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Table 3 Clinical characteristics of reported cases of Kikuchi-Fujimoto disease occurring after the onset of connective tissue disease

From: Fatal Kikuchi-like lymphadenitis associated with connective tissue disease: a report of two cases and review of the literature

Connective tissue disease predating necrotising lymphadenitis
Country and reference Age and gender Connective tissue disease Autoantibodies Outcome
Canada (Silver et al. 2004) 43 year old Female Discoid lupus erythematosus for 10 years None Recovered without treatment
India (Londhey et al. 2010) 39 year old Female SLE for 2 years (not initially diagnosed) ANA and anti-dsDNA No life-threatening sequelae. Responded to corticosteroids and hydroxychloroquine. Asymptomatic at 1 year follow-up.
Canada (Pace-Asciak et al. 2008) 35 year old Female SLE (duration not stated) NA Lymphadenitis resolved with conservative management.
France (Frikha et al. 2008) 30 year old Female Mixed connective tissue disease for 8 years ANA, anticardiolipin Lymphadenitis resolved with corticosteroid therapy.
Taiwan (Chen and Lan 1998) 4 cases All female Ages 21–35 years old SLE for 8 days to 2 years Ribosomal-P antibodies (1 case) RNP (1 case) None (2 cases) No life-threatening sequelae
France (Abraham et al. 2011) 35 year old Female Overlap syndrome for 12 years Antiphospholipid antibodies Lymphadenitis resolved with corticosteroid therapy.
Sabah (Bachi 2002) 27 year old Female SLE – systemic symptoms for 2 months ANA, anti-Ro, anti-La at onset of lymphadenitis Complete remission with corticosteroid therapy.
Japan (Wano et al. 2000) 37 year old Female SLE for 2 years NA Haemophagocytic syndrome. Complete remission with corticosteroid pulse therapy.
India (Londhey et al. 2010) 39 year old Female Onset of connective tissue disease for 4 years. Lymphadenitis first appeared after 2 years. SLE diagnosis established later. ANA, anti-dsDNA. Complete remission with corticosteroid therapy.
Spain (Sopena et al. 2012) 2 cases 22 and 32 years old Both Female Both SLE. Onset not stated. ANA only (1 case) ANA, anticardiolipin, anti-Ro, RNP (1 case) Good response to therapy
Italy (Ruaro et al. 2014) 19 year old Male SLE, onset 2–3 months prior to diagnosis of Kikuchi lymphadenitis ANA only Initial good response to therapy, single episode of relapse 2 years after initial onset, subsequently symptom free on corticosteroid and methotrexate therapy.