Kikuchi Disease

Kikuchi Disease

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Kikuchi disease, also called histiocytic necrotizing lymphadenitis or Kikuchi-Fujimoto disease, is an uncommon, idiopathic, generally self-limited cause of lymphadenitis. Kikuchi first described the disease in 1972 in Japan. Fujimoto and colleagues independently described Kikuchi disease in the same year. [1]

The most common clinical manifestation of Kikuchi disease is cervical lymphadenopathy, with or without systemic signs and symptoms. [2, 3, 4, 5] Clinically and histologically, the disease can be mistaken for lymphoma or systemic lupus erythematosus (SLE). [1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16]

Kikuchi disease almost always runs a benign course and resolves in several weeks to months. Disease recurrence is unusual, and fatalities are rare, although they have been reported. [1, 17]

In a retrospective study of 24 cases of Kikuchi disease in southern India over a 4-year period, the findings included the following [18] :

Treatment of Kikuchi disease is generally supportive. (See Treatment.) Pharmacologic therapy that has been recommended includes the following:

See also Cutaneous Kikuchi Disease

For patient education resources, see Swollen Lymph Nodes and Lymphoma.

The cause of Kikuchi disease is unknown, although infectious and autoimmune etiologies have been proposed. [19] The most favored theory proposes that Kikuchi disease results when one or more unidentified agents trigger a self-limited autoimmune process. Lymphadenitis results from apoptotic cell death induced by cytotoxic T lymphocytes. Some human leukocyte antigen (HLA) class II genes are more frequent in patients with Kikuchi disease, suggesting a genetic predisposition to the proposed autoimmune response. Case reports of Kikuchi disease associated with other autoimmune disorders, including Hashimoto thyroiditis, also support the hypothesis of an autoimmune etiology. [20]

Features that support a role for an infectious agent include the generally self-limited course of the disease and its frequent association with symptoms similar to those of upper respiratory tract infections (URTIs). Several viral candidates have been proposed, including cytomegalovirus, Epstein-Barr virus, [21] human herpesvirus, varicella-zoster virus, parainfluenza virus, parvovirus B19, and paramyxovirus. However, serologic and molecular studies have failed to link Kikuchi disease to a specific pathogen, and more than one pathogen may be capable of triggering the characteristic hyperimmune reaction leading to Kikuchi disease.

Several authors have reported an association between Kikuchi disease and SLE. [6, 8, 9, 11] {ref1239-INVALID REFERENCE} Kikuchi disease has been diagnosed before, during, and after a diagnosis of SLE was made in the same patient. Additionally, the histologic appearance of lymph nodes in patients with Kikuchi disease is similar to that of lymph nodes in patients with SLE lymphadenitis. Some authors have suggested that Kikuchi disease may represent a forme fruste SLE, but this theory has not been substantiated, and the association of Kikuchi disease with SLE, if any, remains unclear.

 

Although uncommon, Kikuchi disease has been reported throughout the world and in all races. Most cases have been reported from East Asia and Japan, with fewer cases from Europe and North America. [1]

The course of Kikuchi disease is generally benign and self-limited. Lymphadenopathy most often resolves over several weeks to 6 months, although the disease occasionally persists longer. The disease recurs in about 3% of cases. Four deaths have been reported, from hemophagocytic syndrome and severe infection, pulmonary hemorrhage, acute heart failure, and multiorgan failure and disseminated intravascular coagulation. [22]

Kikuchi disease was first diagnosed and described in Japan. To date, most cases have been reported from East Asia. More recently, the disease has been reported throughout the world and in all races. Outside of Asia, it is possible that Kikuchi disease has been underdiagnosed and therefore underreported. Dorfman and Berry reported 108 cases, including 68 in the United States; 63% of the 108 patients were white. [6] In a study of 91 cases from France, Dumas et al reported increased risk for severe disease in patients of North African origin. [12]

Kikuchi disease is reported more often in women, with a female-to-male ratio as high as 4:1 in some studies. [1] However, other studies have shown a smaller female preponderance, with a ratio closer to 2:1. [23]

A study of Korean patients by Jung et al reported clinical differences between males and females with Kikuchi disease. Females were more likely to have autoimmune features, including antinuclear antibodies, while males were more likely to present with a profile of fever, headache, bilateral lymphadenopathy, thrombocytopenia, and elevated levels of liver enzymes, C-reactive protein, and lactate dehydrogenase (LDH). [24]

Kikuchi disease occurs in a wide age range of patients (ie, 2-75 y), but it typically affects young adults (mean age, 20-30 y). In a Korean study of children with severe Kikuchi disease, the mean age was 13.2 years, and the male-to-female ratio was 1:1.32. [25]

Masab M, Farooq H. Kikuchi Disease. Clin Rheumatol. 2017 Jun. 27(8):1073-5. [Medline]. [Full Text].

Bosch X, Guilabert A, Miquel R, Campo E. Enigmatic Kikuchi-Fujimoto disease: a comprehensive review. Am J Clin Pathol. 2004 Jul. 122(1):141-52. [Medline].

Alam H, Saeed MO, Saeed AB, Zaidi A. Kikuchi disease. J Pak Med Assoc. 2015 Dec. 65 (12):1349-50. [Medline].

Lamzaf L, Harmouche H, Maamar M, Adnaoui M, Aouni M, Tazi Mezalek Z. Kikuchi-Fujimoto disease: report of 4 cases and review of the literature. Eur Ann Otorhinolaryngol Head Neck Dis. 2014 Dec. 131 (6):329-32. [Medline].

Kuo TT. Kikuchi’s disease (histiocytic necrotizing lymphadenitis). A clinicopathologic study of 79 cases with an analysis of histologic subtypes, immunohistology, and DNA ploidy. Am J Surg Pathol. 1995 Jul. 19(7):798-809. [Medline].

Dorfman RF, Berry GJ. Kikuchi’s histiocytic necrotizing lymphadenitis: an analysis of 108 cases with emphasis on differential diagnosis. Semin Diagn Pathol. 1988 Nov. 5(4):329-45. [Medline].

Gallien S, Lagrange-Xelot M, Crabol Y, et al. [Systemic lupus erythematosus and Kikuchi-Fujimoto disease mimicking tuberculosis] [French]. Med Mal Infect. 2008 Jun 17. epub ahead of print. [Medline].

Yilmaz M, Camci C, Sari I, et al. Histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto’s disease) mimicking systemic lupus erythematosus: a review of two cases. Lupus. 2006. 15(6):384-7. [Medline].

Poulose V, Chiam P, Poh WT. Kikuchi’s disease: a Singapore case series. Singapore Med J. 2005 May. 46(5):229-32. [Medline]. [Full Text].

Scagni P, Peisino MG, Bianchi M, et al. Kikuchi-Fujimoto disease is a rare cause of lymphadenopathy and fever of unknown origin in children: report of two cases and review of the literature. J Pediatr Hematol Oncol. 2005 Jun. 27(6):337-40. [Medline].

Hedia G, Jamel A, Maher A, et al. Kikuchi-Fujimoto disease associated with systemic lupus erythematosus. J Clin Rheumatol. 2005 Dec. 11(6):341-2. [Medline].

Dumas G, Prendki V, Haroche J, Amoura Z, Cacoub P, et al. Kikuchi-Fujimoto disease: retrospective study of 91 cases and review of the literature. Medicine (Baltimore). 2014 Nov. 93 (24):372-82. [Medline]. [Full Text].

Spies J, Foucar K, Thompson CT, LeBoit PE. The histopathology of cutaneous lesions of Kikuchi’s disease (necrotizing lymphadenitis): a report of five cases. Am J Surg Pathol. 1999 Sep. 23(9):1040-7. [Medline].

Sierra ML, Vegas E, Blanco-González JE, et al. Kikuchi’s disease with multisystemic involvement and adverse reaction to drugs. Pediatrics. 1999 Aug. 104(2):e24. [Medline]. [Full Text].

Lee S, Yoo JH, Lee SW. Kikuchi Disease: Differentiation from Tuberculous Lymphadenitis Based on Patterns of Nodal Necrosis on CT. AJNR Am J Neuroradiol. 2011 Dec 15. [Medline].

Găman M, Vlădăreanu AM, Dobrea C, Onisâi M, Marinescu C, Voican I, et al. A Challenging Case of Kikuchi-Fujimoto Disease Associated with Systemic Lupus Erythematosus and Review of the Literature. Case Rep Hematol. 2018. 2018:1791627. [Medline]. [Full Text].

Hutchinson CB, Wang E. Kikuchi-Fujimoto disease. Arch Pathol Lab Med. 2010 Feb. 134(2):289-93. [Medline].

Supari D, Ananthamurthy A. Kikuchi-fujimoto disease: a study of 24 cases. Indian J Otolaryngol Head Neck Surg. 2014 Jan. 66(1):69-73. [Medline]. [Full Text].

Sopeña B, Rivera A, Vázquez-Triñanes C, Fluiters E, González-Carreró J, Del Pozo M, et al. Autoimmune Manifestations of Kikuchi Disease. Semin Arthritis Rheum. 2011 Dec 20. [Medline].

Lee EJ, Lee HS, Park JE, Hwang JS. Association Kikuchi disease with Hashimoto thyroiditis: a case report and literature review. Ann Pediatr Endocrinol Metab. 2018 Jun. 23 (2):99-102. [Medline]. [Full Text].

Hudnall SD. Kikuchi-Fujimoto disease. Is Epstein-Barr virus the culprit?. Am J Clin Pathol. 2000 Jun. 113(6):761-4. [Medline]. [Full Text].

Barbat B, Jhaj R, Khurram D. Fatality in Kikuchi-Fujimoto disease: A rare phenomenon. World J Clin Cases. 2017 Feb 16. 5 (2):35-39. [Medline]. [Full Text].

Shim EJ, Lee KM, Kim EJ, Kim HG, Jang JH. CT pattern analysis of necrotizing and nonnecrotizing lymph nodes in Kikuchi disease. PLoS One. 2017. 12 (7):e0181169. [Medline]. [Full Text].

Jung IY, Ann HW, Kim JJ, Lee SJ, Kim J, Seong H, et al. The incidence and clinical characteristics by gender differences in patients with Kikuchi-Fujimoto disease. Medicine (Baltimore). 2017 Mar. 96 (11):e6332. [Medline]. [Full Text].

Kang HM, Kim JY, Choi EH, Lee HJ, Yun KW, Lee H. Clinical Characteristics of Severe Histiocytic Necrotizing Lymphadenitis (Kikuchi-Fujimoto Disease) in Children. J Pediatr. 2016 Feb 4. [Medline].

Atwater AR, Longley BJ, Aughenbaugh WD. Kikuchi’s disease: case report and systematic review of cutaneous and histopathologic presentations. J Am Acad Dermatol. 2008 Jul. 59(1):130-6. [Medline].

Yasukawa K, Matsumura T, Sato-Matsumura KC, et al. Kikuchi’s disease and the skin: case report and review of the literature. Br J Dermatol. 2001 Apr. 144(4):885-9. [Medline].

Khishfe BF, Krass LM, Nordquist EK. Kikuchi disease presenting with aseptic meningitis. Am J Emerg Med. 2014 Oct. 32 (10):1298.e1-2. [Medline].

Singh YP, Agarwal V, Krishnani N, Misra R. Enthesitis-related arthritis in Kikuchi-Fujimoto disease. Mod Rheumatol. 2008 May 10. epub ahead of print. [Medline].

Tong TR, Chan OW, Lee KC. Diagnosing Kikuchi disease on fine needle aspiration biopsy: a retrospective study of 44 cases diagnosed by cytology and 8 by histopathology. Acta Cytol. 2001 Nov-Dec. 45(6):953-7. [Medline].

Viguer JM, Jiménez-Heffernan JA, Pérez P, et al. Fine-needle aspiration cytology of Kikuchi’s lymphadenitis: a report of ten cases. Diagn Cytopathol. 2001 Oct. 25(4):220-4. [Medline].

Kwon SY, Kim TK, Kim YS, et al. CT findings in Kikuchi disease: analysis of 96 cases. AJNR Am J Neuroradiol. 2004 Jun-Jul. 25(6):1099-102. [Medline]. [Full Text].

Kato H, Kanematsu M, Kato Z, Teramoto T, Kondo N, Hirose Y, et al. MR imaging findings of cervical lymphadenopathy in patients with Kikuchi disease. Eur J Radiol. 2011 Dec. 80 (3):e576-81. [Medline].

Ryoo I, Suh S, Lee YH, Seo HS, Seol HY. Comparison of Ultrasonographic Findings of Biopsy-Proven Tuberculous Lymphadenitis and Kikuchi Disease. Korean J Radiol. 2015 Jul-Aug. 16 (4):767-75. [Medline]. [Full Text].

Tabata T, Takata K, Miyata-Takata T, Sato Y, Ishizawa S, Kunitomo T, et al. Characteristic Distribution Pattern of CD30-positive Cytotoxic T Cells Aids Diagnosis of Kikuchi-Fujimoto Disease. Appl Immunohistochem Mol Morphol. 2018 Apr. 26 (4):274-282. [Medline].

Jang YJ, Park KH, Seok HJ. Management of Kikuchi’s disease using glucocorticoid. J Laryngol Otol. 2000 Sep. 114(9):709-11. [Medline].

Hyun M, So IT, Kim HA, Jung H, Ryu SY. Recurrent Kikuchi’s Disease Treated by Hydroxychloroquine. Infect Chemother. 2016 Jun. 48 (2):127-31. [Medline].

Kishimoto K, Tate G, Kitamura T, Kojima M, Mitsuya T. Cytologic features and frequency of plasmacytoid dendritic cells in the lymph nodes of patients with histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto disease). Diagn Cytopathol. 2009 Nov 25. epub ahead of print. [Medline].

John Boone, MD Consulting Staff, Department of Otolaryngology, Naval Hospital Oak Harbor

John Boone, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery

Disclosure: Nothing to disclose.

Charles S Kuzma, MD Consulting Staff, Clinical Research Coordinator, First Health of the Carolinas Cancer Center

Charles S Kuzma, MD is a member of the following medical societies: American College of Physicians, American Society of Hematology, California Medical Association

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Lawrence H Brent, MD Associate Professor of Medicine, Sidney Kimmel Medical College of Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center

Lawrence H Brent, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Physicians, American College of Rheumatology

Disclosure: Stock ownership for: Johnson & Johnson.

Koyamangalath Krishnan, MD, FRCP, FACP Dishner Endowed Chair of Excellence in Medicine, Professor of Medicine, James H Quillen College of Medicine at East Tennessee State University

Koyamangalath Krishnan, MD, FRCP, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, American Society of Hematology, Royal College of Physicians

Disclosure: Nothing to disclose.

Karen Seiter, MD Professor, Department of Internal Medicine, Division of Oncology/Hematology, New York Medical College

Karen Seiter, MD is a member of the following medical societies: American Association for Cancer Research, American College of Physicians, American Society of Hematology

Disclosure: Received honoraria from Novartis for speaking and teaching; Received consulting fee from Novartis for speaking and teaching; Received honoraria from Celgene for speaking and teaching.

Kikuchi Disease

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