Periodic fever, aphthous stomatitis, pharyngitis and adenitis

The key symptoms of PFAPA are those in its name: periodic high fever at intervals of about 3–5 weeks, as well as aphthous ulcers, pharyngitis and adenitis. In between episodes, and even during the episodes, the children appear healthy. At least 6 months of episodes. ==Cause==

The cause of PFAPA is unknown. It is frequently discussed together with other periodic fever syndromes. The changes in the immune system are complex and include increased expression of complement related genes (C1QB, C2, SERPING1), interleukin-1-related genes (interleukin-1β, interleukin 1 RN, CASP1, interleukin 18 RAP) and interferon induced (AIM2, IP-10/CXCL10) genes. T cell associated genes (CD3, CD8B) are down regulated. Flares are accompanied by increased serum levels of activated T lymphocyte chemokines (IP-10/CXCL10, MIG/CXCL9), G-CSF and proinflammatory cytokines (interleukin 6, interleukin 18). Flares also manifest with a relative lymphopenia. Activated CD4+/CD25+ T-lymphocyte counts correlated negatively with serum concentrations of IP-10/CXCL10, whereas CD4+/HLA-DR+ T lymphocyte counts correlated positively with serum concentrations of the counterregulatory IL-1 receptor antagonist. ==Diagnosis==

Diagnosis requires recurrent negative throat cultures and that other causes (such as EBV, CMV, FMF) be excluded. ==Treatment==

PFAPA syndrome typically resolves spontaneously. Treatment options are used to lessen the severity of episodes. These treatments are either medical or surgical: One treatment often used is a dose of a corticosteroid at the beginning of each fever episode. Surgical removal of the tonsils appears to be beneficial compared to no surgery in symptom resolution and number of future episodes. ==Prognosis==

According to present research, PFAPA does not lead to other diseases and spontaneously resolves as the child gets older, with no long term physical effects. However, PFAPA has been found in adults and may not spontaneously resolve. Children with PFAPA experience lower physical, emotional, and psychosocial functioning. Their performance in school is also substantially impacted. == References ==