Kikuchi–Fujimoto Disease: Review of 11 Cases Diagnosed Over 10 Years at a Tertiary Care Hospital in Doha, Qatar

INTRODUCTION: Kikuchi-Fujimoto (KF) disease is a rare and self-limiting disorder of unknown aetiology characterized by cervical lymphadenopathy (CLN) and fever. The pathophysiology remains unclear and may be triggered by an infectious agent leading to a self-limiting autoimmune process. There are no confirmatory laboratory tests and lymph node biopsy is required to differentiate KF disease from other serious conditions. MATERIALS AND METHODS: We report 11 cases of KF disease diagnosed at Hamad General Hospital, Qatar, between 2006 to 2016. The diagnosis is based on clinical presentation, investigations and histopathological examination of lymph nodes. RESULTS: All patients had painful neck swelling (average duration of 2.9 weeks) and 10 had fever (average duration of 3.2 weeks). Five patients developed mild leucopenia which resolved completely. HIV and tuberculosis (TB) screening including sputum for AFB, a PPD skin test and chest x-ray was done for all patients and came back negative. Autoimmune screening was done for all patients and excluded any rheumatological disease. Ultrasound and CT of the neck confirmed cervical lymphadenopathy. Except for hepatomegaly in one patient, CT scans of the chest and abdomen were negative for any lymphadenopathy or organomegaly (performed in seven patients). Diagnosis was confirmed on lymph node excision biopsy. Histopathological examination showed findings consistent with the diagnosis of histiocytic necrotizing lymphadenitis (KF disease). CONCLUSION: KF disease should be kept in mind for patients presenting with fever and CLN. Lymphoma, TB and autoimmune diseases like systemic lupus erythematosus should be excluded in such patients. LEARNING POINTS: Kikuchi-Fujimoto (KF) disease should be kept in mind in patients presenting with fever and cervical lymphadenopathy with or without other constitutional symptoms. There are no confirmatory blood tests and KF disease requires histopathological examination of involved lymph nodes for confirmation. Autoimmune disease like systemic lupus erythematosus, tuberculosis, lymphoma and other malignancies should be ruled out by appropriate investigations. Recurrence of disease, although rare, is a possibility and needs histopathology for confirmation.