hereditary periodic fever syndrome; periodic fevers; recurrent fever syndrome; familial autoinflammatory disease

Introduction

Episodes of systemic inflammation in the absence of infection of autoimmunity.

Etiology

• familial Mediterranean fever
• hyperimmunoglobulinemia D with periodic fever syndrome
• cryopyrin associated periodic syndrome
  • Muckle-Wells syndrome
  • neonatal-onset multisystem inflammatory disease
  • familial cold autoinflammatory syndrome
• familial hibernian fever

Clinical manifestations

• intermittent episodes of fever
• other systemic manifestations
  • arthralgia
  • abnominal pain
  • rash

Laboratory

• see ARUP consult

Differential diagnosis

• may be cause of fever of unknown origin

More general terms

• genetic disease of the immune system
• genetic syndrome (multisystem disorder)
• fever syndrome

More specific terms

• cryopyrin associated periodic syndrome (CAPS)
• familial hibernian fever (FHF); tumor necrosis factor (TNF) receptor-associated periodic syndrome (TRAPS)
• familial Mediterranean fever (FMF, recurrent polyserositis)
• hyper IgD syndrome; hyperimmunoglobulinemia D & periodic fever syndrome; mevalonate kinase deficiency; HIDS
• Muckle-Wells syndrome (MWS)