Clinical features and prognosis of 118 children with histiocytic necrotizing lymphadenitis / 中华儿科杂志

Objective: To explore the clinical features and prognosis of children with histiocytic necrotizing lymphadenitis (HNL). Methods: The clinical data of 118 children with HNL diagnosed and treated in the Department of Rheumatology and Immunology of Children's Hospital, Capital Institute of Pediatrics from January 2014 to December 2021 were retrospectively analyzed. The clinical symptoms, laboratory examination, imaging examination, pathological findings, treatment and follow-up were analyzed. Results: Among the 118 patients, 69 were males and 49 were females. The age of onset was 10.0 (8.0, 12.0) years, ranging from 1.5 to 16.0 years. All the children had fever lymph node enlargement, blood system involvement in 74 cases (62.7%), skin injury in 39 cases (33.1%). The main manifestations of laboratory examination were increased erythrocyte sedimentation rate in 90 cases (76.3%), decreased hemoglobin in 58 cases (49.2%), decreased white blood cells in 54 cases (45.8%) and positive antinuclear antibody in 35 cases (29.7%). Ninety-seven cases (82.2%) underwent B-mode ultrasound of lymph nodes, showing nodular lesions with low echo in the neck; 22 cases (18.6%) underwent cervical X-ray and (or) CT; 7 cases (5.9%) underwent cervical magnetic resonance imaging. Lymph node biopsy was performed in all 118 cases, and the pathological results did not support malignant diseases such as lymphoma or Epstein-Barr virus infection, suggesting HNL. Fifty-seven cases (48.3%) recovered without treatment, 61 cases (51.7%) received oral steroid therapy, and 4 cases (3.4%) received indomethacin as anal stopper. The 118 cases were followed up for 4 (2, 6) years, ranging from 1 to 7 years, 87 cases (73.7%) had one onset and did not develop into other rheumatological diseases, and 24 cases (20.3%) had different degrees of recurrence, 7 cases (5.9%) had multiple system injuries, and all of the tested autoantibodies were positive for medium and high titers. All of them developed into other rheumatic immune diseases, among which 5 cases developed into systemic lupus erythematosus and 2 cases developed into Sjogren's syndrome; 7 cases were given oral steroid therapy, including 6 cases plus immunosuppressant and 2 cases receiving methylprednisolone 20 mg/kg shock therapy. Conclusions: The first-onset HNL portion is self-healing, hormone-sensitive and has a good prognosis. For HNL with repeated disease and multiple system injury, antinuclear antibody titer should be monitored during follow-up, and attention should be paid to the possibility of developing into other rheumatological diseases, with poor prognosis.

Adenomegalia dolorosa: doença de Kikuchi entre os diagnósticos diferenciais / Painful adenomegaly: Kikuchi disease among differential diagnoses

RESUMO: A doença de Kikuchi-Fujimoto ou linfadenite histiocítica necrosante, de curso benigno, é prevalente em mulheres jovens, e associada à febre e leucopenia. Estudos recentes têm demostrado que sua etiologia ainda é incerta, sendo uma doença rara, com incidência de 0,5 a 5% de todas as adenopatias analisadas histologicamente. O diag-nóstico diferencial por imunohistoquímica foi decisivo, descartando outras hipóteses diagnósticas como: linfoma, tuberculose ganglionar e lúpus eritematoso sistêmico. Este relato de caso mostra as características da apresentação da doença em uma mulher, caucasiana e todas as etapas da investigação, destacando a importância dos diagnósticos diferenciais em adenopatias dolorosas e as dificuldades quando avaliamos portadores de doenças raras. (AU)

ABSTRACT: Kikuchi-Fujimoto disease or benign necrotizing histiocytic lymphadenitis is prevalent in young women and associated with fever and leukopenia. Recent studies have shown that its etiology is still uncertain, being a rare disease, with an incidence of 0.5 to 5% of all histologically analyzed adenopathies. Differential diagnosis by immunohistochemistry was decisive, ruling out other diagnostic hypotheses such as lymphoma, ganglion tuberculosis, and systemic lupus erythematosus. This case report shows the characteristics of the presentation of the disease in a Caucasian woman and all stages of the investigation, highlighting the importance of differential diagnoses in painful adenopathies and the difficulties when evaluating rare-disease patients. (AU)

Efficacy of medicinal penetration on acupoint as adjunctive therapy on histiocytic necrotizing lymphadenitis of heat-toxin syndrome: a randomized controlled trial / 中国针灸

OBJECTIVE@#To evaluate the clinical efficacy of medicinal penetration on acupoint combined with medication for histiocytic necrotizing lymphadenitis (HNL) of heat-toxin syndrome, and to explore the methods of improving the clinical effect.@*METHODS@#A total of 72 cases with HNL with heat-toxin syndrome were randomly divided into an observation group and a control group, 36 cases in each group. The patients in the control group were treated with oral administration of prednisone tablets for 40 days (first 5 days: 10 mg, three times a day; since then, reduced by 5 mg every 7 days). In the observation group, on the basis of the medication in the control group, the patients were treated with acupoint application and ultrasonic drug penetration therapy, once a day for 14 days. The acupoints of Waiguan (TE 5), Fengchi (GB 20) of affected side and points were selected. The changes of target lymph node swelling, visual analogue score (VAS), axillary temperature and total score of symptoms and signs were evaluated before treatment and 7, 14, 28 and 40 d into treatment; the changes of white blood cell (WBC), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and lactic dehydrogenase (LDH) were evaluated on 14 d and 40 d into treatment; the patients were followed-up for half a year.@*RESULTS@#① Fourteen days into treatment, the clinical cured rate in the observation group was 38.9% (14/36), which was superior to 16.7% (6/36) in the control group (0.05). ④ The recurrence rate in the observation group was 5.6% (2/36), which was similar to 16.7% (6/36) in the control group (>0.05).@*CONCLUSION@#The medicinal penetration on acupoint as adjunctive treatment could effectively relieve the discomfort symptoms of HNL patients with syndrome of heat and toxin, improve the clinical cured rate, and provide the research direction for shortening the course of medication.

Piriformis Pyomyositis in a Patient with Kikuchi-Fujimoto Disease - A Case Report and Literature Review / Piomiosite do piriforme em um paciente com doença de Kikuchi-Fujimoto-relato de caso e revisão da literatura

Abstract Primary pyomyositis is a deep bacterial infection of the skeletal muscle. If left undiagnosed and untreated, the infection spreads, leading to sepsis, septic shock, and even death. The authors report a 23-year-old female presenting with piriformis pyomyositis during a treatment for Kikuchi-Fujimoto disease. Pyomyositis is a rare but potentially severe infection, which can lead to septic shock. The present case shows the need for a high degree of clinical suspicion for patients with compromised immune systems to begin treatment at an early stage. The literature demonstrates that outcomes of the treatment of piriformis pyomyositis are good.

Resumo A piomiosite primária é uma infecção bacteriana profunda do músculo esquelético. Quando não diagnosticada ou tratada, a infecção pode evoluir para sepse, choque séptico e até morte. Os autores relatam o caso de uma paciente do sexo feminino, 23 anos, apresentando piomiosite do músculo piriforme durante o tratamento da doença de Kikuchi-Fujimoto. A piomiosite é uma infecção rara, mas potencialmente grave, que pode levar ao choque séptico. Esse caso mostra a necessidade em se elevar o grau de suspeição clínica em pacientes com comprometimento do sistema imunológico, para que o tratamento seja iniciado em estágio precoce. A literatura mostra que os resultados do tratamento da piomiosite do piriforme são bons.

Kikuchi-Fujimoto Disease in a Filipino Boy: A Case Report

@#<p><strong>OBJECTIVE:</strong> To report a case of a 12-year-old boy with Necrotizing Lymphadenitis (Kikuchi-Fujimoto disease) presenting as lymphadenitis secondary to multiple diagnosis.</p><p> </p><p><strong>METHODS:</strong></p><p><strong>Design :</strong> Case Report</p><p><strong>Setting:</strong> Tertiary Private Hospital</p><p><strong>Patient:</strong> One</p><p> </p><p><strong>RESULTS:</strong> A 12-year-old boy consulted for two-month history of cervical lymphadenopathy with the underlying cause remaining unclear despite multiple consults, diagnosis and medical treatment. Lymphoma was considered and excision biopsy with further investigations confirmed a diagnosis of Kikuchi-Fujimoto disease. Supportive management was given with no recurrence of symptoms noted on 18 months of follow up. </p><p> </p><p><strong>CONCLUSION:</strong>  Kikuchi-Fujimoto disease in this case was a diagnosis of exclusion. Even with a proper history and physical examination, experts in otolaryngology can be misled to manage this case as malignant. Awareness of the disease and appropriate examinations including immunohistochemistry are important for a timely diagnosis and proper intervention.</p><p> </p><p><strong>KEYWORDS: </strong>lympadenitis; Kikuchi-Fujimoto disease; lymphoma; cervical lymph nodes</p>

Acoustic radiation force impulse imaging of biopsy-proven Kikuchi disease: initial experiences for evaluating feasibility in pediatric patients

PURPOSE: This study evaluated the feasibility of acoustic radiation force impulse (ARFI) elastography and characterized the sonographic features of lymph nodes (LNs) with Kikuchi disease in pediatric patients. METHODS: Seventy-six cervical LN biopsies were performed for the diagnosis of cervical lymphadenopathy. ARFI imaging was performed, and the characteristic ultrasound features of the biopsied LNs and the contralateral LNs were analyzed. We also reviewed clinical and conventional ultrasonographic findings. RESULTS: On histology, 56 patients were diagnosed with Kikuchi disease. These LNs were large and elongated, with increased perinodal echogenicity and capsular thickening. In 38 of them, ARFI elastography was performed, and the median shear wave velocity (SWV) of the biopsied LNs with Kikuchi disease (2.19 m/sec; range, 1.45 to 4.57 m/sec) was higher than of the contralateral LNs (1.72 m/sec; range, 0.95 to 2.65 m/sec; P < 0.001). In patients with reactive hyperplasia, the mean SWV of the biopsied LNs (2.00 m/sec; range, 1.49 to 2.26 m/sec) was higher than that of the contralateral LNs (1.55 m/sec; range, 1.21 to 2.32 m/sec; P=0.031). CONCLUSION: The SWV of LNs with Kikuchi disease was significantly higher than that of the contralateral LNs. Morphologically, LNs with Kikuchi disease showed an enlarged, elongated, and oval shape, increased perinodal echogenicity, and capsular thickening. In addition to the conventional ultrasonographic findings, the application of ARFI is feasible even in pediatric patients for the evaluation of cervical lymphadenopathy.

A Case of Kikuchi-Fujimoto Disease Associated with Mycoplasma Pneumoniae Infection / 임상소아혈액종양

Kikuchi-Fujimoto disease (KFD) is a self-limiting disease characterized by subacute necrotizing lymphadenitis. This benign disease is frequently associated with prolonged fever and mostly occurs in young Asian women. KFD is generally diagnosed using a biopsy of affected lymph nodes and spontaneously resolves in several months. Although the causative agent is believed to be infectious, the etiology remains unknown. Some cases of KFD are associated with viral infections, including Epstein-Barr virus, human herpes virus 6, and parvovirus B19 infection. Herein, we report a case of KFD associated with Mycoplasma pneumoniae infection.

Enfermedad de Kikuchi-Fujimoto en una paciente con lupus eritematoso sistémico y linfadenopatía generalizada. Reporte de caso / Kikuchi-Fujimoto disease in a patient with systemic lupus erythematosus and generalised lymphadenopathy. Case report

Resumen La enfermedad de Kikuchi-Fujimoto es un trastorno benigno que afecta principalmente a mujeres jóvenes, se caracteriza por adenopatías de predominio en la región cervical, asociadas a fiebre y leucopenia. Aunque de etiología desconocida, hay evidencia de que una infección viral o una enfermedad autoinmune puede desencadenar la enfermedad. Se reporta un caso infrecuente en Colombia de enfermedad de Kikuchi-Fujimoto en una paciente con lupus eritematoso sistémico.

Abstract Kikuchi-Fujimoto disease is a benign disorder that mainly affects young women, and is characterised by predominantly cervical lymphadenopathy associated with fever and leukopenia. Although of unknown aetiology, there is evidence that a viral infection or autoimmune disease can trigger the disease. An uncommon case in Colombia is presented of Kikuchi-Fujimoto disease in a patient with Systemic Lupus Erythematosus.

Report of a rare case of histiocytic necrotizing lymphadenitis with bilateral pleural effusion diagnosed via cervical lymph node biopsy

ABSTRACT CONTEXT: Histiocytic necrotizing lymphadenitis (HNL) is a rare disorder that is often benign and self-limiting. There have been reports of co-occurrence of HNL with other diseases, including systemic lupus erythematosus, hemophagocytic syndrome and antiphospholipid syndrome. CASE REPORT: Here, we report a case in which a patient experienced unexplained fever, swelling of the cervical lymph node and bilateral pleural effusion and was ultimately diagnosed with HNL based on results from a lymph node biopsy. After treatment with glucocorticoid, the patient regained normal body temperature, the swelling of the lymph nodes disappeared and the pleural effusion was reabsorbed. CONCLUSIONS: The pathogenesis of HNL remains unclear, and pleural effusion is rarely reported in HNL patients. We presented this case to improve diagnostic awareness of this condition among clinicians and help reduce the likelihood of misdiagnosis.

Clinical Observations of Kikuchi-Fujimoto Disease in Children and Adolescents: A Single Center Experience / 이화의대지

OBJECTIVES: Kikuchi-Fujimoto disease (KFD) is characterized by lymphadenopathy and fever, and is usually self-limited. This study analyzed the clinical characteristics of pediatric patients with KFD. METHODS: This retrospective, observational, single-center study was conducted in South Korea from March 2008 to October 2015. KFD was diagnosed based on clinical, radiological or histological findings and excluded when there were any other causes of lymphadenopathy. Medical records were reviewed for clinical and laboratory manifestations. RESULTS: A total of 35 cases were included. The mean patient age was 12.1±2.9 years (range, 5 to 17 years); the male-to-female ratio was 1:0.8. The main clinical manifestations were cervical lymphadenopathy and fever in 34 cases (97%). The mean duration of fever was 12.2±8.3 days (range, 2 to 37 days). We noted enlargement of lymph nodes in the cervical, mesenteric (n=5, 14%), axillary (n=2, 6%), and inguinal (n=1, 3%) regions. Hepatosplenomegaly, loss of appetite, and rash were observed. On laboratory examinations, elevation of ferritin, leukopenia, and positivity for anti-nuclear antibodies were frequently observed. Twelve patients underwent biopsy and 23 cases were diagnosed by radiological findings. The mean duration of hospitalization for all cases was 7.9±2.9 days (range, 3 to 13 days) and steroids were administered in 10 cases. KFD recurrence was observed in 2 cases (5.7%) with the time to relapse of 7 months and 4 years. There were no cases with systemic lupus erythematous or other autoimmune disease. CONCLUSION: KFD should be considered in pediatric patients with lymphadenopathy and prolonged fever. Patients with KFD should be monitored for recurrence and the development of autoimmune disease.

Outcome in Patients with Fever of Unknown Origin whose ¹⁸Fluoro-Deoxyglucose Positron Emission Tomography/Computerized Tomography Finding is Non-Diagnostic

Prognosis has not been known for patients with fever of unknown origin (FUO) whose ¹⁸fluoro-deoxyglucose (¹⁸F-FDG) positron emission tomography/computerized tomography (PET/CT) finding is non-diagnostic. A total of eight patients with FUO that underwent ¹⁸F-FDG PET/CT were retrospectively identified January 2016 - June 2017 in a tertiary hospital in Korea. Of these, two patients were diagnosed with microscopic polyangitis and Kikuchi's disease and one patient was transferred to another hospital. Of five patients whose diagnoses were not confirmed, four patients received non-steroidal anti-inflammatory drug and/or low dose steroid and symptoms disappeared. Our study suggests that outcome of patients with FUO whose ¹⁸F-FDG PET/CT finding is non-diagnostic would be favorable.

Outcome in Patients with Fever of Unknown Origin whose ¹⁸Fluoro-Deoxyglucose Positron Emission Tomography/Computerized Tomography Finding is Non-Diagnostic

Prognosis has not been known for patients with fever of unknown origin (FUO) whose ¹⁸fluoro-deoxyglucose (¹⁸F-FDG) positron emission tomography/computerized tomography (PET/CT) finding is non-diagnostic. A total of eight patients with FUO that underwent ¹⁸F-FDG PET/CT were retrospectively identified January 2016 - June 2017 in a tertiary hospital in Korea. Of these, two patients were diagnosed with microscopic polyangitis and Kikuchi's disease and one patient was transferred to another hospital. Of five patients whose diagnoses were not confirmed, four patients received non-steroidal anti-inflammatory drug and/or low dose steroid and symptoms disappeared. Our study suggests that outcome of patients with FUO whose ¹⁸F-FDG PET/CT finding is non-diagnostic would be favorable.

Bilateral Frosted Branch Angiitis in Kikuchi-Fujimoto Disease

PURPOSE: A case of frosted branch angiitis in Kikuchi-Fujimoto disease is reported. CASE SUMMARY: A 33-year-old male complained of a sudden decrease in visual acuity that developed in both eyes 5 days prior. He suffered from a headache, chills, myalgia, and flank pain 1 week before. The initial best-corrected visual acuity (BCVA) was 0.1 in the right eye and 0.2 in the left eye. On slit lamp examination, no inflammatory finding was observed in the anterior chamber and vitreous body of both eyes. On fundus examination, a diffuse vascular sheathing-like frosted branch was found in the retinal vessels, and retinal hemorrhage was observed. Fluorescein angiography showed staining and leakage of dye along the vascular sheathing. Serological findings were negative, showing no evidence of an autoimmune disease or viral infection. Neck ultrasonography revealed non-tender left cervical lymph node enlargement >1 cm in diameter. Ultrasound-guided fine needle aspiration cytology showed findings compatible with Kikuchi-Fujimoto disease, including necrotic changes and pronounced karyorrhexis, plus histiocyte and lymphocyte infiltration without neutrophils. We started systemic steroid therapy. One month after treatment, the BCVA of both eyes improved to 1.0. CONCLUSIONS: In patients with frosted branch angiitis, systemic disease such as Kikuchi-Fujimoto disease should be considered.

Systemic Lupus Erythematosus Associated Pitfalls on ¹⁸F-FDG PET/CT: Reactive Follicular Hyperplasia, Kikuchi-Fujimoto Disease, Inflammation and Lymphoid Hyperplasia of the Spleen Mimicking Lymphoma / 대한핵의학회잡지

Systemic lupus erythematosus (SLE) is associated with a variety of inflammatory processes that can affect the lymph nodes, brain, kidneys, and spleen. We present two patients with SLE in whom SLE-associated conditions complicated interpretation of ¹⁸F-fluoro-2-deoxy-D-glucose (18F-FDG) positron emission tomography/computed tomography (PET/CT) imaging of the lymph nodes and the spleen. The imaging findings mimicked lymphoma, but histopathological evaluation showed benign processes including reactive follicular hyperplasia in the lymph nodes, Kikuchi-Fujimoto disease in perisplenic lymph nodes, and inflammatory changes and lymphoid hyperplasia in the spleen.

Association Kikuchi disease with Hashimoto thyroiditis: a case report and literature review

Kikuchi-Fujimoto disease (KFD) is a benign and self-limited disease characterized by fever and lymphadenopathy. The etiology of KFD is unknown, but an autoimmune cause has been suggested. Hashimoto thyroiditis is the most common autoimmune thyroid disorder in children and is known to be associated with other autoimmune diseases. Only a few cases of Hashimoto thyroiditis associated with KFD have been documented. We report a case of a 16-year-old girl who was first diagnosed with KFD and developed Hashimoto thyroiditis 2 years and 6 months later during her follow-up period. Physicians of patients with KFD should consider the possibility of autoimmune diseases like Hashimoto’s thyroiditis.

Kikuchi Fujimoto disease: A series of three cases

@#<p style="text-align: justify;"><strong>INTRODUCTION:</strong> Kikuchi-Fujimoto disease (KFD) is a  rare self-limited disorder manifested by painful cervical lymphadenopathies commonly associated with fever and night sweats.This is a series of three female patients presenting with fever and lymphadenopathies diagnosed with KFD.<br /><strong>CASE:</strong> The first case is a 34-year-old female admitted due to fever of 10 days associated with lymphadenopathies and joint pains.Excision biopsy done showed necrotizing histiocytic lymphadenitis consistent with KFD.Other laboratories showed hypocomplementemia, positive ANA and anti-dsDNA.Patient was discharged improved with low dose oral corticosteroid and hydroxychloroquine.The second case is a 53-year-old female with fever,lymphadenopathies,polyarthritis and morning stiffness.Biopsy of the cervical lymph node was done showing KFD and lupus serologies (ANA 1:640 speckled, anti-dsDNA and anti-Smith) revealed positive results as well.Patient was then diagnosed with SLE and was started on low dose oral corticosteroid and hydroxychloroquine which resulted to resolution of fever and gradual resolution of lymph nodes on out-patient follow up.The last case is a 45-year-old female admitted due to persistent fever, painful lymphadenopathies and headache. Serological work-up including autoantibody tests for SLE were all unremarkable but showed associated iron deficiency anemia. Biopsy of the cervical lymph node showed Kikuchi's disease. Patient was discharged with oral methylprednisolone.<br /><strong>CONCLUSION:</strong> The rarity of KFD makes defining an autoimmune etiology a challenge to clinicians.Careful disease course follow up is then recommended for patients who initially lack parameters for SLE diagnosis.</p>

Kikuchi Fujimoto disease: A series of three cases

INTRODUCTION: Kikuchi-Fujimoto disease (KFD) is a  rare self-limited disorder manifested by painful cervical lymphadenopathies commonly associated with fever and night sweats.This is a series of three female patients presenting with fever and lymphadenopathies diagnosed with KFD.CASE: The first case is a 34-year-old female admitted due to fever of 10 days associated with lymphadenopathies and joint pains.Excision biopsy done showed necrotizing histiocytic lymphadenitis consistent with KFD.Other laboratories showed hypocomplementemia, positive ANA and anti-dsDNA.Patient was discharged improved with low dose oral corticosteroid and hydroxychloroquine.The second case is a 53-year-old female with fever,lymphadenopathies,polyarthritis and morning stiffness.Biopsy of the cervical lymph node was done showing KFD and lupus serologies (ANA 1:640 speckled, anti-dsDNA and anti-Smith) revealed positive results as well.Patient was then diagnosed with SLE and was started on low dose oral corticosteroid and hydroxychloroquine which resulted to resolution of fever and gradual resolution of lymph nodes on out-patient follow up.The last case is a 45-year-old female admitted due to persistent fever, painful lymphadenopathies and headache. Serological work-up including autoantibody tests for SLE were all unremarkable but showed associated iron deficiency anemia. Biopsy of the cervical lymph node showed Kikuchi's disease. Patient was discharged with oral methylprednisolone.CONCLUSION: The rarity of KFD makes defining an autoimmune etiology a challenge to clinicians.Careful disease course follow up is then recommended for patients who initially lack parameters for SLE diagnosis.

A Case of Recurrent Steroid-dependent Kikuchi-Fujimoto Disease Successfully Treated with Hydroxychloroquine / 임상소아혈액종양

Kikuchi-Fujimoto disease (KFD) is known as a self-limiting disease. The most common symptoms include fever, cervical lymphadenopathy, and pain, but nonspecific symptoms such as joint pain, nausea, chills, diaphoresis, and diarrhea may also be present. Its clinical course is generally benign, and symptoms including fever disappear within several months without special treatment. Thus, there is no specific recommended treatment for patients with KFD. However, some patients suffer from prolonged fever or systemic symptoms such as splenomegaly, skin rash, arthralgia, and aseptic meningitis. Many studies have reported the effectiveness of corticosteroids in patients with prolonged fever and systemic symptoms. Our patient also responded favorably to steroids; however, the disease relapsed while tapering. Recently, some studies reported the effectiveness of hydroxychloroquine (HC) in patients with KFD. Herein, we report successful treatment with HC in an adolescent patient with recurrent KFD dependent on steroids without any symptoms of autoimmune disease.

Kikuchi Fujimoto disease: A series of three cases

INTRODUCTION: Kikuchi-Fujimoto disease (KFD) is a  rare self-limited disorder manifested by painful cervical lymphadenopathies commonly associated with fever and night sweats.This is a series of three female patients presenting with fever and lymphadenopathies diagnosed with KFD.CASE: The first case is a 34-year-old female admitted due to fever of 10 days associated with lymphadenopathies and joint pains.Excision biopsy done showed necrotizing histiocytic lymphadenitis consistent with KFD.Other laboratories showed hypocomplementemia, positive ANA and anti-dsDNA.Patient was discharged improved with low dose oral corticosteroid and hydroxychloroquine.The second case is a 53-year-old female with fever,lymphadenopathies,polyarthritis and morning stiffness.Biopsy of the cervical lymph node was done showing KFD and lupus serologies (ANA 1:640 speckled, anti-dsDNA and anti-Smith) revealed positive results as well.Patient was then diagnosed with SLE and was started on low dose oral corticosteroid and hydroxychloroquine which resulted to resolution of fever and gradual resolution of lymph nodes on out-patient follow up.The last case is a 45-year-old female admitted due to persistent fever, painful lymphadenopathies and headache. Serological work-up including autoantibody tests for SLE were all unremarkable but showed associated iron deficiency anemia. Biopsy of the cervical lymph node showed Kikuchi's disease. Patient was discharged with oral methylprednisolone.CONCLUSION: The rarity of KFD makes defining an autoimmune etiology a challenge to clinicians.Careful disease course follow up is then recommended for patients who initially lack parameters for SLE diagnosis.

Ultrasonographic findings of Kikuchi cervical lymphadenopathy in children

PURPOSE: The purpose of this study was to analyze the ultrasonographic (USG) findings of Kikuchi cervical lymphadenopathy in pediatric patients. METHODS: Between April 2007 and September 2016, 84 children (42 male and 42 female; mean±standard deviation age, 12.9±3.2 years; range, 5 to 18 years) confirmed with Kikuchi disease were enrolled. Clinical findings and USG findings of Kikuchi cervical lymphadenopathy were retrospectively reviewed. Localized symptoms, systemic symptoms, and laboratory findings including the white blood cell count, erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP) were analyzed. An analysis of the USG findings included evaluation of the location, size, and presence of intranodal abscess; intranodal calcification; perinodal fat swelling; localized fluid collection; and loss of nodal echogenic hilum. RESULTS: Among the patients, 49 (58%) showed localized tenderness at the cervical lymphadenopathy. Fever was present in 55 (66%), while 27 (32%) had prolonged fever. Of 74 with lab results, 54 (73%) had leukopenia but none had leukocytosis. Among the same 74, there was a high ESR (>50 mm/hr) in 10 (14%) and a high CRP level (>5 mg/dL) in seven (9%). The USG findings of most of the patients (n=72, 86%) showed unilateral neck involvement, especially in the left side neck (45 of 72, 63%). The most common site of Kikuchi lymphadenopathy involvement was the area at cervical lymph node level V, at the posterior triangle (n=77, 92%). Conglomerated nodal distribution (n=57, 68%), preserved central nodal echogenic hilum (n=84, 98%), and perinodal fat swelling (n=55, 65%) were common USG findings in the children with Kikuchi. In addition, multiple cervical lymph nodes showed a relatively even size distribution (n=73, 87%). CONCLUSION: The common USG findings of Kikuchi disease in the pediatric population of our study were multiple conglomerated unilateral cervical lymphadenopathy showing perinodal fat swelling and even size distribution.