Kikuchi–Fujimoto disease triggered by Salmonella enteritidis in a child with concurrent auto-immune thyroiditis and papilloedema

Abstract

Kikuchi-Fujimoto disease (KFD) is a histiocytic necrotising lymphadenitis characterised by painful cervical lymphadenopathy, fever, malaise and weight loss. Infections, auto-immune pathogenesis and a genetic association have been implicated. A 12-year-old boy presented with a 1-month history of fever, abdominal pain, constipation and weight loss, and a painful lymph node was detected in the right axilla. Chest CT demonstrated multiple lymph nodes, especially in the left mediastinum. Salmonella enteritidis group D was detected in a blood culture and he was treated with ceftriaxone, followed by meropenem. An axillary lymph node biopsy demonstrated necrotising histiocytic lymphadenitis and KFD was diagnosed. He was discharged 35 days after admission. He was re-admitted 3 weeks later with recurrence of symptoms and headache and was found to have papilloedema of the left eye and auto-immune thyroiditis. Intravenous immunoglobulin (IVIG) 400 mg/kg/day was administered for 5 days. The fever and papilloedema slowly resolved and, subsequently, the thyroiditis, and he has remained well on follow-up. This is the first report of an association of S. enteritidis infection and papilloedema with KFD. IVIG may be required in prolonged or recurrent cases and in those with an auto-immune association.

Kikuchi-Fujimoto Disease With Encephalopathy in Children: Case Reports and Literature Review

“…According to Rezai et al (24), hydroxychloroquine has fewer adverse effects and is more efficacious than corticosteroids. The role of IVIG in KFD is for its anti-inflammatory effects for conditions where no autoantibody has been demonstrated (25). Immunosuppressive therapy has also been recommended for complicated cases with increased LDH and raised serum antinuclear antibody titers to prevent fatal outcomes (22).…”