clinical features

Last reviewed 04/2022

The onset of the disease is usually between 5 and 15 years of age. The frequency and duration of attacks varies greatly with attacks lasting 24 h to 10 days and varying in frequency from 1 per year to 1 per fortnight.

Possible features include:

• fever: is present during most attacks
• abdominal pain: in 95% of cases. The pain usually begins in one quadrant and spreads to effect the whole abdomen. Often the abdomen is also distended.
• chest pain: 75% of patients develop acute pleuritic chest pain with or without abdominal pain. The chest pain is usually unilateral and associated with a friction rub, decreased breath sounds, or a pleural effusion.
• joint pain: may involve one or several joints. Effusions are common. Large joints are most often involved. There are no specific radiological findings.
• skin manifestations: in approximately 25% of patients. There may be areas of a painful, erythema that are usually seen on the lower legs. They may occur with or without abdominal or chest pain.
• there has been other involvement of serosal membranes reported.