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.
Female ; Male ; Humans ; Child ; Histiocytic Necrotizing Lymphadenitis/drug therapy* ; Antibodies, Antinuclear ; Epstein-Barr Virus Infections ; Retrospective Studies ; Herpesvirus 4, Human ; Prognosis ; Steroids

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 (<0.01); the clinical cured rates were both 100% in the two groups on 40 d into treatment. ② The VAS score, axillary temperature and the total score 7 d into treatment, as well as node swelling, VAS score, axillary temperature and the total score 14, 28 and 40 d into treatment in the two groups were significantly improved (<0.01); the total score and VAS score of 7 d into treatment, target lymph node swelling, VAS score and total score of 14 d into treatment in the observation group were significantly improved than those in the control group (<0.01). ③ Compared before treatment, WBC, CRP, ESR in the two groups were significantly improved 14 d and 40 d into treatment (<0.01), and LDH in the two groups were decreased 40 d into treatment (<0.01), but there was no significant difference between the two groups (>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.
Acupuncture Points ; Acupuncture Therapy ; Histiocytic Necrotizing Lymphadenitis ; therapy ; Humans ; Medicine, Chinese Traditional ; Prednisone ; therapeutic use ; Treatment Outcome

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.
Asian Continental Ancestry Group ; Biopsy ; Female ; Fever ; Herpesvirus 4, Human ; Histiocytic Necrotizing Lymphadenitis ; Humans ; Lymph Nodes ; Lymphadenitis ; Mycoplasma pneumoniae ; Mycoplasma ; Parvovirus ; Pneumonia, Mycoplasma

OBJECTIVE: To report a case of a 12-year-old boy with Necrotizing Lymphadenitis (Kikuchi-Fujimoto disease) presenting as lymphadenitis secondary to multiple diagnosis.

METHODS:

Design : Case Report

Setting: Tertiary Private Hospital

Patient: One

RESULTS: 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. 

CONCLUSION:  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.

KEYWORDS: lympadenitis; Kikuchi-Fujimoto disease; lymphoma; cervical lymph nodes

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.
Acoustics* ; Biopsy ; Diagnosis ; Elasticity Imaging Techniques* ; Histiocytic Necrotizing Lymphadenitis* ; Humans ; Hyperplasia ; Lymph Nodes ; Lymphatic Diseases ; Pediatrics ; Ultrasonography

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.
Adult ; Anterior Chamber ; Autoimmune Diseases ; Biopsy, Fine-Needle ; Chills ; Flank Pain ; Fluorescein Angiography ; Headache ; Histiocytes ; Histiocytic Necrotizing Lymphadenitis ; Humans ; Lymph Nodes ; Lymphocytes ; Male ; Myalgia ; Neck ; Neutrophils ; Retinal Hemorrhage ; Retinal Vessels ; Slit Lamp ; Ultrasonography ; Vasculitis ; Visual Acuity ; Vitreous Body

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.
Adolescent ; Autoimmune Diseases ; Child ; Female ; Fever ; Follow-Up Studies ; Hashimoto Disease* ; Histiocytic Necrotizing Lymphadenitis* ; Humans ; Lymphatic Diseases ; Thyroid Gland ; Thyroiditis

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.
Brain ; Electrons ; Histiocytic Necrotizing Lymphadenitis ; Humans ; Hyperplasia ; Inflammation ; Kidney ; Lupus Erythematosus, Systemic ; Lymph Nodes ; Lymphoma ; Positron-Emission Tomography and Computed Tomography ; Spleen

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.
Adolescent* ; Antibodies ; Appetite ; Autoimmune Diseases ; Biopsy ; Child* ; Exanthema ; Ferritins ; Fever ; Histiocytic Necrotizing Lymphadenitis* ; Hospitalization ; Humans ; Korea ; Leukopenia ; Lymph Nodes ; Lymphatic Diseases ; Medical Records ; Pediatrics ; Recurrence ; Retrospective Studies ; Steroids

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.
Diagnosis ; Electrons* ; Fever of Unknown Origin* ; Fever* ; Histiocytic Necrotizing Lymphadenitis ; Humans ; Korea ; Positron-Emission Tomography and Computed Tomography ; Prognosis ; Retrospective Studies ; Tertiary Care Centers