[Medical study of 69 cases diagnosed as Kikuchi's disease]

We studied clinical manifestations, laboratory results, treatment, recurrence, and complications in 69 patients diagnosed with Kikuchi's disease by lymph node pathology from January 1, 1998, to December 31, 2007. Subjects were 34 men and 35 women (median age: 28 years, range: 12 to 58 years). Of the 69, 67 were Japanese and 2 were Korean. Major clinical symptoms and signs were fever >37 degrees (71%) and lymphadenopathy (100%). Lymphadenopathy was cervical in 93% and systemic in 3%. Some experienced night sweats and weight loss. The median white blood cell count was 3800 microL (1,700-9,300 microl), and 50.7% of subjects had leukocytopenia (<4,000 microL). The median serum LDH concentration was 245U/L (129-923 U/L). The median ferritin concentration, measured in 26 cases, was 769 ng/mL (4.5-2,580 ng/mL). The median concentration of soluble IL-2 receptor, measured in 27 cases, was 639 U/mL (0.5-4,000 U/ml). Having observed several cases with abnormally high ferritin and soluble IL2 receptor, we note the importance of carefully considering differential diagnosis from Still's disease and malignant lymphoma. Treatment included no medication in 30% of subjects, nonsteroidal anti-inflammatory drugs alone in 37.7%, steroids alone in 7%, and combined nonsteroidal anti-inflammatory drugs and steroids in 22%. Of the 29% administered steroids, we mostly used prednisolone (0.5-1.0 mg/kg), tapering the dose as clinical features improved. Two developed aseptic meningitis and 2 systemic lupus erythematosus. In total, 75% improved in less than 3 months, whereas 6% showed improvement only after at least 6 months of continued treatment. All were cured, but the condition recurred in 8%. Recurrence was not associated initial the disease duration. Recurrence cannot be predicted but can occur. In some cases, we could not distinguish lymphadenitis from malignant lymphoma or tuberculous lymphadenitis based on clinical features or laboratory data. Diagnosis must thus be based on lymph node pathology. We also must consider the possibility of recurrence or attacks of systemic lupus erythematosus.