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

Background and Objective: Periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome (PFAPA) is an auto-immune based disease which is known as a syndrome for pediatrics which typically occurs in children ≤5 years of age. Aim of this study was report of the first case of PFAPA with splenomegaly in Iran Patient Report: Our case is first adult-onset PFAPA patient in Iran which has been accompanied by splenomegaly. First time since March 2017, the patient suffered from periodic febrile attacks (39-40 °C). During these fever attacks, the patient has many aphthous ulcers in his mouth, swollen glands in his neck and sore in the back of the throat. The patient was admitted to hospital due to weakness, lethargy, high-temperature fever and slight abdominal pain in LUQ (left upper quadrant) area. Abdominal sonography was done and spleen size was 32×140 mm (Splenomegaly). 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 long-acting Betamethasone ampoule at the onset of the fever attacks. Also, Montelukast has given to the patient and we have seen his febrile attacks intervals have increased.

کلیدواژه ها:

Adult ، PFAPA syndrome ، Splenomegaly ، Iran