Family history and PFAPA
Sjögren syndrome, PFAPA, rheumatic diseases, Bechterev disease, PFAPA syndrome, first-degree, PFAPA - predominantly antibody immunodeficiencies
RHEUMATOID ARTHRITIS, FAMILIAL HAEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS, SELECTIVE IGA DEFICIENCY, RHEUMATIC DISEASES, PFAPA SYNDROME
In our group, almost one third (31.69%) of patients had a positive family history for PFAPA (46.7% boys and 53.3% girls). Girls had a higher number of parents with clinical symptomatology of PFAPA than boys. Boys had a higher number of siblings with similar clinical symptomatology. A higher number of girls with PFAPA had more than two first-degree relatives with clinical symptomatology of PFAPA. However, differences between genders were not significant. We also recorded a positive family history of rheumatic diseases in 6 patients with PFAPA syndrome (4 patients with rheumatoid arthritis, 1 patient with Sjögren syndrome and 1 patient with Bechterev disease). Immunodeficiencies occurred in 4 relatives of patients with PFAPA - predominantly antibody immunodeficiencies (3 selective IgA deficiency) and the parent of one patient was carrier of a mutation for familial haemophagocytic lymphohistiocytosis.