Kimura disease

A.Prof Frank Gaillard et al.

Kimura disease is a rare benign inflammatory disease that characteristically manifests as enlargement of cervical lymph nodes and salivary glands.

Kimura disease typically affects males (80%) between 20 and 40 years of age (80% of cases) 1-2, and is most frequently seen in Asia. Sporadic cases are seen in other geographic regions, however these are uncommon. An infectious agent is presumed to be the cause of an immunological response, however no specific pathogen has as yet been identified 4-5.

Presentation is with subcutanous masses and enlargement of the lymph nodes of the head and neck, particularly near the angle of the mandible and post-auricular groups. Salivary glands (particularly the parotid and submandibular glands)  and lymph nodes of the axilla, groin, popliteal fossa, medial epicondyle 6 and elsewhere may also be involved 1,4-5. They are usually not painful. Involvement of adjacent soft tissues is uncommon, although direct extension into the pinna of the ear has been described.

These changes are usually associated with eosinophilia in the peripheral blood and in tissues, and marked increase in serum levels of immunoglobulin E (IgE) 4-5.

There have been case reports of eosinophillic panniculitis 7 in patients with Kimura disease and some authors have reported presentations with nephrotic syndrome 8

Histologically Kimura disease is characterised by:

  • proliferation of lymphoid follicles
  • cellular infiltrates
    • eosinophils (majority), sometimes progressing to eosinophilic abscesses
    • also plasma cells lymphocytes, and mast cells
  • vascular proliferation of post-capillary venules
  • fibrosis

Peripheral eosinophilia is common.

Ultrasound is a useful modality for assessment of the neck and to aid in biopsy.
Sonographic features include 2:

  • solid, enlarged nodes sometimes maintaining hilar architecture
  • hypoechoic, usually homogeneous: ~90%
  • foci of necrosis uncommon: ~15%
  • increased vascularity usually in a hilar distribution: ~90%
  • salivary glands also hypoechoic, but usually more heterogeneous

CT demonstrates nonspecific appearances of

  • markedly enlarged cervical nodes +/- parotid and submandibular glands
  • intense enhancement of nodes
  • heterogenous enhancement of salivary glands

MRI signal intensity and enhancement varies depending on the amount of fibrosis and vascular proliferation present 1.

  • T1: hypointense or isointense compared with salivary tissue
  • T2
    • typically hyperintense compared to salivary gland tissue
    • variable according to degree fibrosis
  • T1 C+ (Gd): usually homogeneous enhancement

Kimura disease is benign, and no firmly established treatment protocols have been described. Options include 4-5:

  • conservative management
  • radiotherapy
  • steroid and oxyphenbutazone (with only transient improvement)1
  • resection
  • cyclosporine

Recurrence rate is high for steroids and surgery, and as such radiotherapy is favoured by some authors 4, although as the condition is benign and patients are young, not all agree 1. There were seven patients reportedly cured with cyclosporine (8).

First described in China by Kim and Szeto in 1937 and then in 1948 by Kimura et al in Japan 5.

Differential diagnosis on imaging is that of lymph node enlargement, and includes:

Histologically Kimura disease was initially thought to be related to angiolymphoid hyperplasia with eosinophilia (ALHE), however clinically and radiologically these two entities are clearly different:

  • ALHE affects primarily female Caucasians
  • ALHE involves dermis without lymph node enlargement
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Article information

rID: 1555
Synonyms or Alternate Spellings:
  • Eosinophylic lympogranuloma
  • Kimura lymphadenopathy
  • Eosinophilic hyperplastic lymphogranuloma
  • Kimura's disease

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Cases and figures

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    Kimura disease
    Case 1: CT C+
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    Kimura disease FS T2
    Case 1: MRI T2
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    Kimura disease FS...
    Case 1: MRS T1 FS C+
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