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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



Canada (Silver et al. 2004)

43 year old Female

Discoid lupus erythematosus for 10 years


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)


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


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.