Kikuchi–Fujimoto disease developed into autoimmune disease: a report of two cases

Ogata, Shohei; Bando, Yuki; Saito, Norimitsu; Katsuoka, Kensei; Ito, Masahiro

doi:10.1007/s10165-009-0269-7

Cited by 10 publications

(9 citation statements)

References 19 publications

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“…Apart from systemic lupus erythematosus, Kikuchi-Fujimoto disease was also reported with mixed connective tissue disease, anti-phospholipid syndrome, thyroiditis, polymyositis, scleroderma, autoimmune hepatitis and adult-onset Still's disease [9,21,22,30]. Only a few studies have also suggested that KFD may be associated with pSS [11][12][13][14][15][16][17][18].…”

Section: Discussionmentioning

confidence: 99%

“…Ogata et al [15] reported a male, diagnosed with KFD and treated with prednisone for three months. One and a half years later, due to a relapse of symptoms, pSS diagnosis was established.…”

Section: Discussionmentioning

confidence: 99%

“…This is a reason why lymphadenopathy, a possible symptom of pSS, needs careful diagnostic follow-up. The correlation of pSS and KFD has been previously noted worldwide and may impact further diagnostic procedures and treatment [11][12][13][14][15][16][17][18].…”

Section: Introductionmentioning

confidence: 99%

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Kikuchi-Fujimoto disease associated with primary Sjögren’s syndrome – literature review based on a case report

Wiśniewska¹,

Pawlak-Buś²,

Leszczyński³

2020

Reumatologia

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Primary Sjögren's syndrome (pSS) is a chronic, autoimmune disease predominantly involving exocrine glands. Lymphadenopathy is one of the possible symptoms of pSS. It may also suggest development of non-Hodgkin lymphoma (NHL), the most severe complication of pSS, or be a symptom of less common diseases, such as Kikuchi-Fujimoto disease (KFD), presented in this paper. Kikuchi-Fujimoto disease is an extremely rare, benign and self-limiting disorder, characterized by regional lymphadenopathy. This paper presents a case of previously unreported association of pSS, KFD and renal cancer in a patient with recurrent cervical lymphadenopathy, as well as a discussion on the coexistence of these diseases based on available literature searching for PubMed, Scopus and Google Scholar databases, particularly in this subject. These three clinical entities may manifest lymphadenopathy each, causing a diagnostic dilemma. The treatment is also challenging under such circumstances. In this particular situation, it was a combination of immunosuppressive therapy and surgery.

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Section: Discussionmentioning

confidence: 99%

“…Ogata et al [15] reported a male, diagnosed with KFD and treated with prednisone for three months. One and a half years later, due to a relapse of symptoms, pSS diagnosis was established.…”

Section: Discussionmentioning

confidence: 99%

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Kikuchi-Fujimoto disease associated with primary Sjögren’s syndrome – literature review based on a case report

Wiśniewska¹,

Pawlak-Buś²,

Leszczyński³

2020

Reumatologia

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“…Suspeita-se de mecanismos patogenéticos semelhantes na DKF e no LES; acredita-se que ocorra ativação linfocitária desencadeada por um mesmo fator causal, mas, provavelmente não existe uma causa única para a DKF. 10 A Doença de Kikuchi-Fujimoto é uma doença rara, pouco conhecida e subdiagnosticada no Brasil. Pode ter um curso benigno, devendo ser considerada como diagnóstico diferencial de adenopatias, uma vez que pode estar associada a doenças autoimunes, infecciosas ou linfoproliferativas.…”

Section: Discussionunclassified

Doença de Kikuchi-Fujimoto: apresentação de um caso

Oliveira¹,

Galdino²

2017

Rev. Med. UFC

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A doença de Kikuchi-Fujimoto (DKF) ou linfadenite histiocítica necrotizante é uma doença benigna e auto-limitada, rara, caracterizada por linfadenopatia generalizada, febre e leucopenia. É um importante diagnóstico diferencial com patologias linfoproliferativas, autoimunes, infecciosas, podendo em raros casos estar associada a estas. Relatamos o caso de um paciente de 24 anos, com quadro de febre, linfadenopatia cervical e inguinal e lesões cutaneomucosas. O Fator Anti-Nuclear (FAN) foi positivo, hemograma evidenciou pancitopenia, anti-DNA negativo. A biópsia de linfonodo inguinal evidenciou linfadenite histiocítica necrotizante. O paciente evoluiu com piora clínica por atividade de doença associada a sepse, evoluindo com pericardite aguda e tamponamento cardíaco. Realizado pulsoterapia com ciclofosfamida, com melhora substancial das manifestações cutâneas e sistêmicas. O diagnóstico de pacientes com DKF deve implicar necessariamente no seguimento para avaliação do surgimento de doenças autoimunes, assim como doenças infecciosas e linfoproliferativas.

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“… Japan (Ogata et al 2010 ) 7 year old Male Mixed connective tissue disease after 2 months Weak anti-Ro, anti-La, anti-Sm, antiphospholipid and anti-RNP at onset of connective tissue disease Complete remission with corticosteroid therapy. Japan (Ogata et al 2010 ) 6 year old Female SLE after 2 years ANA, anti-dsDNA, anti-Sm, anti-RNP at onset of SLE. Complete remission with corticosteroid and cyclophosphamide pulse therapy.…”

Section: Discussionmentioning

confidence: 99%

Fatal Kikuchi-like lymphadenitis associated with connective tissue disease: a report of two cases and review of the literature

Sharma

Rankin

2015

SpringerPlus

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IntroductionKikuchi-Fujimoto disease, is usually a benign self-limiting disease which typically affects young females under the age of 30 years and resolves without treatment within six months. However, when it occurs in the context of connective tissue disease, particularly systemic lupus erythematosus (SLE), it is usually associated with a flare-up of the patient’s symptoms, requiring treatment, and can lead to severe, potentially life-threatening sequelae.Case descriptionHere, we report and compare two cases of unclassifiable connective tissue disease who developed a Kikuchi-like lymphadenitis and sepsis-like clinical syndrome, including disseminated intravascular coagulation, which proved rapidly fatal.Discussion and evaluationIn our review of the literature, we found 55 cases of Kikuchi-Fujimoto disease occurring in the context of definite connective tissue disease, 50 of which were associated with SLE. Of the 55 cases, 22 (40%) had simultaneous onset with, 19 (35%) predated the onset of and 14 (25%) developed after the associated connective tissue disease. Life-threatening autoimmune sequelae were reported in 8 cases, 2 of which were fatal. The aetiology of the association remains unknown.ConclusionKikuchi-Fujimoto disease is a histopathological diagnosis, and although the classical form appears to represent a distinct entity, it is unclear whether it is always the same entity, regardless of the context in which it occurs, or whether it represents a histological pattern with a variety of possible causes. In any case, the possibility of auto-immune sequelae in patients with known autoimmune disease should always be considered if these patients present with a sepsis-like clinical syndrome and no infective source is identified.

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Kikuchi–Fujimoto disease developed into autoimmune disease: a report of two cases

Cited by 10 publications

References 19 publications

Kikuchi-Fujimoto disease associated with primary Sjögren’s syndrome – literature review based on a case report

Kikuchi-Fujimoto disease associated with primary Sjögren’s syndrome – literature review based on a case report

Doença de Kikuchi-Fujimoto: apresentação de um caso

Fatal Kikuchi-like lymphadenitis associated with connective tissue disease: a report of two cases and review of the literature

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