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

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.