Syndromes of Periodic Fever -⁠ the Syndrome of Mevalonic Aciduria and HyperimmunoglobulinemiaD

Overview

Periodic fever is defined as recurrences of fever that last from a few days to a few weeks, separated bysymptom-free intervals of variable duration. This pattern of fever can be caused by recurrent infections orneoplastic disorders but also by noninfectious inflammatory disorders. It is important to review the medical historycarefully in patients with recurrent febrile attacks. Patients with periodic fever that persists for more than twoyears rarely have infections of malignant disorders. Attacks with a predictable course and a similar set ofsymptoms, along with a family history of such attacks, may suggest the presence of a noninfectious form of periodicfever. Although numerous disorders, such as juvenile rheumatoid arthritis, Still’s disease and, Crohn’s disease, cancause periodic fever, this article will focus on hereditary periodic fever syndromes. The authors present two casesof the mevalonic aciduria/hyper IgD syndrome of various severity. For differential diagnostic reasons they alsodraw attention to other, etiologically diverse types of hereditary fever syndromes, such as familial Mediterraneanfever, the tumor necrosis factor (TNF) receptor-associated periodic syndrome, the Muckle-Wells syndrome andthe familial cold autoinflammatory syndrome.

Key words:
periodic fever, mevalonic aciduria, hyper IgD syndrome, diagnosis