今日の臨床サポート 今日の臨床サポート

著者: 樋口翔1) 大同病院膠原病・リウマチ内科

著者: 土師陽一郎2) 大同病院 膠原病・リウマチ内科

監修: 岸本暢将 杏林大学医学部 腎臓・リウマチ膠原病内科

著者校正/監修レビュー済:2025/04/23
患者向け説明資料

改訂のポイント
  1. 定期レビューを行った(変更なし)。

概要・推奨   

  1. 菊池病は1972年に本邦の菊池、藤本らによって初めて報告され、組織球性壊死性リンパ節炎とも呼ばれる[1][2]
  1. 若年女性に比較的多く、発熱・頸部リンパ節腫脹を特徴とする良性疾患で、アジアから多く報告されている。
  1. 多くは1ヵ月ほどで自然寛解するが、悪性リンパ腫、結核性(感染性)リンパ節炎、全身性エリテマトーデス(SLE)を除外することが重要である。

病態・疫学 

病態  
  1. 菊池病の病因は不明であるが、臨床症状、経過、組織学的変化から、感染因子に対するT細胞や組織球の免疫応答が示唆される。
  1. 感染源としてはEBウイルス、ヘルペスウイルス6型/8型、HIV、パルボウイルスB19などがあげられている[3][4][5][6]
疫学  
  1. 男女比は女性にやや多いと報告されているが(62〜85%)、小児では男女比が同等との報告もある[7][8][9][10]
  1. 比較的若年発症で、一部高齢発症もあるが、ほとんどの症例は40歳未満である。

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文献 

菊池昌弘:特異な組織像を呈するリンパ節炎について.日血会誌.1972;35:379–80.
藤本吉秀:頸部の亜急性壊死性リンパ節炎―新しい病態の提唱.内科.1972;30:920–7.
Yen A, Fearneyhough P, Raimer SS, Hudnall SD.
EBV-associated Kikuchi's histiocytic necrotizing lymphadenitis with cutaneous manifestations.
J Am Acad Dermatol. 1997 Feb;36(2 Pt 2):342-6. doi: 10.1016/s0190-9622(97)80413-6.
Abstract/Text The clinical and pathologic findings of Kikuchi's histiocytic necrotizing lymphadenitis may mimic those of malignant lymphoma. We describe a 6-year-old boy with generalized lymphadenopathy, spiking fever, chills, myalgias, malaise, and erythematous, crusted papules. Although cutaneous manifestations have been noted in 16% to 40% of patients with histiocytic necrotizing lymphadenitis, only three publications described skin lesions. The skin lesions and affected lymph nodes revealed histiocytic aggregates, atypical lymphoid cells, karyorrhectic debris, and patchy necrosis. Spontaneous resolution occurred in 2 months. Results of serologic studies, Epstein-Barr virus (EBV) latent membrane protein immunoperoxidase staining, EBER-1 RNA in-situ hybridization, and EBV EBNA-1 DNA polymerase chain reaction implicate EBV as the causative agent.

PMID 9039215
Hudnall SD, Chen T, Amr S, Young KH, Henry K.
Detection of human herpesvirus DNA in Kikuchi-Fujimoto disease and reactive lymphoid hyperplasia.
Int J Clin Exp Pathol. 2008 Jan 1;1(4):362-8. Epub 2008 Jan 1.
Abstract/Text Kikuchi-Fujimoto disease (KFD), or histiocytic necrotizing lymphadenitis, is a subacute inflammatory disorder most often seen in young women with clinicopathologic features suggestive of an infectious etiology. The most commonly suspected infectious agents in KFD are the human herpesviruses EBV, HHV6, HHV7 and HHV8. In order to identify herpesviruses in KFD, we have compared the frequency of detection of herpesvirus DNA with a recently developed real time PCR method, EBER in situ hybridization, and EBV latent membrane protein (LMP) immunostaining in 30 cases of KFD and 12 cases of reactive lymphoid hyperplasia (RLH). EBV DNA was commonly detected, while HSV2, CMV, HHV6, and HHV7 DNA were seldomly detected, and HSV1, VZV, and HHV8 DNA were not detected in KFD. EBV was also commonly detected in RLH. EBER-positive cells with apoptotic features were identified in necrotizing regions of many KFD cases, and LMP-positive cell debris was detected in one case. Viable EBER-positive cells were identified in four of twelve RLH cases, and rare LMP positivity detected in three cases. These data lend support to the notion that the necrotizing lesions in KFD may in some cases be due to a vigorous immune response to EBV-infected lymphoid cells.

PMID 18787614
Huh J, Kang GH, Gong G, Kim SS, Ro JY, Kim CW.
Kaposi's sarcoma-associated herpesvirus in Kikuchi's disease.
Hum Pathol. 1998 Oct;29(10):1091-6. doi: 10.1016/s0046-8177(98)90419-1.
Abstract/Text Kikuchi's disease is a fairly common self-limited disorder among Orientals that usually involves the cervical lymph nodes of young individuals and occurs predominantly in females. Frequently, the disease is associated with fever or flu-like symptoms, an elevated erythrocyte sedimentation rate (ESR), neutropenia, and lymphocytosis with atypical lymphocytes in the peripheral blood, suggesting a viral origin. However, no infectious agent has been identified. The presence of Kaposi sarcoma-associated herpesvirus, known as human herpesvirus 8 (KSHV/HHV 8), was investigated by polymerase chain reaction (PCR) in archival tissue from 26 cases of Kikuchi's disease using published sequences of KSHV/HHV 8 as primers. PCR products were further characterized by Southern blot analysis. Forty reactive lymph nodes and a case of Kaposi sarcoma (KS) were included as negative and positive controls, respectively. Patients consisted of 10 men and 16 women with a mean age of 27 years. All patients were previously healthy and presented with cervical or axillary lymphadenopathy. None were positive for human immunodeficiency virus (HIV) or otherwise immunocompromised. Viral DNA was amplified by PCR in six cases of Kikuchi's disease (23%) and the control KS tissue. Southern blot analysis confirmed that the amplified products were KSHV/HHV 8. In the reactive lymph nodes, no viral genome was amplified by PCR. The presence of DNA sequences of KSHV/HHV 8 in a substantial portion of Kikuchi's disease suggests that KSHV/HHV 8 might play an important role in the pathogenesis of a subset of Kikuchi's disease.

PMID 9781647
Yufu Y, Matsumoto M, Miyamura T, Nishimura J, Nawata H, Ohshima K.
Parvovirus B19-associated haemophagocytic syndrome with lymphadenopathy resembling histiocytic necrotizing lymphadenitis (Kikuchi's disease).
Br J Haematol. 1997 Mar;96(4):868-71. doi: 10.1046/j.1365-2141.1997.d01-2099.x.
Abstract/Text A 15-year-old girl developed a haemophagocytic syndrome caused by human parvovirus B19 (PVB19). The cervical lymph node histology, resembling that of histiocytic necrotizing lymphadenitis (HNL, Kikuchi's disease), included several transformed lymphocytes, numerous histocytes, and massive necrosis. We detected PVB19-positive cells in the lymph node by immunohistochemistry. Possible autoimmune mechanisms in HNL-like diseases triggered by PVB19 are discussed.

PMID 9074434
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.
Abstract/Text Kikuchi's necrotizing lymphadenitis has now become recognized in many parts of the world as a well-defined clinicopathologic entity with a remarkable predilection for cervical lymph nodes of young women. The morphologic features encountered in lymph nodes are distinctive and should enable pathologists to establish the diagnosis with confidence. Nonetheless, this analysis of 108 cases, encountered over a 15-year period, has emphasized the difficulty that many pathologists have in recognizing this disorder, and in particular, in distinguishing it from malignant lymphoma. Although in the vast majority of cases, lymphadenopathy and other symptomatology resolves spontaneously, two of our patients, thought initially to have Kikuchi's disease, developed systemic lupus erythematosus. This raises consideration for the proposal that Kikuchi's disease may reflect a self-limited SLE-like auto-immune condition (a "forme fruste" of SLE), perhaps induced by virus-infected transformed lymphocytes. Moreover, such observations indicate that patients with Kikuchi's disease should be kept under observation for several years to ensure that they are not at risk for the development of systemic lupus erythematosus.

PMID 3217625
Asano S, Akaike Y, Jinnouchi H, Muramatsu T, Wakasa H.
Necrotizing lymphadenitis: a review of clinicopathological, immunohistochemical and ultrastructural studies.
Hematol Oncol. 1990 Sep-Oct;8(5):251-60. doi: 10.1002/hon.2900080503.
Abstract/Text Necrotizing lymphadenitis (NEL) has been reported to be a reactive process described under differing terminology by Fujimoto et al. (1972), Kikuchi (1972), Wakasa et al. (1973) and other Japanese pathologists. Recently, this type of lymphadenitis has also been reported in America and Europe. In Japan, NEL is observed more frequently in the northern area, however, no characteristic seasonal occurrence has been noted. The disease affects young females more than males, particularly from the third and fourth decades onwards. Common cold-like symptoms, lymphadenopathy of the cervical region and leukopenia are characteristic clinical findings in the early stages. Morphological features of the involved lymph nodes include the presence of numerous immunoblasts, histiocytes and macrophages, the latter with phagocytized nuclear debris derived from degenerated lymphocytes. However, granulocytes are generally absent. Tubular inclusions are observed ultrastructurally. Immunohistochemical studies of peripheral blood using monoclonal antibodies have revealed that the helper/suppressor (Leu 3a/2a) ratio increases gradually with the clinical course because of a decrease in Leu 2a + cells. The pathogenesis of NEL is uncertain, but it has been speculated that there is cytolytic infection of lymphocytes by a virus or other organism, accompanied by secondary blastic transformation of suppressor T-lymphocytes.

PMID 2249796
Lin HC, Su CY, Huang CC, Hwang CF, Chien CY.
Kikuchi's disease: a review and analysis of 61 cases.
Otolaryngol Head Neck Surg. 2003 May;128(5):650-3. doi: 10.1016/S0194-59980223291-X.
Abstract/Text OBJECTIVE: Kikuchi's disease (KD), or histiocytic necrotizing lymphadenitis, is a rare self-limiting disorder that typically affects the cervical lymph nodes. It has occasionally been misdiagnosed as malignant lymphoma or another serious diseases; hence, clinicians should be made more aware of this disease.
METHODS: From January 1986 to January 2001, a series of 61 patients who underwent a biopsy of a cervical lymph node and proved histologically to have KD were enrolled in this study. We retrospectively reviewed the clinical records and pathologic parameters of each patient. The patients were followed up from 6 months to 14.3 years (mean, 6.9 years).
RESULTS: There were 34 women and 27 men (1.26:1 ratio; age range, 6 to 46 years; mean age, 21 years). The affected cervical lymph nodes were commonly located in the posterior cervical triangle (54 of 61, or 88.5%). Unilateral and bilateral cervical lymph nodes were affected in 54 and 7 patients, respectively. The dimensions of affected lymph nodes were commonly in the range of 0.5 to 4 cm (57 of 61, or 93.4%). In 2 patients, the size of the enlarged lymph nodes was greater than 6 cm. Leukopenia was observed in 14 patients (23%); fever, in 18 patients (29.5%). Five patients had a past history of tuberculosis. Two patients developed systemic lupus erythematosus, 1 month and 5 years later, respectively. The cervical lymphadenopathy usually resolved without any medical treatment within 6 months after definite diagnosis was made. In 3 patients, the enlarged lymph nodes eventually disappeared after more than 1 year. No recurrence has since been noted.
CONCLUSION: The results of this study support the theory that KD is a self-limiting disorder that does not require any specific management. The female predominance was not as striking as in the studies performed in Western countries. We suggest that the patients with KD require a systemic survey and regular follow-up for several years; 2 of our patients developed systemic lupus erythematosus. An effective communication between the otolaryngologist and pathologist is needed because the clinical and pathologic characteristics of KD are essential in making an accurate diagnosis.

PMID 12748557
Seo JH, Shim HS, Park JJ, Jeon SY, Kim JP, Ahn SK, Hur DG, Ahn SY, Kwon OJ.
A clinical study of histiocytic necrotizing lymphadenitis (Kikuchi's disease) in children.
Int J Pediatr Otorhinolaryngol. 2008 Nov;72(11):1637-42. doi: 10.1016/j.ijporl.2008.07.019. Epub 2008 Sep 11.
Abstract/Text BACKGROUND AND PURPOSE: Histiocytic necrotizing lymphadenitis (HNL) is a cervical lymphadenitis that occurs rarely in children. This study was conducted to identify the clinical characteristics of lymphadenitis in children and compare the clinical characteristics observed in children and adults.
SUBJECT AND METHODS: The clinical characteristics, diagnosis, treatment, and prognosis of the 20 patients 18 years of age or younger who were diagnosed with HNL based on histological tests conducted at Gyeongsang University Hospital from January 1998 to December 2006 were analyzed.
RESULTS: HNL affected males and females at a 1:1 ratio, but HNL was more common in boys (8:3) among the children and HNL affected more girls among the adolescents (2:7). Cervical lymphadenopathy was the main symptom. The antinuclear antibody test was positive in three patients. Two relapse cases were reported, but both patients recuperated within several months without complications.
CONCLUSIONS: HNL affected males and females at an equal incidence (1:1). But HNL occurred more often in male children than in female children, and more often in female adolescents than in male adolescents. Relapse occurred in 10% of the cases. HNL could progress into systemic lupus erythematosus in the three cases that were ANA-positive. Thus, a long follow-up period is important.

PMID 18789543
Asano S, Mori K, Yamazaki K, Sata T, Kurata A, Sato Y, Odajima H, Akaike Y, Wakasa H, Kojima M.
Necrotizing lymphadenitis (NEL) is a systemic disease characterized by blastic transformation of CD8+ cells and apoptosis of CD4+ cells.
Virchows Arch. 2014 Jan;464(1):95-103. doi: 10.1007/s00428-013-1516-z. Epub 2013 Nov 30.
Abstract/Text This clinicopathological, immunohistochemical, electron microscopic, and serological study of 382 cases (148 male, 234 female) of necrotizing lymphadenitis (NEL) in Japan confirms NEL as a self-limited disease with characteristic clinical features: high fever (38-40 °C), painful cervical lymphadenopathy (88.3 %), and leukopenia (under 4,000/mm(3)) without seasonal occurrence. Patient age varied from 5 to 80 years, but 62.8 % was younger than 30 years. There were five recurrent cases and four familial cases. In several cases, elevated serum aminotransaminase and antinuclear antibodies were found. Early in the disease, peripheral blood CD8+ cells were more abundant than CD4+ cells, but CD8+ cells decreased gradually with clinical progression, leading to an increasing ratio of CD4+/CD8+ cells during clinical course. Morphological features of involved lymph nodes are numerous CD8+ large immunoblasts, smaller CD4+ lymphocytes, plasmacytoid dendritic cells, histiocytes, and macrophages, the latter with phagocytized CD4+ apoptotic lymphocytes. Granulocytes are generally absent. These characteristics suggest that NEL is a reactive disease characterized by diploid disrupted CD4+ cells and CD8+ cells transforming to blastic cells. The etiology of the disease remains unknown, although viral infection is suggested, and its pathogenesis might include autoimmunity. Clinical characteristics and cytological and histological findings on lymph node biopsies can improve NEL diagnosis.

PMID 24292234
Kucukardali Y, Solmazgul E, Kunter E, Oncul O, Yildirim S, Kaplan M.
Kikuchi-Fujimoto Disease: analysis of 244 cases.
Clin Rheumatol. 2007 Jan;26(1):50-4. doi: 10.1007/s10067-006-0230-5. Epub 2006 Mar 15.
Abstract/Text Kikuchi-Fujimoto Disease (KFD) was first described in Japan in 1972. The disease frequently mimics tuberculous lymphadenitis, malign lymphoma, and many other benign and malignant conditions. To our knowledge, there is no previous study comparing the clinical and laboratory characteristics of patients from different geographical parts of the world. We searched literature records beginning from 1991 and analyzed epidemiological, clinical, and laboratory data of 244 patients (including cases diagnosed in our institution) reported in 181 publications. Of the 244 cases, 33% were male and 77% were female. Mean age was 25 (1-64) and 70% was younger than 30. Most of the cases were reported from Taiwan (36%), USA (6.6%), and Spain (6.3%). Fever (35%), fatigue (7%) and joint pain (7%) were the most frequent symptoms, while lymphadenomegaly (100%), erythematous rashes (10%), arthritis (5%), hepatosplenomegaly (3%), leucopenia (43%), high erythrocyte sedimentation rate (40%), and anemia (23%) being the most common findings. KFD was associated with SLE (32 cases), non-infectious inflammatory diseases (24 cases), and viral infections (17 cases). SLE was more frequent in cases from Asia than Europe (28 and 9%, respectively). The disease was self-limiting in 156 (64%) and corticosteroid treatment was necessary in 16 (16%) of the cases. The mortality rate was 2.1%. Early diagnosis is crucial as the clinical and laboratory presentation generally imitates situations needing lengthy and costly diagnostic and therapeutic interventions. Additionally, association with SLE needs further investigation.

PMID 16538388
Dumas G, Prendki V, Haroche J, Amoura Z, Cacoub P, Galicier L, Meyer O, Rapp C, Deligny C, Godeau B, Aslangul E, Lambotte O, Papo T, Pouchot J, Hamidou M, Bachmeyer C, Hachulla E, Carmoi T, Dhote R, Gerin M, Mekinian A, Stirnemann J, Charlotte F, Farge D, Molina T, Fain O.
Kikuchi-Fujimoto disease: retrospective study of 91 cases and review of the literature.
Medicine (Baltimore). 2014 Nov;93(24):372-382. doi: 10.1097/MD.0000000000000220.
Abstract/Text Kikuchi-Fujimoto disease (KFD) is a rare cause of lymphadenopathy, most often cervical. It has been mainly described in Asia. There are few data available on this disease in Europe. We conducted this retrospective, observational, multicenter study to describe KFD in France and to determine the characteristics of severe forms of the disease and forms associated with systemic lupus erythematosus (SLE). We included 91 cases of KFD, diagnosed between January 1989 and January 2011 in 13 French hospital centers (median age, 30 ± 10.4 yr; 77% female). The ethnic origins of the patients were European (33%), Afro-Caribbean (32%), North African (15.4%), and Asian (13%). Eighteen patients had a history of systemic disease, including 11 with SLE. Lymph node involvement was cervical (90%), often in the context of polyadenopathy (52%), and it was associated with hepatomegaly and splenomegaly in 14.8% of cases. Deeper sites of involvement were noted in 18% of cases. Constitutional signs consisted mainly of fever (67%), asthenia (74.4%), and weight loss (51.2%). Other manifestations included skin rash (32.9%), arthromyalgia (34.1%), 2 cases of aseptic meningitis, and 3 cases of hemophagocytic lymphohistiocytosis. Biological signs included lymphocytopenia (63.8%) and increase of acute phase reactants (56.4%). Antinuclear antibodies (ANAs) and anti-DNA antibodies were present in 45.2% and 18% of the patients sampled, respectively. Concomitant viral infection was detected in 8 patients (8.8%). Systemic corticosteroids were prescribed in 32% of cases, hydroxychloroquine in 17.6%, and intravenous immunoglobulin in 3 patients. The disease course was always favorable. Recurrence was observed in 21% of cases. In the 33 patients with ANA at diagnosis, SLE was known in 11 patients, diagnosed concomitantly in 10 cases and in the year following diagnosis in 2 cases; 6 patients did not have SLE, and 4 patients were lost to follow-up (median follow-up, 19 mo; range, 3-39 mo). The presence of weight loss, arthralgia, skin lesions, and ANA was associated with the development of SLE (p < 0.05). Male sex and lymphopenia were associated with severe forms of KFD (p < 0.05). KFD can occur in all populations, irrespective of ethnic origin. Deep forms are common. An association with SLE should be investigated. A prospective study is required to determine the risk factors for the development of SLE.

PMID 25500707
Kwon SY, Kim TK, Kim YS, Lee KY, Lee NJ, Seol HY.
CT findings in Kikuchi disease: analysis of 96 cases.
AJNR Am J Neuroradiol. 2004 Jun-Jul;25(6):1099-102.
Abstract/Text BACKGROUND AND PURPOSE: Although CT findings of Kikuchi disease (KD), or histiocytic necrotizing lymphadenitis, are reported in several case reports, large-scale analysis of the disease has not been undertaken. We characterized the clinical and CT findings in a large group of patients with KD.
METHODS: Between 1990 and 2002, 96 patients (68 women, 28 men; mean age, 24.4 years) underwent biopsy of the cervical lymph nodes and had histologically proved KD at our institution. We reviewed their clinical and CT findings and recorded the total number of affected nodes; location and size of the lymph nodes; and characteristic findings including necrosis, perinodal infiltration, and contrast enhancement.
RESULTS: We identified 1196 affected lymph nodes (12.5 nodes per patient). The affected lymph nodes were 0.5-3.5 cm (mean, 1.62 cm). Perinodal infiltration was found in 78 patients (81.3%). Eighty (83.3%) had homogeneous nodal contrast enhancement. Nine patients (9.4%) had lymph nodes with a focal low attenuation, and seven (7.3%) had ring-shaped lymph nodes. Unilateral and bilateral cervical lymph nodes were affected in 76 and 20 patients, respectively. Lymph nodes were mainly located at levels II (IIA, 174 nodes; IIB, 254 nodes), III (222 nodes), IV (160 nodes), and V (VA 126 nodes, VB 130 nodes). Follow-up CT in 32 patients showed complete resolution in 14 and partially improved lymphadenopathy in 18.
CONCLUSION: Awareness of the various CT appearances of KD and follow-up CT may be helpful for more accurate diagnosis of the disease.

PMID 15205157
Youk JH, Kim EK, Ko KH, Kim MJ.
Sonographic features of axillary lymphadenopathy caused by Kikuchi disease.
J Ultrasound Med. 2008 Jun;27(6):847-53. doi: 10.7863/jum.2008.27.6.847.
Abstract/Text OBJECTIVE: The purpose of this study was to document the sonographic findings of axillary lymphadenopathy in Kikuchi disease.
METHODS: The medical records and sonographic findings of 7 patients with a pathologic diagnosis of Kikuchi disease in the axillary lymph node by sonographically guided core needle biopsy (n=6) or excisional biopsy (n=1) were reviewed. On sonograms, lymph nodes were assessed for their distribution, size, shape, border, echogenicity, and internal architecture, and those sonographic features of each node were evaluated to determine whether the findings favored malignant or benign lymphadenopathy.
RESULTS: Of the 7 patients (1 man and 6 women; mean age +/- SD, 34.3+/-7.7 years), 29 affected lymph nodes (5-38 mm; mean, 14.8+/-7.2 mm) were identified on sonograms. The sonographic characteristics were as follows: the shortest axis/longest axis ratio of the node (mean, 0.595) was 0.5 or greater in 22 nodes (76%); the border was sharp in 16 (55%); the cortex was hypoechoic in 20 (69%) and isoechoic in 9 (31%); the hilum was narrow in 1 (4%) and absent in 16 (55%); and cortical thickening was found in 13 (45%, concentric in 6 and eccentric in 7). Nineteen lymph nodes (66%) were classified as having malignant-favoring features, and 10 (34%) were classified as having benign-favoring features.
CONCLUSIONS: Many axillary lymph nodes in Kikuchi disease look suspicious sonographically. When lymph nodes in the axilla show suspicious findings on sonograms of relatively young patients, Kikuchi disease can be considered a possible differential diagnosis, and image-guided percutaneous biopsy should be done.

PMID 18499844
Park SG, Koo HR, Jang K, Myung JK, Song CM, Ji YB, Park JS, Tae K.
Efficacy of Ultrasound-Guided Needle Biopsy in the Diagnosis of Kikuchi-Fujimoto Disease.
Laryngoscope. 2021 May;131(5):E1519-E1523. doi: 10.1002/lary.29160. Epub 2020 Oct 1.
Abstract/Text OBJECTIVES/HYPOTHESIS: Ultrasound-guided fine-needle aspiration cytology (US-FNAC) is a well-established procedure performed to establish the diagnosis of Kikuchi-Fujimoto disease (KFD). Ultrasound-guided core needle biopsy (US-CNB) is an alternative diagnostic tool for KFD. However, the efficacy of US-CNB is not well evaluated. This study aimed to evaluate the efficacy of US-CNB and compare it with that of US-FNAC in the diagnosis of KFD.
STUDY DESIGN: Retrospective cohort study.
METHODS: We analyzed 170 patients who were diagnosed with KFD between January 2009 and May 2019. US-FNAC, US-CNB, and excisional biopsy were performed in 47, 114, and 9 patients, respectively. Diagnostic accuracies of US-FNAC and US-CNB were analyzed and compared.
RESULTS: Of the 170 patients, 45 and 125 were men and women, respectively. The mean age was 26.9 ± 9.1 years. The most common symptom was cervical lymphadenopathy, followed by fever, headache, and myalgia. The diagnosis of KFD was established primarily by US-FNAC in 21 (44.7%) of the 47 patients, by US-CNB in 109 (95.6%) of the 114 patients, and by excisional biopsy in all 9 patients. There was no specific major complication related to US-FNAC and US-CNB.
CONCLUSION: US-CNB can be considered safe and effective and used as the primary modality for the pathological diagnosis of KFD.
LEVEL OF EVIDENCE: 4. Laryngoscope, 131:E1519-E1523, 2021.

© 2020 American Laryngological, Rhinological and Otological Society Inc, "The Triological Society" and American Laryngological Association (ALA).
PMID 33002212
Perry AM, Choi SM.
Kikuchi-Fujimoto Disease: A Review.
Arch Pathol Lab Med. 2018 Nov;142(11):1341-1346. doi: 10.5858/arpa.2018-0219-RA.
Abstract/Text Kikuchi-Fujimoto disease (KFD) is a rare entity characterized by subacute necrotizing lymphadenopathy and frequently associated with fever. Young adults of Asian ancestry are most commonly affected, but it has been reported worldwide. Despite many studies in the literature, the cause of KFD remains uncertain. Histologically, KFD is characterized by paracortical lymph node expansion with patchy, well-circumscribed areas of necrosis showing abundant karyorrhectic nuclear debris and absence of neutrophils and eosinophils. Three evolving histologic patterns-proliferative, necrotizing, and xanthomatous-have been recognized. By immunohistochemistry, histiocytes in KFD are positive for myeloperoxidase. There is a marked predominance of T cells in the lesions (with mostly CD8-positive cells) with very few B cells. The differential diagnosis of KFD includes infectious lymphadenitis, autoimmune lymphadenopathy (primarily systemic lupus erythematosus), and lymphoma. Clinicians and pathologists are poorly familiar with this entity, which frequently causes significant diagnostic challenges.

PMID 30407860
Tsang WY, Chan JK, Ng CS.
Kikuchi's lymphadenitis. A morphologic analysis of 75 cases with special reference to unusual features.
Am J Surg Pathol. 1994 Mar;18(3):219-31.
Abstract/Text Seventy-five cases of Kikuchi's lymphadenitis, a self-limiting pseudomalignant condition, were reviewed to determine the spectrum of histologic findings. There were 55 females and 20 males; ages ranged from 9 to 57 years (mean, 25.5). Most patients presented with cervical lymphadenopathy (68 cases). Associated clinical findings were fever (20/52) and leukopenia (15/33). Serum antinuclear antibodies were negative in 15 patients among 16 tested. Among 32 patients with follow-up information, 31 remained well, including one who developed recurrence after 2 years. One patient died of fatal myocardial disease during the active disease. Histologically, the lymph nodes showed paracortical hyperplasia, often associated with a starry-sky appearance resulting from interspersed histiocytes and immunoblasts. The consistent finding was the presence of variable-sized discrete or confluent nodules in the paracortex composed of the following: (a) karyorrhectic and eosinophilic granular debris; (b) histiocytes, many of which were phagocytic and possessed distinctive peripherally placed crescentic nuclei and voluminous cytoplasm containing eosinophilic or karyorrhectic debris (for which we propose the designation crescentic histiocytes), mixed with nonphagocytic histiocytes having twisted or reniform nuclei which were often centrally placed; (c) plasmacytoid monocytes, which were medium-sized cells with eccentrically placed round nuclei and amphophilic cytoplasm; and (d) variable numbers of immunoblasts, which sometimes showed atypia such as irregular nuclear foldings and coarse chromatin. Neutrophils were absent or very sparse. In some nodules, coagulative necrosis was present in the center (45 cases). Foamy histiocytes were found in 23 cases, and they predominated in 11. Small clusters of plasmacytoid monocytes were noted in the paracortex in 40 cases. Perinodal inflammation was a common finding, and perinodal involvement by the karyorrhectic process occurred in 15 cases. In addition, we found a number of previously unreported features. Signet-ring histiocytes with clear or homogeneous lightly amphophilic cytoplasm and nuclei compressed into thin crescents, found in seven cases, could mimic signet-ring cell adenocarcinoma. In three cases, some germinal centers were involved by the karyorrhectic process. Foci of lymphocyte-depleted fibrovascular organization were present in eight cases, probably representing the resolving phase of the karyorrhectic process. Despite the broad morphologic spectrum, the intermingling of the distinctive crescentic histiocytes, karyorrhectic debris, and plasmacytoid monocytes in the form of nodules, together with the paucity of neutrophils, are the consistent findings that should permit a confident histologic diagnosis of Kikuchi's lymphadenitis.

PMID 8116791
Jang YJ, Park KH, Seok HJ.
Management of Kikuchi's disease using glucocorticoid.
J Laryngol Otol. 2000 Sep;114(9):709-11. doi: 10.1258/0022215001906561.
Abstract/Text Histiocytic necrotizing lymphadenitis, or Kikuchi's disease, is a self-limiting cervical lymphadenitis of unknown origin. Since no specific treatment has been reported for Kikuchi's disease to date, once a diagnosis of Kikuchi's disease has been established, the role of the physician has been limited only to treating the symptoms. Sometimes, however, the clinical manifestations of the disease can be very distressing for the patients. Thus, a more aggressive form of treatment may be required for the patients who suffer from severe and persisting symptoms and recurrence. We present three cases of Kikuchi's disease that benefited significantly from systemic administration of prednisone.

PMID 11091837
Lin DY, Villegas MS, Tan PL, Wang S, Shek LP.
Severe Kikuchi's disease responsive to immune modulation.
Singapore Med J. 2010 Jan;51(1):e18-21.
Abstract/Text Kikuchi's disease, although an uncommon entity, has been increasingly reported since it was first discovered in 1972. The most common manifestation of Kikuchi's disease, cervical lymphadenopathy, has no clinically distinguishable features. Therefore, a diagnosis of Kikuchi's disease has largely been based on clinical suspicion and histopathological confirmation. We present a 15-year-old Chinese girl with severe Kikuchi's disease, whose relapsing course was only responsive to highdose steroid and intravenous immunoglobulin therapy.

PMID 20200761
Rezai K, Kuchipudi S, Chundi V, Ariga R, Loew J, Sha BE.
Kikuchi-Fujimoto disease: hydroxychloroquine as a treatment.
Clin Infect Dis. 2004 Dec 15;39(12):e124-6. doi: 10.1086/426144. Epub 2004 Nov 21.
Abstract/Text We describe a case of recurrent Kikuchi's disease in a South Asian-American man that was treated successfully with chloroquine and on recurrence with hydroxychloroquine. Each treatment led to a very prompt response.

PMID 15578393
Song JY, Lee J, Park DW, Sohn JW, Suh SI, Kim IS, Kim WJ, Kim MJ, Cheong HJ.
Clinical outcome and predictive factors of recurrence among patients with Kikuchi's disease.
Int J Infect Dis. 2009 May;13(3):322-6. doi: 10.1016/j.ijid.2008.06.022. Epub 2009 Feb 8.
Abstract/Text OBJECTIVES: To evaluate the clinical outcome and predictive factors of recurrence among patients with Kikuchi's disease.
METHODS: Between January 2001 and December 2006, all patients with Kikuchi's disease were included in the study. Data were collected on co-morbidities, clinical manifestations, and ultrasound/laboratory findings, and the differences were compared between recurrent disease and non-recurrent disease groups.
RESULTS: The study included 102 patients with a mean age of 26.7 years. Among these patients, three developed systemic lupus erythematosus during the follow-up period, while two cases were later associated with tuberculosis. Eight patients (7.8%) experienced early relapse and 13 (12.7%) showed late recurrence. Patients with recurrent episodes were more likely to have fever and fatigue with extranodal involvement. Compared to the non-recurrent cases, recurrent cases remained symptomatic for a rather longer duration. The positive rate of the fluorescence anti-nuclear antibody (FANA) test was significantly higher in the recurrent disease group compared to the non-recurrent disease group.
CONCLUSIONS: Kikuchi's disease took a self-limiting clinical course in most cases, but the recurrence rate found in the present study was higher than that of previous reports. Kikuchi's disease might be a phenotype of diverse disease entities. The prognosis is different according to the underlying cause. The FANA test would be useful in predicting recurrence.

PMID 19208493
薬剤監修について:
オーダー内の薬剤用量は日本医科大学付属病院 薬剤部 部長 伊勢雄也 以下、渡邉裕次、井ノ口岳洋、梅田将光および日本医科大学多摩永山病院 副薬剤部長 林太祐による疑義照会のプロセスを実施、疑義照会の対象については著者の方による再確認を実施しております。
※薬剤中分類、用法、同効薬、診療報酬は、エルゼビアが独自に作成した薬剤情報であり、 著者により作成された情報ではありません。
尚、用法は添付文書より、同効薬は、薬剤師監修のもとで作成しております。
※同効薬・小児・妊娠および授乳中の注意事項等は、海外の情報も掲載しており、日本の医療事情に適応しない場合があります。
※薬剤情報の(適外/適内/⽤量内/⽤量外/㊜)等の表記は、エルゼビアジャパン編集部によって記載日時にレセプトチェックソフトなどで確認し作成しております。ただし、これらの記載は、実際の保険適応の査定において保険適応及び保険適応外と判断されることを保証するものではありません。また、検査薬、輸液、血液製剤、全身麻酔薬、抗癌剤等の薬剤は保険適応の記載の一部を割愛させていただいています。
(詳細はこちらを参照)
著者のCOI(Conflicts of Interest)開示:
樋口翔 : 特に申告事項無し[2025年]
土師陽一郎 : 講演料(日本イーライリリー(株))[2024年]
監修:岸本暢将 : 講演料(日本イーライリリー(株),UCB,ブリストル・マイヤーズスクイブ(株),中外製薬(株),田辺三菱製薬(株),第一三共(株),アッヴィ合同会社,エーザイ(株),ヤンセンファーマ(株),旭化成ファーマ(株))[2025年]

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