Clinical Presentations, Laboratory Results and Outcomes of Patients with Kikuchi's Disease: Emphasis on the Association Between Recurrent Kikuchi's Disease and Autoimmune Diseases

Background/PurposeKikuchi's disease (KD), also known as histiocytic necrotizing lymphadenitis, is a benign and self-limiting disease of unknown etiology that mainly affects young women. There is limited data on the long-term prognosis of patients with KD.MethodsWe describe the clinical manifestations and outcomes of 195 patients, diagnosed as having KD at National Taiwan University Hospital from March 1989 to September 2006.ResultsAll together, 53.3% of our patients presented with tender lymphadenopathy, 37.9% with fever and 16.9% with headache. The most common laboratory findings were elevated erythrocyte sedimentation rate (78.9%), elevated serum lactate dehydrogenase (52.5%), elevated C-reactive protein (38.3%), monocytosis (26.9%), elevated serum alanine aminotransferase (23.3%) and leukopenia (18.9%). A total of 183 patients followed a benign course, with spontaneous resolution of fever and lymphadenopathy. However, 14 patients (14.6%) with follow-up of more than 6 months had clinical recurrence of KD; hence, long-term follow-up is suggested. Five of these patients developed an autoimmune disease, namely, systemic lupus erythematosus (n = 2), Graves' disease (n = 2), or mixed connective tissue disease (n = 1). One patient with recurrent KD died of intracranial hemorrhage due to thrombocytopenia.ConclusionThe prognosis for KD patients is generally optimistic; however, a concurrent autoimmune disease or the risk of developing an autoimmune disease requires careful monitoring.