinflammatory disorder characterized by recurrent fever episodes.
    • First described in 1984, HIDS presents with elevated polyclonal IgD levels and a history of periodic fevers.
    • A recent review identified 60 cases, predominantly from Europe, with a mean age of 27 years.

    Discussion:

    • HIDS exhibits a median age of onset of 0.5 years, with attacks lasting 3-7 days and occurring monthly or bimonthly.
    • Clinical manifestations include sustained fever (38-41°C), abdominal symptoms, lymphadenopathy, splenomegaly, arthritis, and vasculitis.
    • While a family history of periodic fever is common (40%), a family history of hyper-IgD is not.

    Key Insights:

    • The aetiopathogenesis of HIDS remains unknown, and the specific role of IgD in its pathogenesis requires further elucidation.

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  • Despite recurrent symptoms, the prognosis for HIDS is considered benign.
  • Current therapeutic approaches for HIDS are primarily supportive.

    • Outlook:

    • Further research into the genetic and molecular underpinnings of HIDS is warranted.
    • Investigating novel therapeutic strategies beyond supportive care may improve patient outcomes.
    • Enhanced understanding of IgD's role could reveal targeted treatment options.