Evaluation of macrophage activation syndrome in hospitalised patients with Kikuchi-Fujimoto disease based on the 2016 EULAR/ACR/PRINTO classification criteria

Ahn, Sung Soo; Lee, Byeori; Kim, Dam; Jung, Seung Min; Lee, Sang Won; Park, Min Chan; Park, Yong Bum; Hwang, Yong Gil; Song, Jason Jungsik

doi:10.1371/journal.pone.0219970

Cited by 12 publications

(20 citation statements)

References 37 publications

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“…In addition, the proportion of patients with KFD requiring glucocorticoid treatment was significantly higher in patients with MAS than in patients without MAS. 6 Despite previously stating that histopathological examination is the main stay in the diagnosis of KFD, there are other histological differential diagnosis such as lymphoid malignancies particularly non-Hodgkin's lymphomas, lymphadenopathy due to autoimmune disorders, primarily systemic lupus erythematosus (SLE) and infectious etiologies, such as Epstein-Barr virus, herpes simplex virus, Bartonella henselae and toxoplasmosis. 7 Hence experience and consultation with a senior pathologist is a must before providing a report of KFD.…”

Section: Discussionmentioning

confidence: 99%

Misled by the Xpert-Kikuchi’s disease masquerading as tuberculosis

Krishnakumar

Elayaraja

Ravichandran

et al. 2020

Int J Contemp Pediatr

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Kikuchi-Fujimoto disease (KFD), also called histiocytic-necrotizing lymphadenitis is a rare, idiopathic and self-limiting cause of lymphadenitis. Clinical presentation of KFD closely resembles nodal tuberculosis (TB). Here we present a case of an adolescent female whose diagnosis of KFD was made despite being misled in the course. A 15-year-old female, previously treated for tuberculous lymphadenitis was brought with complaints of fever for 1 month. Various possibilities considered were TB reactivation, autoimmune disorders, lymphoma. Lymph node biopsy for gene Xpert showed TB bacilli detected but low with no RIF resistance. Despite anti tuberculous treatment initiation, there was no improvement in the clinical condition. Histopathology of cervical node showed features of Kikuchi lymphadenitis. Literature search revealed that gene Xpert can detect the intact DNA of Mycobacterium tuberculosis even years after the previous treatment. On stopping anti tuberculosis therapy (ATT) and starting IV steroid, she started improving dramatically. KFD should always be kept as a differential diagnosis in any individual with fever and lymphadenopathy. Diagnosis can be misled in any patient based on gene Xpert reports alone which often turns out to be positive if there is past history of TB as gene Xpert detects DNA from non-intact cells suggesting that dead bacilli contribute to the false positivity.

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Section: Discussionmentioning

confidence: 99%

Misled by the Xpert-Kikuchi’s disease masquerading as tuberculosis

Krishnakumar

Elayaraja

Ravichandran

et al. 2020

Int J Contemp Pediatr

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“…The fever is periodic, irregular, and oscillates between 38.6°C and 40.5°C. 2,7,11 Chills, nausea and vomiting, rash, headache and joint pain, weight loss, night sweats, splenomegaly, and parotid gland enlargement are other symptoms often found in KFD. Cutaneous symptoms appear in up to 40% of KFD cases, most often affecting the face, trunk, and upper limbs.…”

Section: Symptomsmentioning

confidence: 99%

“…Both HLH and KFD are associated with abnormal activation of histiocytes. 2,4,11 The duration of KFD is varied. Symptoms usually resolve within 6 months, however in individual cases, they can persist for up to a year.…”

Section: Symptomsmentioning

confidence: 99%

“…7 Autoimmune diseases (e.g., SLE, Kawasaki disease, sarcoidosis), congenital disorders (e.g., lingual-thyroid cyst, dermoid cyst, and pharyngeal clefts) and infectious diseases should be include in the differential diagnosis of KFD. 2,4,6,11 Epstein-Barr virus, cytomegalovirus, HIV, rhino-, adenovirus and rubella virus infection were mentioned among the viral causes of lymphadenopathy. 2 Staphylococcus aureus and Streptococcus pyogenes are the most common causes of purulent lymphadenitis.…”

Section: Diagnosticsmentioning

confidence: 99%

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Kikuchi-Fujimoto disease: State of the art

2021

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Introduction: Kikuchi-Fujimoto disease (KFD), also called histiocytic necrotizing lymphadenitis, is a rare disease that occurs with swollen lymph nodes and associated fever. This disease occurs in both children and adults. Aim: The aim of our work was to review the literature and to remind family doctors, otolaryngologists, hematologists and rheumatologists about this rare disease that should be included in the differential diagnosis of long-term cervical lymphadenopathy. Material and methods: Current information on Kikuchi-Fujimoto disease was sought and presented based on literature that was published in reputable magazines in the period 2007–2020 in English. We searched for articles in the Medline, PubMed, and Scopus databases. Results and discussion: KFD occurs in both children and adults. This disease is found all over the world, most often in the Asian population. The etiology of Kikuchi-Fujimoto disease is not entirely known, however, two causal theories are suspected, which are discussed in detail in our article. The course of the disease is mild and usually disappears on its own. A biopsy of an involved lymph node presented as the standard for diagnosis. KFD treatment was causal – nonsteroidal anti-inflammatory drugs and/or glucocorticosteroids were used. Conclusions: There are few reports in the literature about Kikuchi-Fujimoto disease. KFD is associated with cervical lymphadenopathy and associated fever. If the diagnosis of the above-mentioned symptoms is not obvious, then in the differential diagnosis rarer diseases, such as KFD, should be included.

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“…SPTCL occasionally shows striking histologic overlap with lupus panniculitis [33] and some patients, including those with wild type HAVCR2, have underlying autoimmune diatheses including SLE [34]. Although there is excellent overall survival, the low SPTCL mortality rate is almost invariably attributable to concomitant hemophagocytic lymphohistiocytosis (HLH), which can occur albeit Dermatology Online Journal || Case Report with a much lower frequency in the settings of SLE, KFD, and SLE/KFD overlap [13,35,36]. Therefore, patients presenting with KFD or LEP should also be monitored for the development of HLH and efforts, including T-cell clonality testing, can be considered in select cases to help exclude SPTCL.…”

Section: A B C D E Fmentioning

confidence: 99%