Overview of Hereditary Periodic Fever Syndromes

Familial Mediterranean fever Familial Mediterranean Fever Familial Mediterranean fever is an autosomal recessive disorder characterized by recurrent bouts of fever and peritonitis, sometimes with pleuritis, skin lesions, arthritis, and, rarely, pericarditis... read more

Hyper-IgD syndrome Hyper-IgD Syndrome Hyper-IgD syndrome is a rare autosomal recessive disorder in which recurring attacks of chills and fever begin during the first year of life. Episodes usually last 4 to 6 days and may be triggered... read more

Tumor necrosis factor (TNF) receptor–associated periodic syndrome TNF Receptor–Associated Periodic Syndrome (TRAPS) Tumor necrosis factor receptor–associated periodic syndrome is an autosomal dominant disorder causing recurrent fever and painful, migratory myalgias with tender overlying erythema. Diagnosis... read more

The hereditary cryopyrin-associated periodic syndromes (cryopyrinopathies) Hereditary Cryopyrin-Associated Periodic Syndromes (Cryopyrinopathies) The hereditary cryopyrin-associated periodic syndromes are a group of autosomal dominant autoinflammatory conditions triggered by cold ambient temperatures; they include familial cold autoinflammatory... read more : Familial cold autoinflammatory syndrome, Muckle-Wells syndrome, and neonatal-onset multisystem inflammatory disease (NOMID)

PAPA PAPA Syndrome PAPA (pyogenic arthritis, pyoderma gangrenosum, and acne) syndrome is an autosomal dominant disorder that affects the skin and joints. PAPA syndrome is caused by Mutations in the proline-serine-threonine... read more (pyogenic arthritis, pyoderma gangrenosum, and acne) syndrome

PFAPA PFAPA Syndrome PFAPA (periodic fevers with aphthous stomatitis, pharyngitis, and adenitis) syndrome is a periodic fever syndrome that typically manifests between ages 2 years and 5 years; it is characterized... read more (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis) syndrome, which may not be hereditary