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Table 4 Clinical characteristics of reported cases of Kikuchi-Fujimoto disease occurring before 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

Necrotising lymphadenitis predating connective tissue disease
Country and reference Age, gender Connective tissue disease Autoantibodies Outcome
Saudi Arabia (el-Ramahi et al. 1994) 22 year old Female SLE after 5 months ANA, anti-Sm, anti-dsDNA, anti-Ro, anti-La. Lymphadenitis resolved with corticosteroid therapy.
Saudi Arabia (el-Ramahi et al. 1994) 34 year old Female SLE after several months ANA, anti dsDNA, anti-Sm Not stated.
Thailand (Sanpavat et al. 2006) 28 year old Female SLE after 44 weeks ANA, anti-dsDNA Lymphadenitis resolved with corticosteroid therapy.
Spain (Paradela et al. 2008) 17 year old Female SLE onset after 9 months ANA, anti-dsDNA No life-threatening sequelae.
Australia (Goldblatt et al. 2008) 22 year old Female SLE after 3 months ANA, anti-Ro, anti-Sm at onset of SLE. Complete remission with corticosteroid therapy.
Australia (Goldblatt et al. 2008) 39 year old Female SLE after 14 months ANA, anti-dsDNA, anti-Ro, anti-Sm, anticardiolipin. Complete remission with immunosuppression.
Australia (Goldblatt et al. 2008) 31 year old Female SLE after 6 months ANA, anti-dsDNA, anti-Ro at onset of SLE Treated with corticosteroids, azathioprine and hydroxychloroquine. 2 years later, developed meningoencephalitis leading to a persistent neuro-vegetative state.
Australia (Goldblatt et al. 2008) 14 year old Female SLE after a few days Anti-dsDNA at onset of SLE. Complex clinical course with lupus nephritis. Eventual remission with B-cell depletion therapy.
Spain (Alijotas-Reig et al. 2008) 31 year old Female SLE after years. Lymphadenitis was pregnancy-associated and did not recur at onset of SLE. ANA, anti-RNP, borderline anti-dsDNA at onset of SLE. Persisting arthralgias and asthenia.
Japan (Ogata et al. 2010) 7 year old Male Mixed connective tissue disease after 2 months Weak anti-Ro, anti-La, anti-Sm, antiphospholipid and anti-RNP at onset of connective tissue disease Complete remission with corticosteroid therapy.
Japan (Ogata et al. 2010) 6 year old Female SLE after 2 years ANA, anti-dsDNA, anti-Sm, anti-RNP at onset of SLE. Complete remission with corticosteroid and cyclophosphamide pulse therapy.
Spain (Sopena et al. 2012) 4 cases 27–32 years old All Female Sjogren syndrome (2 cases) SLE (1 case) SLE-like (1 case) ANA, anti-Ro (Sjogren cases) ANA only (SLE-like case) 1 case had a near-fatal clinical course, treated with intravenous immunoglobulins. 2 of the remaining cases had relapses of lymphadenitis.
USA (Zuo et al. 2012) 23 year old Female SLE after three months ANA and rheumatoid factor at onset of lymphadenitis ANA, anti-Ro, anti-La, anti-dsDNA, anti-Smith, anti-RNP at onset of SLE Not stated.
India (Patra and Bhattacharya 2013) 30 year old Female SLE after 2 years ANA, anti-dsDNA at onset of SLE Complete remission with immunosuppression.
Italy (Di Lernia et al. 2013) 42 year old Female Cutanerous lupus after two months Anti-Ro, ENA at onset of cutaneous lupus Complete remission with prednisolone and hydroxychloroquine.
India (Patra and Bhattacharya 2014) 30 year old Female SLE after two years ANA and anti-dsDNA at onset of SLE Complete remission with immunosuppression