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Table 2 Clinical characteristics of previously reported cases of Kikuchi-Fujimoto disease occurring simultaneously with 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 occurring simultaneously with connective tissue disease

Country and reference

Age and gender

Connective tissue disease

Autoantibodies

Outcome

Greece (Gionanlis et al. 2009)

23 year old Female

SLE

ANA, anti-dsDNA, Anti-Ro, Anti-La, anti-RNP

Renal failure.TTP. Responded to immunosuppression + rituximab.

USA (Smith and Petri 2013)

27 year old Female

SLE

ANA, anti dsDNA, anticardiolipin IgG and IgM, anti-Smith, anti-Ro, anti-La.

Developed lupus nephritis. Condition stable.

Japan (Aota et al. 2009)

36 year old Male

SLE

ANA

Lymphadenitis resolved with corticosteroid therapy. Developed lupus nephritis. Condition stable at 4 month follow up.

France (Gallien et al. 2008)

47 year old Male

SLE

ANA, anti-dsDNA

Lymphadenitis resolved with corticosteroid therapy.

France (Frikha et al. 2008)

14 year old Female

SLE

ANA, anti-RNP, anti-Ro

Lymphadenitis resolved with corticosteroid therapy.

France (Frikha et al. 2008)

23 year old Female

SLE

ANA, anti-dsDNA, anti-Sm, anti-RNP, anti-Ro, anti-La.

Lymphadenitis resolved with corticosteroid therapy.

Germany (Hoffmann et al. 1991)

37 year old Male

SLE

HHV-6

No life-threatening sequelae.

England (Shusang et al. 2008)

20 year old Female

SLE Had 3 year history of autoimmune hepatitis

ANA (at onset of autoimmune hepatitis) Anti-Ro, anticardiolipin (at onset of SLE)

Lymphadenitis resolved with corticosteroid therapy.

Turkey (Yilmaz et al. 2006)

53 year old Female

SLE

ANA

No life-threatening sequelae.

Singapore (Chua et al. 1996)

9 year old Female

SLE with erythema multiforme

ANA, anti-dsDNA

No life-threatening sequelae.

USA (Eisner et al. 1996)

Young male

SLE

ANA

Concurrent lupus nephritis. Complete remission with corticosteroid therapy and cyclophosphamide.

France (Leyral et al. 2005)

26 year old Female

SLE

ANA, anti-dsDNA

Haemophagocytic syndrome with EBV infection. Complete remission with corticosteroids.

Romania (Tanasescu et al. 2003)

17 year old Female

SLE and autoimmune hepatitis

Anti-Sm

Complete remission with corticosteroid therapy

Brazil (Santana et al. 2005)

20 year old Female

SLE

ANA, anti-Ro, anticardiolipin

Lymphadenitis resolved with corticosteroid therapy.

Spain (Jimenez Saenz et al. 2001)

30 year old Female

SLE

ANA, anti-dsDNA, anti-RNP, anti-scl 70

Complete remission with corticosteroid therapy.

USA (Mahajan et al. 2007)

56 year old Female

SLE

ANA, anti-dsDNA

Partial improvement with hydroxychloroquine, followed by complete remission.

USA (Gordon et al. 2009)

33 year old Male

SLE

ANA, anti-Smith, anti-RNP, anti-Ro.

Complete remission with corticosteroid therapy.

Germany (Cramer et al. 2010)

33 year old Male

SLE

ANA, anti-dsDNA

Clinical improvement with corticosteroid and mycophenolate therapy.

USA (Quintas-Cardama et al. 2003)

38 year old Female

SLE

ANA

Severe auto-immune-related clinical sequelae and cardiogenic shock. Patient died despite pulse corticosteroid and cyclophosphamide therapy.

Spain (Sopena et al. 2012)

27 year old Female

SLE

ANA

Good response to therapy.

Spain (Diez-Morrondo et al. 2012)

22 year old Female

SLE

ANA

Good response to hydroxychloroquine.

Thailand (Kampitak 2008)

50 year old Male

SLE

ANA

Developed haemophagocytic syndrome and severe hospital acquired pneumonia and septic shock. Patient died.