Risk Assessment of Recurrence and Autoimmune Disorders in Kikuchi Disease

Abstract

PURPOSE: Kikuchi disease (KD) is typically a benign disease. Recent studies reporting recurrence or serious cases suggest a possible association of KD with systemic autoimmune disorders. We performed a long-term analysis of the characteristics of KD in patients of all ages and assessed KD recurrence or progress to systemic autoimmune disorders. Patients and METHODS: Electronic medical records of patients diagnosed with KD between April 1995 and May 2017 were reviewed for clinical and laboratory manifestations. RESULTS: In total, 480 patients were confirmed to have KD based on histopathology findings. The mean age at KD diagnosis was 24.4 years. Recurrence occurred in 11.3% of patients; 2.7% developed autoimmune diseases after KD diagnosis. Patients who experienced recurrence had more extranodal symptoms, lymphopenia, and a longer lymphopenia-recovery duration. Patients who developed autoimmune diseases after KD were more likely to have extranodal symptoms, KD recurrence, and anti-nuclear antibody positivity. CONCLUSION: KD patients with risk factors need to be followed-up for KD recurrence and the development of systemic autoimmune diseases.