Background Kikuchi disease (KD) and systemic lupus erythematosus (SLE) share many clinical and histological features. The association of both entities has been reinforced over time with the publication of numerous case reports and small series (1). Some reviews about this topic were also published (2-3). However, no systematic review of the literature describing the characteristics of patients with KD and SLE has been performed to date.
Objectives To describe the clinical, laboratory and outcome of reported cases of SLE in adult patients with KD.
Methods We conducted a systematic literature review by searching through the databases MEDLINE (source: PubMed, 1996 to July 2011), EMBASE (1980 to July 2011) and Web of Science. The following terms were used: “Kikuchi”, “Lupus” and “Histiocytic necrotizing lymphadenitis” combining as MeSH terms and text words, without language restrictions. Studies were eligible when included adults (>14-years-age) and contained sufficient clinical and analytical data to establish whether the patient met the ACR criteria for SLE and if there was histological confirmation of KD diagnosis. Clinical, immunological and analytical variables were gathered.
Results The search strategy yielded 115 papers of which 63 (54%) were excluded because insufficient data or because patients did not meet criteria for SLE. The article could not be recovered in 21 cases (18%). So, 67 patients with confirmed KD and definite SLE were included, of them 52 were women (female/male ratio 1:3.5). The mean age at KD diagnosis was 30 years (range 14-56). With respect to the time of diagnosis: SLE preceded KD in 10 (15%), was simultaneous with KD in 36 cases (54%), and after KD in 21 cases (31%). SLE was diagnosed an average of 23 months after KD (range 1-120), but in 45% of patients the diagnosis was made in the first year. The most frequent haematological abnormality was leukopenia in 75.5% of cases, especially at the expense of lymphopenia (75%), neutropenia was recorded in only 22% of patients. ANA (99%) and anti-DNA-native (60%) were the commonest auto-antibodies recorded, followed by anti-Ro (40%), anti-Sm (26%), anti-RNP (22%) and anti-cardiolipin (19%). Only 13% of SLE were diagnosed with lupus nephritis confirmed by renal biopsy (3 type II, 2 type III, 3 type 1 type IV and V).
Conclusions Although most patients with KD and SLE were diagnosed simultaneously, one-third of SLE were developed after KD in some cases several years thereafter. Although 60% of SLE patients had anti-DNA native, only 13% of patients developed lupus nephritis.
Sopeña B, Rivera A, Vázquez-Triñanes C, et al. Autoimmune manifestations of Kikuchi Disease. Semin Arthritis Rheum 2011; Dec 20 [Epub ahead of print].
Kucukardali Y, Solmazgul E, Kunter E, et al. Kikuchi-Fujimoto disease: analysis of 244 cases. Clin Rheumatol 2007; 26:50-4.
Santana A, Lessa B, Galrao L, Líma I, Santiago M. Kikuchi-Fujimoto’s disease associated with systemic lupus erythematosus: case report and review of the literarture. Clin Rheumatol 2005; 24:60-3.
Disclosure of Interest None Declared
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