Kikuchi Fujimoto Lymphadenitis: A Rare Association With COVID-19 Vaccination.

Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is an uncommon cause of protracted cervical lymphadenopathy in both children and adults. Although the majority of cases have been documented in Asia, this disease has been characterized globally since it was first identified in Japan in 1972. KFD's etiology is not entirely understood, although various theories have been postulated. Usually benign, Kikuchi-Fujimoto disease resolves within six months. KFD must be distinguished from other causes of chronic lymphadenopathy, such as lymphoma, inflammatory illnesses, autoimmune conditions, viral lymphadenopathy, and also from tuberculosis, especially in India, where it is still endemic. Here, we present the case report of a healthcare worker with a known case of hypothyroidism and autoimmune skin disorder who presented with prolonged fever, joint pain, and generalized lymphadenopathy post-COVID vaccination and was diagnosed as having KFD on biopsy, which can be associated with a very unusual presentation of this disease.

Clinical scoring approach for detection of histiocytic necrotising lymphadenitis in adults.

BACKGROUND: Histiocytic necrotising lymphadenitis (HNL) is rare and can be easily ignored. AIMS: To summarise the characteristics of HNL and find a simple scoring approach to detect HNL in adult patients. METHODS: Adult patients with lymphadenopathy diagnosed by lymph node biopsy were enrolled. Chi-squared test and t-test were used to determine the significant variables. The cut-off values and scores assigned to each factor were performed by receiver operating characteristic (ROC) curves and coefficients in the logistic regression respectively. The performance of the scoring system was evaluated by ROC curves. RESULTS: There were 32 HNL cases and 1162 other cases in the present study. These features, including age, the frequency of presentations of fever, cervical and painful lymph nodes, decrease of white blood cells (WBC), ratio of neutrophil to WBC (N ratio) and elevated lactate dehydrogenase (LDH), were different between patients with HNL and other diseases. Based on the multivariate analysis, the scoring approach was defined as follows: score = 3 (fever) + 2 (cervical lymphadenopathy) + 2 (decreased WBC) + 1 (decreased N ratio) + 2 (elevated LDH). The cut-off was score 4. This approach performed will detect HNL with an area under the curve of 0.889. CONCLUSION: The present study suggests that the novel scoring approach we put forward might be useful to detect HNL in adult patients though further studies are needed.

Kikuchi-Fujimoto disease: a rare cause of histiocytic necrotising lymphadenitis in young ethnic women.

Kikuchi-Fujimoto is a rare but self-limiting disease usually affecting young ethnic females. The main clinical presentation is fever and cervical lymphadenopathy. Here we describe the cases of two patients with cervical lymphadenopathy, fever and fatigue. In addition, patients can present with neutropenia; hence, early diagnosis is crucial to exclude a malignant cause.

Methotrexate-associated lymphoproliferative disorder with histopathological features of histiocytic necrotizing lymphadenitis.

An 84-year-old Japanese woman suffering from rheumatoid arthritis (RA), who had been treated with methotrexate (MTX) for 15 years, was admitted to our hospital for generalised lymphadenopathy, thrombocytopenia, anaemia, elevated aminotransferases, and elevated CRP levels. Pathological findings of cervical lymph node biopsy were compatible with histiocytic necrotising lymphadenitis (HNL). Small lymphocytes positive for Epstein-Barr virus (EBV)-encoded small RNA were detected in the tissue. We suspected a MTX-associated lymphoproliferative disorder (MTX-LPD), withdrew MTX and administered leucovorin (folic acid). The patient's symptoms gradually resolved following discontinuation of MTX. We considered that this patient developed HNL as an MTX-LPD when EBV was reactivated. This is the first case of HNL associated with MTX treatment for RA, which we report here along with clinical course.

Intensive care management of a rare case of pyrexia of unknown origin (Kikuchi-Fujimoto disease).

Kikuchi-Fujimoto disease is a rare, benign cause of necrotising lymphadenitis often presenting with fever. We describe a case of a 17-year-old boy with non-verbal autism presenting to our intensive care unit with prolonged fever of unknown cause. This case highlights the role of the intensive care unit in cases of diagnostic dilemma. The critical care community should be aware of Kikuchi-Fujimoto disease as although it is usually benign, it can rarely lead to acute airway compromise.

Kikuchi-Fujimoto Disease and Prognostic Implications.

Kikuchi-Fujimoto disease (KFD) is a rare cause of lymphadenitis seen mostly in Asian populations (Kikuchi in Nippon Ketsueki Gakkai Zasshi 35:379-80, 1972). First described in 1972, KFD is a benign and self-limiting disease characterised by lymphadenopathy, mild fever, fatigue, and leukopenia (Fujimoto in Naika 30:920-7, 1972; Lin et al. in Otolaryngol Head Neck Surg 128(5): 650-3, 2003). We present a case of a 38-year-old woman with a 6-week history of cervical lymphadenopathy. Her ultrasound scan and fine needle aspiration cytology results were inconclusive. Excisional biopsy of the lymph node confirmed presence of KFD. The aetiology of KFD is unknown; however, there is strong association with systemic lupus erythematosus (SLE). Kucukardali reported 9% of European KFD patients and 28% of East Asian patients had concomitant SLE (Kucukardali et al. in Clin Rheumatol 26(1):50-4, 2007). We describe a follow-up algorithm for newly diagnosed KFD cases, based on the current literature. KFD is a rare cause of cervical lymphadenopathy. It is associated with increased risk of developing SLE, therefore early diagnosis and long-term follow-up are recommended.

Immunopathology of Kikuchi-Fujimoto disease: A reappraisal using novel immunohistochemistry markers.

AIMS: Kikuchi-Fujimoto disease (KFD) is a self-limited disease characterised by destruction of the lymph node parenchyma. Few studies have assessed the immunohistological features of KFD, and most employed limited antibody panels that lacked many of the novel immunohistochemistry markers currently available. METHODS AND RESULTS: We used immunohistochemistry to reappraise the microanatomical distribution of plasmacytoid dendritic cells (pDCs), follicular helper T cells and cytotoxic T cells, B cells, follicular dendritic cell (FDC) meshworks, and histiocytes in lymph nodes involved by KFD. The study group consisted of 138 KFD patients (89 women; 64.5%) with a median age of 27 years (range, 3-50 years). Cervical lymph nodes were most commonly involved, in 108 (78.3%) patients. The numbers of pDCs were increased, predominantly around and within apoptotic areas and the paracortex, and tapering off within xanthomatous areas. pDCs formed sizeable tight clusters, most notably around apoptotic/necrotic areas. T cells consisted mostly of CD8-positive cells with predominant expression of T-cell receptor-ß. There were notable increases in the numbers of CD8-positive T cells within lymphoid follicles, and their numbers correlated with alterations in FDC meshworks (P < 0.001). The number of follicular helper T cells was decreased within distorted FDC meshworks. CD21 highlighted frequent distortion of FDC meshworks, even in lymph node tissue that was distant from apoptotic/necrotic areas. Distorted FDC meshworks spanned all morphological patterns, and FDC meshwork characteristics (intact; distorted; remnant/nearly absent) correlated with morphological patterns (P < 0.01). CONCLUSIONS: The immunohistological landscape of KFD is complex and characterised by increased numbers of pDCs that frequently cluster around apoptotic/necrotic foci, increased numbers of cytotoxic T cells, and substantial distortion of FDC meshworks.

Kikuchi-Fujimoto disease in children.

AIM: Kikuchi-Fujimoto disease (KFD) is an important cause of lymphadenitis in children. The primary aim of this study was to investigate the clinical characteristics of children with KFD and to assess the recurrence of this disease. METHODS: This is a retrospective study of patients younger than 18 years old, who were diagnosed with KFD from January 2000 to September 2017 at KK Women's and Children's Hospital. Records of children with a histological diagnosis of KFD from a lymph node biopsy were obtained from the Department of Pathology. Case notes and electronic medical records of the patients were reviewed. Data collected included patient characteristics, symptoms, clinical and laboratory findings, treatment and follow-up. RESULTS: A total of 98 patients were identified. There were 52 boys and 46 girls with a median age of 11.2 years old. Recurrence occurred in 12 (12.2%) patients. One patient developed systemic lupus erythematosus 10 years after diagnosis of KFD. Recurrent cases were more likely to be managed as an inpatient and have fever at presentation of their first episode of KFD. CONCLUSION: In our study, KFD in children had a higher prevalence among boys, and had a recurrence rate of 12.2%, with 1% of patients developing systemic lupus erythematosus. We recommend that patients be followed up for recurrence and advised to monitor for symptoms of recurrence.

Kikuchi-Fujimoto disease triggered by Salmonella enteritidis in a child with concurrent auto-immune thyroiditis and papilloedema.

Kikuchi-Fujimoto disease (KFD) is a histiocytic necrotising lymphadenitis characterised by painful cervical lymphadenopathy, fever, malaise and weight loss. Infections, auto-immune pathogenesis and a genetic association have been implicated. A 12-year-old boy presented with a 1-month history of fever, abdominal pain, constipation and weight loss, and a painful lymph node was detected in the right axilla. Chest CT demonstrated multiple lymph nodes, especially in the left mediastinum. Salmonella enteritidis group D was detected in a blood culture and he was treated with ceftriaxone, followed by meropenem. An axillary lymph node biopsy demonstrated necrotising histiocytic lymphadenitis and KFD was diagnosed. He was discharged 35 days after admission. He was re-admitted 3 weeks later with recurrence of symptoms and headache and was found to have papilloedema of the left eye and auto-immune thyroiditis. Intravenous immunoglobulin (IVIG) 400 mg/kg/day was administered for 5 days. The fever and papilloedema slowly resolved and, subsequently, the thyroiditis, and he has remained well on follow-up. This is the first report of an association of S. enteritidis infection and papilloedema with KFD. IVIG may be required in prolonged or recurrent cases and in those with an auto-immune association.

Kikuchi-Fujimoto Disease: A Rare Presentation with Localized Iliac Lymphadenitis.

Kikuchi-Fujimoto disease (KFD) is a benign, self-limiting disease characterized by histiocytic necrotising lymphadenitis. Though several viral agents or an autoimmune etiology has been proposed as causative, the exact cause remains unknown. It has a female predilection and most commonly seen among young Asian people. Patients usually present with a febrile illness and the presence of lymphadenopathy may provide a clue to diagnosis. The most common site of lymphadenopathy is cervical lymph nodes while intra-abdominal involvement is uncommon. Cases of KFD presenting with intra-abdominal lymphadenopathy have been reported to occur with equal frequency in both sexes. Abdominal tuberculosis, non-Hodgkin's lymphoma, and systemic lupus erythematosus are close differential diagnoses for this type of presentation. Treatment is mostly supportive as the disease usually resolves spontaneously; steroids are only required in severe cases. We report a 32-year-old male patient of intra-abdominal lymphadenitis that presented as fever of unknown origin (FUO) and diagnosed by excisional biopsy as a case of KFD.

Una relación potencialmente peligrosa: lupus eritematoso sistémico y enfermedad de Kikuchi - Fujimoto / A dangerous relation: Systemic lupus erythematosus disease and Kikuchi Fujimoto disease

Resumen La enfermedad de Kikuchi-Fujimoto (EKF) es conocida como una linfadenitis necrotizante histiocítica, tiene una presentación benigna y autolimitada, afecta principalmente a mujeres jóvenes y generalmente resuelve sin tratamiento en los primeros seis meses de los síntomas. Presentamos el caso de una mujer de 48 años quien consultó por un cuadro de dolor abdominal, mialgias y adenopatía cervical al examen físico. Se le encontró en los exámenes paraclínicos anticuerpos antinucleares 1:640, anti DNA de doble cadena asociado a consumo de complemento y anticuerpos anticardiolipina positivos. Se realizó biopsia de ganglio cervical que mostró necrosis cortical y población de histiocitos y linfocitos. Al iniciar el manejo con esteroides y cloroquina se comprobó mejoría franca y posteriormente la paciente fue dada de alta, continuando controles de manera ambulatoria por los servicios de reumatología y medicina interna.

Abstract Kikuchi disease, also known as histiocytic necrotizing lymphadenitis, isa benign and self-limited condition that mainly affects young women andresolve without treatment within six months of symptoms. It was first describedin Japan in 1972 and is more common in Asian population. We report the case of a woman of 48 years who consults for abdominal pain, myalgia, and cervical lymphadenopathy on physical examination. He found himself in the presence of antinuclear antibodies paraclinical 1: 640 positive DNA double chain associated with complement consumption and positive cardiolipin antibodies We perform cervical node biopsy that showed the presence of cortical necrosis and histiocytes and lymphocytes population. When you start handling steroid and chloroquine, a high frank and subsequent improvement was observed, continuing control rheumatology and internal medicine.

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

INTRODUCTION: Kikuchi-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 DESCRIPTION: Here, 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 EVALUATION: In 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. CONCLUSION: Kikuchi-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.

Kikuchi-fujimoto disease from eastern India.

Kikuchi's disease, a rare disorder which usually presents with fever painful lymphadenopathy, rash and arthritis, all of which are close mimickers of infective and immunological disorders. It is essentially a histopathological diagnosis and tests to rule out other connective tissue disorders or infective etiology must be undertaken. We present a series of two cases of kikuchi-fujimoto's disease presenting primarily with lymphadenopathy and fever in all cases. The first is a case of generalized lymphadenopathy and the second case of kikuchi's disease with SLE, a rare association. Lymph node excision biopsy and histopathology documented Kikuchi Fujimoto disease in above cases. All the cases improved on follow up and had no residual stigmata.