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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