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Familial Hibernian fever

L M Williamson, D Hull, R Mehta

    The Quarterly Journal of Medicine
    |January 1, 1982
    PubMed
    Summary
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    An Irish family presents with a rare periodic fever syndrome. This condition, distinct from familial Mediterranean fever, responds well to steroids and has an autosomal dominant inheritance pattern.

    Area of Science:

    • Genetics
    • Internal Medicine
    • Rheumatology

    Background:

    • Periodic fever syndromes represent a group of autoinflammatory disorders.
    • Familial Mediterranean fever (FMF) is the most common periodic fever syndrome.

    Observation:

    • A case study describes an Irish family exhibiting a unique periodic syndrome.
    • Attacks manifest as fever, localized myalgia, and painful erythema.
    • Abdominal pain, pleurisy, leukocytosis, and elevated ESR are noted during episodes.

    Findings:

    • The described syndrome shares similarities with FMF but presents distinct characteristics.
    • A key differentiator is the rapid response to corticosteroid therapy.
    • Autosomal dominant inheritance is observed, differing from FMF's typical inheritance patterns.

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  • The condition follows a benign clinical course with no reported amyloidosis.

    • Implications:

    • This case expands the spectrum of known periodic fever syndromes.
    • The distinct genetic and therapeutic profile suggests a novel autoinflammatory condition.
    • Further research into the genetic basis and precise mechanisms of this syndrome is warranted.