Kimura's disease

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Kimura disease
Classification and external resources
Kimura disease - very high mag.jpg
Micrograph of Kimura disease. H&E stain.
DiseasesDB 31710
eMedicine article/1098777

Kimura disease is a benign rare chronic inflammatory disorder. Its primary symptoms are subdermal lesions in the head or neck or painless unilateral inflammation of cervical lymph nodes.[1]

History[edit]

The first known report of Kimura disease was from China in 1937, when Kimm and Szeto identified seven cases of the condition.[2] It first received its name in 1948 when Kimura and others noted a change in the surrounding blood vessels and referred to it as "unusual granulation combined with hyperplastic changes in lymphoid tissue."[3]

Cause[edit]

The cause of Kimura disease remains unknown. Reasons like an allergic reaction or an alteration of immune regulation are suspected. Other theories like persistent antigenic stimulation following arthropod bites and parasitic or candidal infection have also been proposed. To date, none of these theories have been substantiated.[1]

Pathophysiology[edit]

The pathophysiology of Kimura Disease remains unknown, although an allergic reaction, trauma, and an autoimmune process have all been implicated as the possible cause. The disease is manifested by an abnormal proliferation of lymphoid follicles and vascular endothelium. Peripheral eosinophilia and the presence of eosinophils in the inflammatory infiltrate suggest that Kimura Disease may be a hypersensitivity reaction. Some evidence has indicated that TH2 lymphocytes may also play a role, but further investigation is needed.

Kimura Disease is generally limited to the skin, lymph nodes, and salivary glands, but patients with Kimura Disease and nephrotic syndrome have been reported. The basis of this possible association is unclear.[1]

Frequency and Distribution[edit]

Kimura's Disease is said to be predominantly seen in males of Asian descent.[1]

However a study of 21 cases in the United States showed no racial preference, of 18 males and 3 females (male/female ratio 6:1), 8 to 64 years of age (mean, 32 years), there were 7 Caucasians, 6 Blacks, 6 Asians, 1 Hispanic, and 1 Arabic [4]

Treatment[edit]

Observation is acceptable if the lesions are neither symptomatic nor disfiguring. Intralesional or oral steroids can shrink the nodules but seldom result in cure.

Cyclosporine has been reported to induce remission in patients with Kimura Disease. However, recurrence of the lesions have been observed once this therapy is stopped.

Cetirizine is an effective agent in treating the symptoms of Kimura's disease. Cetirizine's properties of being effective both in the treatment of pruritus (itching) and as an anti-inflammatory agent make it suitable for the treatment of the pruritus associated with these lesions.[5] In a 2005 study, the American College of Rheumatology conducted treatments initially using prednisone, followed by steroid dosages and azathioprine, omeprazole, and calcium and vitamin D supplements over the course of two years.[5] The skin condition of the patient began to improve and the skin lesions lessened. However, there were symptoms of cushingoid and hirsutism observed before the patient was removed from the courses of steroids and placed on 10 mg/day of cetirizine to prevent skin lesions;[5] an agent suitable for the treatment of pruritus associated with such lesions.[5] Asymptomatically, the patient's skin lesions disappeared after treatment with cetirizine, blood eosinophil counts became normal,[5] corticosteroid effects were resolved,[5] and a remission began within a period of two months.[5] It is also thought that the inhibition of eosinophils may be the key to treatment of Kimura's disease due to the role of eosinophils, rather than other cells with regards to the lesions of the skin.[5]

Radiotherapy has been used to treat recurrent or persistent lesions. However, considering the benign nature of this disease, radiation should be considered only in cases of recurrent, disfiguring lesions.

Surgery has been considered the mainstay of therapy. However, recurrence after surgery is common.[6]

Intravenous immunoglobulin. In 2011 it was reported an 8 year old boy had presented with a firm, non-tender, non-fluctuating 15-12-cm mass on the left side of the neck involving the lateral region of the neck and jaw and a 5-7-cm mass on the right side of the neck. He had an eosinophil concentration of 36% (absolute count: 8172/mL), his IgE level was 9187 IU/mL. He was diagnosed with Kimura's disease. Initially treated with corticosteroids, he was given a single dose of intravenous immunoglobulin as a steroid sparing agent after the disease flared while tapering prednisone. After IVIG administration there was a rapid improvement, both left right cervical masses diminished to less than 1cm and his eosinophil and IgE levels returned to normal range. He has been free of disease during a 6 year follow up. It was suggested IVIG may have value in the treatment of Kimura's disease. [7]

Tacrolimus (FK-506) There's an ongoing study to assess the efficacy of tacrolimus on Kimura's disease. One case has so far been described. A patient with refractory Kimura's disease after surgery and treatment with prednisone was treated with tacrolimus. Tacrolimus (FK-506) was administered at an initial dosage of 1 mg every 12 hours, and FK-506 concentration in the blood was monitored monthly. FK-506 blood concentration was controlled within 5 to 15 μg/L. After 6 months, the dosage of tacrolimus was reduced to 0.5 mg daily for another 2 months and then treatment was stopped. Swelling of the bilateral salivary glands disappeared within the first week. No serious side effects were noted and the disease has not recurred in the 2 years of follow-up. Tacrolimus may be an effective treatment for patients with KD, but more research is needed to determine its long-term efficacy and safety as well as its mechanism of action. [8]

See also[edit]

References[edit]

  1. ^ a b c d Sena Lee (May 2007). "Kimura Disease". eMedicine. Retrieved 2008-07-20. 
  2. ^ Kimm HT, Szeto C (1937). "Eosinophilic hyperplastic lymphogranuloma, comparison with Mikulicz's disease". Proc Chin Med Soc: p. 329. 
  3. ^ Kimura T, Yoshimura S, Ishikawa E (1948). "On the unusual granulation combined with hyperplastic changes of lymphatic tissues". Trans Soc Pathol Jpn: pp. 179–180. 
  4. ^ http://www.ncbi.nlm.nih.gov/pubmed/15087670
  5. ^ a b c d e f g h Ben-Chetrit E, Amir G, Shalit M (February 2005). "Cetirizine: An effective agent in Kimura's disease". Arthritis Rheum. 53 (1): 117–8. doi:10.1002/art.20908. PMID 15696573. 
  6. ^ http://emedicine.medscape.com/article/1098777-treatment
  7. ^ http://pediatrics.aappublications.org/content/128/6/e1633.short
  8. ^ http://www.ncbi.nlm.nih.gov/pubmed/22939325