Miscellaneous
Autoimmune Manifestations of Kikuchi Disease

This work was partially presented at the Annual European Congress of Rheumatology EULAR2010, Rome, Italy, 16-19 June 2010.
https://doi.org/10.1016/j.semarthrit.2011.11.001Get rights and content

Objectives

Kikuchi's disease (KD) has been associated with the presence of autoantibodies, systemic lupus erythematosus (SLE), and other autoimmune diseases. The aim of this study was to assess the frequency of autoimmune manifestations in a KD cohort with a long follow-up.

Methods

Twenty patients with histologically confirmed KD since January 1990 until December 2010 were studied; 12 of them were periodically followed up as outpatients. Another 7 patients were contacted by telephone to offer them a specific consultation and a complete autoimmunity study.

Results

Thirteen of 20 patients were women (65%) with a mean age of 29 years (range, 15-79). The age at diagnosis was higher in men (44 vs 27 years, P < 0.05). Lymphopenia was present in 75% of the patients (15/20) and was the more frequent hematological abnormality. The mean follow-up of the 17 patients included in the autoimmunity study was 119 months (range, 15-252). Autoimmune diseases were detected in 9 women (53%): SLE was diagnosed in 4 patients (2 SLE before, 1 simultaneous, and 1 after KD), 2 patients developed primary Sjögren's syndrome after KD, 1 thyroiditis before KD, 1 SLE-like, and 1 antiphospholipid antibodies after KD. Leukocytoclastic vasculitis was found in 2 patients; 1 of them eventually developed SLE. Female sex, painful adenopathies, and cytopenias were significantly associated with autoimmune diseases.

Conclusions

Among patients with KD, only women developed autoimmune manifestations. Therefore, long-term follow-up and active surveillance of autoimmune diseases in patients with KD, especially women, are recommended.

Section snippets

Methods

This study includes 20 patients who were diagnosed in our hospital with histologically confirmed KD. The “Complexo Hospitalario Universitario of Vigo (CHUVI)” has 1250 beds and attends a population that is half urban and half rural, of more than 600,000 inhabitants.

From January 1990 until December 2010, 13 patients with KD were diagnosed and 12 were followed as outpatients with periodic examinations and autoimmunity studies. In 7 additional cases, the electronic records were reviewed and the

General Characteristics of Patients with KD

During the 20-year period, 20 consecutive patients were diagnosed with KD, 13 women (65%) and 7 men (35%) (ratio of women/men, 1.86:1). The mean age at diagnosis was 29 years (range, 15-79). This was higher in men (44 vs 27 years, P < 0.05). Of the 20 patients, 11 were diagnosed with KD during the hospital stay; 5 were diagnosed as outpatients when they were referred for adenopathy study. In the other 3 previously reported cases (8), the disease started after 2 weeks in the hospital for another

Discussion

KD is a rare entity in Western countries that accounts for 0.77% of diagnosis from lymph node biopsies and for which there are few clinical series with more than 9 patients and a prolonged follow-up time (11, 12). This study includes 20 white patients diagnosed with KD with a long follow-up, 17 of them specifically studied to detect the appearance of autoimmune diseases.

Although the general demographic, clinical, and analytical characteristics of the present series were similar to those

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