Self-limiting COVID-19-associated Kikuchi-Fujimoto disease with heart involvement: case-based review.

BACKGROUND: The association between COVID-19 infection and the development of autoimmune diseases is currently unknown, but there are already reports presenting induction of different autoantibodies by SARS-CoV-2 infection. Kikuchi-Fuimoto disease (KFD) as a form of histiocytic necrotizing lymphadenitis of unknown origin. OBJECTIVE: Here we present a rare case of KFD with heart involvement after COVID-19 infection. To our best knowledge only a few cases of COVID-19-associated KFD were published so far. Based on presented case, we summarize the clinical course of KFD and its association with autoimmune diseases, as well we discuss the potential causes of perimyocarditis in this case. METHODS: We reviewed the literature regarding cases of "Kikuchi-Fujimoto disease (KFD)" and "COVID-19" and then "KFD" and "heart" or "myocarditis" by searching medical journal databases written in English in PubMed and Google Scholar. RESULTS: Only two cases of KFD after COVID infection have been described so far. CONCLUSION: SARS-CoV-2 infection can also be a new, potential causative agent of developing KFD.

Kikuchi Disease in Children: Case Report and Review of the Literature.

BACKGROUND: Kikuchi disease (KD) is a rare and generally benign condition of uncertain etiology that presents with nonspecific symptoms including fever and cervical lymphadenopathy. Clinical presentations can vary. Here, we present an atypical case of KD in a 10-year-old girl, as well as an updated literature review of the clinical presentation, laboratory features and management of KD in children. METHODS: Studies (published up until February 2020) were identified through searches of PubMed using the following search items: Kikuchi-Fujimoto disease or histiocytic necrotizing lymphadenitis or Kikuchi disease. Our primary search resulted in 1117 publications. A total of 34 publications with a total of 670 patients were included in the final analysis. RESULTS: All children present with lymphadenopathy. Almost all (96.3%) have cervical lymphadenopathy. Fever is recorded in the majority of children (77.1%). Analysis of laboratory features found that the majority of children have leukopenia (56.0%) and a raised erythrocyte sedimentation rate (56.0%). Over 30% have a raised C-reactive protein and anemia. Other features such as leukocytosis, thrombocytopenia and antinuclear antibodies positivity are less common. KD is mostly self-limiting, but steroids, hydroxychloroquine and intravenous immunoglobulin are used in protracted courses. Their efficacy has yet to be established in clinical trials. CONCLUSIONS: The presentation of KD is variable, and there is no specific set of symptoms or laboratory features that reliably establishes the diagnosis. Thus, histopathology is crucial. Definitive evaluation and establishment of effective treatments will require future prospective research studies for a more comprehensive description of the clinical course and effects of treatment. Given the rarity of the disease, this will have to be performed in collaborative consortia.

Baffled with fever and lymphadenopathy: neither infection nor malignancy, think Kikuchi disease.

Kikuchi disease is a rare, benign condition of unknown aetiology, which usually involves young women and is characterised by cervical lymphadenopathy and fever. Herein, we are reporting a case of a young Asian woman, who presented with fever and lymphadenopathy raising possibility of either infection or malignancy but after appropriate clinical investigations including lymph node biopsy, it turned out to be Kikuchi disease. She made an uneventful complete recovery with only symptomatic treatment.

Distribution of lymphadenopathy in patients with Kikuchi disease.

BACKGROUND: Cervical lymphadenopathy is among the cardinal manifestations of Kikuchi disease (KD). The incidences and locations of extra-cervical lymph nodes (LNs) involvement in KD have not been comprehensively reported. METHODS: From 2003 to 2016, 60 patients with pathologically confirmed KD and with computed tomography and/or whole-body inflammation scans at diagnosis were retrospectively identified. The locations, sizes and characteristics of all affected LNs were analyzed by extensive review of the image studies. The clinical and laboratory parameters were abstracted from medical records and the associations with extra-cervical LNs involvement were identified. RESULTS: Female accounted for 35 (58.3%) patients and the median age of all patients was 21.3 years (ranges, 3-64 years). Of 59 patients with evaluable neck images, 42 (71.2%) and 16 (27.1%) patients presented with unilateral and bilateral nodal involvement, respectively, with the most common locations at level II, III and IV by Som's classification. The largest LNs appeared most commonly in level II. The incidences of extra-cervical lymphadenopathy in abdomen, pelvis, inguina, axilla and mediastinum with available images were respectively 52.9% (9/17), 47.1% (8/17), 41.2% (7/17), 30.6% (11/36) and 14.3% (8/56). When compared to cases with solitary cervical lymphadenopathy, the cases with extra-cervical lymphadenopathy had significantly greater incidences of bilateral cervical lymphadenopathy (P = .0379) and leukopenia (P = .0173). CONCLUSION: Unilateral cervical lymphadenopathy was the most frequent form of LNs involvement of KD. Extra-cervical lymphadenopathy was not uncommon and was associated with the appearance of bilateral distribution of cervical LNs and leukopenia.

A familial case of Kikuchi-Fujimoto disease in dizygotic twins.

BACKGROUND: Kikuchi-Fujimoto disease (KFD) or necrotizing histiocytic lymphadenitis, was described separately by both Kikuchi and Fujimoto in Japan in the early 1970's. Despite its rarity in the pediatric population, it is an important differential in persistent lymphadenopathy. Familial cases of KFD in the literature are rare. Here we describe the first reported case of KFD in non-identical twin sisters. CASE PRESENTATION: Twin 1 presented with a 3-week history of worsening right-sided cervical lymphadenopathy, daily fevers, significant lethargy, weight loss and arthralgia of her knees and ankles at the age of 12 years in 2015. She had had an unremarkable medical history. A biopsy of her lymph nodes showed histiocytic necrosis consistent with KFD. Twin 2 presented with a three-week history of lethargy, fatigue, weight loss and left-sided posterior cervical chain lymphadenopathy at 16 years of age in 2018. She had a history of frequently relapsing nephrotic syndrome and celiac disease. A biopsy of her lymph nodes was undertaken and showed histiocytic necrosis consistent with KFD. CONCLUSIONS: KFD is a rare but self-limiting pathological process of necrotizing histiocytic lymphadenitis. Although further research is needed, there is an increasing amount of evidence which suggests a multifactorial pathological basis of disease. The two cases we document here are the first reported cases of familial KFD in dizygotic HLA-identical twins which reinforces the likely HLA-linkage in the etiology of KFD.

Cervical lymphadenopathy in children: a diagnostic tree analysis model based on ultrasonographic and clinical findings.

OBJECTIVES: To establish a diagnostic tree analysis (DTA) model based on ultrasonography (US) findings and clinical characteristics for differential diagnosis of common causes of cervical lymphadenopathy in children. METHODS: A total of 242 patients (131 boys, 111 girls; mean age, 11.2 ± 0.3 years; range, 1 month-18 years) with pathologically confirmed Kikuchi disease (n = 127), reactive hyperplasia (n = 64), lymphoma (n = 24), or suppurative lymphadenitis (n = 27) who underwent neck US were included. US images were retrospectively reviewed to assess lymph node (LN) characteristics, and clinical information was collected from patient records. DTA models were created using a classification and regression tree algorithm on the basis of US imaging and clinical findings. The patients were randomly divided into training (70%, 170/242) and validation (30%, 72/242) datasets to assess the diagnostic performance of the DTA models. RESULTS: In the DTA model based on all predictors, perinodal fat hyperechogenicity, LN echogenicity, and short diameter of the largest LN were significant predictors for differential diagnosis of cervical lymphadenopathy (overall accuracy, 85.3% and 83.3% in the training and validation datasets). In the model based on categorical parameters alone, perinodal fat hyperechogenicity, LN echogenicity, and loss of fatty hilum were significant predictors (overall accuracy, 84.7% and 86.1% in the training and validation datasets). CONCLUSIONS: Perinodal fat hyperechogenicity, heterogeneous echotexture, short diameter of the largest LN, and loss of fatty hilum were significant US findings in the DTA for differential diagnosis of cervical lymphadenopathy in children. KEY POINTS: • Diagnostic tree analysis model based on ultrasonography and clinical findings would be helpful in differential diagnosis of pediatric cervical lymphadenopathy. • Significant predictors were perinodal fat hyperechogenicity, heterogeneous echotexture, short diameter of the largest LN, and loss of fatty hilum.

Kikuchi-Fujimoto Disease in Children: An Important Diagnostic Consideration for Cervical Lymphadenitis.

Kikuchi-Fujimoto disease (KFD), or histiocytic necrotizing lymphadenitis, is a rare, benign, and self-limited disease that causes lymphadenopathy and has a characteristic histological appearance. The etiology of this disease is unknown, but a possible infectious trigger has been hypothesized. In the adult population this disease is more common in females; however, in the pediatric population it is more common in males. Descriptions in the pediatric literature are lacking, particularly in the United States. The authors report three cases of pediatric KFD that presented at the same institution in a 9-month time period. All three patients were male and of non-Asian descent who were diagnosed with KFD by histopathologic specimen after presenting with unilateral cervical lymphadenitis. Each patient had additional laboratory evidence of a possible bacterial infection at the time of diagnosis. These three cases highlight the importance of considering KFD early when a pediatric patient presents with unilateral cervical lymphadenitis. The authors discuss the epidemiology, etiology, clinical manifestations, diagnostic approaches, and treatment of KFD. [Pediatr Ann. 2019;48(10):e406-e411.].

Kikuchi's disease (histiocytic necrotizing lymphadenitis): A rare presentation with acute kidney injury, peripheral neuropathy, and aseptic meningitis with cutaneous involvement.

Kikuchi's disease (KD) also known as histiocytic necrotizing lymphadenitis is rare, idiopathic, generally self-limited cause of lymphadenitis. We present a case of twenty year young female who presented in critically ill state with fever, cervical and axillary lymphadenopathy, rash, vomiting and altered sensorium and found to have neurological, hepatic, renal and dermatological involvement. Kikuchi's disease should be considered in differential diagnosis of fever and lymphadenopathy and though benign can sometimes present with multi-organ involvement. It is because of rarity of this disease with unusual complications, present case is reported.

[Kikuchi-Fujimoto disease: about a case]. / Maladie de Kikuchi-Fujimoto: à propos d'un cas.

Kikuchi-Fujimoto disease or histiocytic necrotizing lymphadenitis is a rare cause of benign cervical adenopathies. It is an anatomoclinic entity of unknown cause. Diagnosis is based on histologic examination of the lymph nodes. Patient's evolution is generally favorable with spontaneous healing after a few weeks. We here report the case of a 9-year old girl presenting with cervical lymphadenopathy associated with fever. Cervical lymph node biopsy showed Kikuchi-Fujimoto disease. Patient's evolution was marked by regression of adenopathies without receiving any treatment.

The incidence and clinical characteristics by gender differences in patients with Kikuchi-Fujimoto disease.

Kikuchi-Fujimoto disease (KFD) is a rare, self-limiting disorder that typically affects the cervical lymph nodes (LNs). Although initially described in young women, KFD also occurs in men. There are no reports on the clinical manifestations and characteristics of male KFD patients. Therefore, this study was conducted to assess the incidence of KFD among males, as well as the most frequent clinical characteristics of these patients. A retrospective, cross-sectional study was performed at a tertiary hospital of patients pathologically confirmed as having KFD from LN biopsy specimens. Clinical and laboratory data, and treatment outcomes of the enrolled patients, were analyzed by gender. A total of 254 patients diagnosed with KFD were enrolled. There were 189 females and 65 males (2.9:1). The mean age was 32.6 ±â€Š11.3 years. Compared to the female patients, the males had more frequent manifestations of fever (48% vs 67%, P = 0.008), headache (9% vs 20%, P = 0.013), bilateral lymphadenopathy (31% vs 46%, P = 0.029), thrombocytopenia (14% vs 29%, P = 0.014), elevated C-reactive protein (CRP) (35% vs 78.4%, P < 0.001), elevated liver enzymes (15% vs 41%, P < 0.001), and elevated lactate dehydrogenase (LDH) (61% vs 80%, P = 0.021). Male patients had fewer autoimmune features (9% vs 2%, P = 0.043) and fewer positive antinuclear antibodies (32% vs 10%, P = 0.006). In this study, 25.6% of the enrolled patients were male, with a 2.9:1 female-to-male sex ratio. Male patients showed a distinctive profile characterized by a higher frequency of fever, headache, bilateral lymphadenopathy, and thrombocytopenia, as well as elevated liver enzymes, CRP, and LDH.

Clinical association between Kikuchi׳s disease and systemic lupus erythematosus: A systematic literature review.

OBJECTIVE: To perform a systematic review of all cases of the association between Kikuchi's disease (KD) and systemic lupus erythematosus (SLE), and to ascertain the clinical and laboratory characteristics of this association (KD-SLE). METHODS: We conducted a systematic search of the scientific literature until 31 January 2016. For study purposes, only patients aged >14 years, with histologically proven KD, definite SLE and adequate clinical data were included. To compare KD-SLE against isolated KD and SLE, we selected 2 large series of patients with KD and 4 series of SLE patients. RESULTS: The search found 158 adults with proven KD-SLE. Of these, 113 with sufficient clinical information were included; 86 were women (female:male ratio = 5.0); mean age at diagnosis was 34 years (range: 14-56 years); and an ethnic distribution of 50.5% Asian, 34% Caucasian, and 15% other. KD-SLE patients differed significantly from patients with isolated KD, presenting with a higher frequency of high fever (90%), severe KD (88%), and extranodal manifestations. When compared to patients with SLE, those with KD-SLE presented with a higher frequency of fever and systemic symptoms and a lower frequency of lupus nephritis (22%). SLE had been diagnosed before KD in 18% of cases, simultaneously in 51%, and after KD in 31%. No significant differences were found in terms of time of diagnosis. CONCLUSIONS: While KD-SLE patients share many clinical and laboratory manifestations with SLE, they differ in a lower frequency of lupus nephritis. The relative time of diagnosis of each disease did not affect the clinical expression of KD-SLE.

Kikuchi-Fujimoto Disease in aYoung African American Male: Are we Seeing a Paradigm Shift in Disease Epidemiology? A Case Report.

Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis (HNL), is a rare, self-limiting disease most commonly reported in young Asian women worldwide. We present the case of a 27-year-old African American male who had three weeks of high-grade fevers, night sweats, a 10-pound weight loss, and tender unilateral posterior cervical lymphadenopathy. A complete workup of infectious, rheumatologic, and neoplastic diseases was pursued. Lymph node biopsies revealed histiocyte proliferation with areas of necrosis. These findings were diagnostic of KFD. While KFD has been reported most commonly in young Asian women, in the US, this disease must be considered in both males and females and in diverse ethnicities.

Kikuchi-Fujimoto disease: pediatric case report.

The Kikuchi-Fujimoto disease is a rare disease that occurs mostly in young adults, although some cases have been reported in children. It is usually characterized by fever and cervical lymphadenopathy. The etiology of the disease remains unknown. Its course is usually benign and self-limited. It has special histopathological features that allow the differential diagnosis with other entities, which from a clinical point of view can be very complicated. We report a 9 years 11 months old girl with lymphadenopathy and fever with five months evolution, which is the longest evolution among the cases reviewed by the authors in world literature. Given that the presentation of this disease in children is very rare, we estimate that the knowledge of this disease is relevant and pediatricians must consider it in the differential diagnosis of fever of unknown origin in children.

La enfermedad de Kikuchi-Fujimoto es una entidad rara que se presenta principalmente en adultos jóvenes, aunque se han descrito algunos casos en la edad pediátrica. Se caracteriza por adenopatías generalmente cervicales y fiebre. La etiología de la enfermedad permanece desconocida. Su curso es generalmente benigno y autolimitado. Tiene características histopatológicas especiales que permiten el diagnóstico diferencial con otras entidades, lo que desde el punto de vista clínico puede ser muy complicado. Presentamos el caso de una niña de nueve años y 11 meses que cursó con adenopatías y fiebre de cinco meses de evolución. Este tiempo es el más prolongado entre los casos revisados por los autores en la literatura mundial (búsqueda en PubMed y SciELO por “Kikuchi-Fujimoto Disease”, “histiocytic necrotizing lymphadenitis”). Dado que la presentación de esta enfermedad en la edad pediátrica es muy rara, estimamos pertinente su conocimiento y consideración en el diagnóstico diferencial de la fiebre de origen desconocido en niños.

Aportación de la 18F-FDG PET-TAC en el diagnóstico y seguimiento de la linfadenitis histiocítica necrotizante de Kikuchi-Fujimoto / Value of 18F-FDG PET-CT in diagnosis and following of histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto's disease)

No disponible

Kikuchi-Fujimoto Disease: a Case Report.

Kikuchi-Fujimoto disease or histiocytic necrotizing lymphadenitis is an idiopathic, self-limiting disorder and predominantly affects young women. We report a 35-year-old female who presented with soft to firm cervical lymphadenopathy and neck pain. She had multiple enlarged cervical nodes. Examination of other systems was normal. Lymph node biopsy was performed, and the histological features, and immunohistochemistry confirmed the diagnosis. The Patient was treated with non-steroidal anti-inflammatory drugs and low-dose prednisolone. A significant decrease in the size of lymph node and relief of neck pain occurred. During four years of follow-up, the patient developed no malignant disease or systemic and autoimmune diseases such as systemic lupus erythematosus. Kikuchi-Fujimoto disease is rare, clinicians should be aware of this condition as early diagnosis of the disease will lessen concerns of the patient's family.