Clinical presentations, laboratory results and outcomes of patients with Kikuchi's disease: emphasis on the association between recurrent Kikuchi's disease and autoimmune diseases

Abstract

Background/purpose: Kikuchi'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.

Methods: We describe the clinical manifestations and outcomes of 195 patients, diagnosed as having KD at National Taiwan University Hospital from March 1989 to September 2006.

Results: All 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.

Conclusion: The prognosis for KD patients is generally optimistic; however, a concurrent autoimmune disease or the risk of developing an autoimmune disease requires careful monitoring.