Therapeutic response and long-term follow-up in a systemic lupus erythematosus patient presenting with Kikuchi's disease

Abstract

Kikuchi's disease (KD) can occur in association with systemic lupus erythematosus (SLE). The treatment of concomitant diseases, however, is unclear. We describe a case of a 45-y-old man who presented with generalized histiocytic necrotizing lymphadenitis, fever, malaise and weight loss. Ten months later he also developed arthritis, serositis, anemia, leukopenia and lymphopenia. ANA, anti-Smith, anti-snRNP and anti-Ro antibodies were positive. He responded rapidly and favorably to mid-dose prednisone. Hydroxychloroquine, added 5 months later, allowed tapering down and discontinuation of prednisone treatment. He has remained in complete remission for 5 years.