The First Case of Adult-Onset Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis Syndrome with Splenomegaly in Iran

BACKGROUND: Periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome (PFAPA) is an auto-immune based disease known as a syndrome for pediatrics which typically occurs in children ≤ 5 years of age, but in 2008, for the first time, one adult case of this disease was reported. CASE PRESENTATION: Our case, a 19 year-old young man who is the first adult-onset PFAPA patient in Iran and was accompanied by splenomegaly. Since March 2017, the patient suffered from periodic febrile attacks (39-40 °C). During these fever attacks, the patient had many aphthous ulcers in his mouth, swollen glands in his neck and sore in the back of the throat. The patient was admitted to a hospital in Tehran during a severe fever attack due to weakness, lethargy, high-temperature and slight abdominal pain in the left upper quadrant (LUQ) area. Abdominal sonography was done and spleen size was larger than normal and was determined to be 32×140 mm (splenomegaly). All data were collected from a reliable governmental laboratory in Tehran. CONCLUSION: Following the rejection of the causes of other diseases, according to the patient's symptoms, the diagnosis of adult-onset PFAPA was given to the patient and the patient was cured with one dose of long-acting Betamethasone ampoule 1ml intravenous at the onset of fever attacks. The disease has remitted after injection of this medicine at the onset of each attack rapidly after about 2-3 hours. Also, Montelukast has been given to the patient and we saw his febrile attack intervals increased.