autosomal recessive periodic fever syndrome
episodic attacks of fever lasting 3-7 days, usually accompanied by chills, cervical lymphadenopathy, abdominal pain, and vomiting or diarrhoea
some experience headache, arthralgias or arthritis, aphthous ulceration, a pleomorphic rash, and, occasionally, splenomegaly
2/3rds begin in 1st year of life
most have IgD >100 international units/mL
80% have elevated IgA
raised ESR, CRP occur during attacks and may remain high between attacks