RESUMEN
OBJECTIVE: To analyze the clinical data of histiocytic necrotizing lymphadenitis(HNL), comparing the similarities and differences between children and adults, to deepen the understanding of the disease by clinical physicians, and to improve diagnostic rate and reduce misdiagnosis and mistreatment. METHODS: The clinical data of hospitalized patients with histiocytic necrotizing lymphadenitis diagnosed by biopsy from January 2010 to August 2023 in Peking University First Hospital were collec-ted, and the clinical features, laboratory examinations, pathological features, treatments with antibiotics and glucocorticoids, and prognosis of histiocytic necrotic lymphadenitis were analyzed. Grouped based on age, the differences of clinical characteristics, laboratory tests, treatment, and prognosis between the children group (< 16 years old) and the adult group (≥16 years old) were compared. RESULTS: Among the 81 enrolled patients, there were 42 males and 39 females. The median age was 21(14, 29) years, the median duration of disease was 20.0(13.0, 30.0) days, and the median length of hospital stay was 13.0 (10.0, 15.0) days. The first symptoms were fever, lymphadenopathy, and both. All the patients had enlarged lymph nodes with different parts and sizes, 96.3% (78 of 81) of the patients had cervical lymphadenopathy, 50.6% (41 of 81) had bilateral cervical lymphadenopathy, 55.6% (45 of 81) had supraclavicular, axillary or inguinal lymphadenopathy, and the median lymph node diameter was 20.0(20.0, 30.0) mm. Only one patient had no fever, the other 80 patients had fever, the median peak body temperature was 39.0(38.0, 39.8) â. Accompanying symptoms: rash (8.6%, 7/81), fatigue (34.6%, 28/81), night sweating (8.6%, 7/81), chills (25.3%, 25/81), muscle soreness (13.6%, 11/81), and joint pain (6.2%, 5/81). There were 17 cases (21.0%, 17/81) of hepatosplenomegaly, of which 12 cases (70.6%, 12/17) were splenomegaly. 68.8%(55/80) of patients had a decrease in white blood cell (WBC) count, with 47.5%(38/80)increased in lymphocyte(LY)proportion, 53.4%(39/73) increased in high-sensitivity C-reactive protein(CRP), 79.2%(57/72) increased in erythrocyte sedimentation rate(ESR), 22.2%(18/81) increased in alanine transaminase(ALT), 27.2%(22/81) elevated in aspartate transaminase(AST), and 81.6%(62/76) elevated in lactate dehydrogenase(LDH). All the 81 patients underwent lymph node biopsy, and 77.8%(63/81) of the patients showed that most of the structures in the lymph nodes were destroyed or disappeared, and 16.0%(13/81) of the lymph nodes were still in existence, hyperplasia and normal lymph node were 1.2%(1/81) respectively, and 3.7%(3/81) had normal lymph node structures. Immunohistochemical staining was performed in 67 cases. The percentages of CD3+ and CD68(KP1)+ were respectively 97.0%(65/67), and MPO+ were 94.0%(63/67). In the study, 51 patients (63.0%, 51/81) were treated with glucocorticoid therapy after diagnosis. The median time for temperature to return to normal was 1.0(1.0, 4.0) days after glucocorticoid therapy. when the glucocorticoid treatment worked best, the body temperature could drop to normal on the same day. There were significant differences in length of stay, predisposing factors, chills, the rate of increase in high-sensitivity CRP, antibiotic and glucocorticoid treatment between the adults and children groups (P < 0.05). CONCLUSION: In clinical practice, if there are cases with unexplained fever, superficial lymph node enlargement, and reduced white blood cells as clinical characteristics, and general antibiotics treatment is ineffective, histiocytic necrotic lymphadenitis should be considered. Lymph node biopsy should be performed as early as possible to clarify the diagnosis, reduce misdiagnosis and mistreatment, and symptomatic treatment should be the main treatment. Glucocorticoids therapy has a definite therapeutic effect.
Asunto(s)
Linfadenitis Necrotizante Histiocítica , Humanos , Masculino , Linfadenitis Necrotizante Histiocítica/diagnóstico , Linfadenitis Necrotizante Histiocítica/tratamiento farmacológico , Linfadenitis Necrotizante Histiocítica/patología , Femenino , Adolescente , Adulto , Adulto Joven , Niño , Antibacterianos/uso terapéutico , Glucocorticoides/uso terapéutico , Pronóstico , Fiebre/etiología , Ganglios Linfáticos/patología , Linfadenopatía/patologíaRESUMEN
Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare, benign condition affecting young Oriental-Asian females. It is characterized by fever and tender cervical lymphadenopathy with an unclear aetiology, and in most longitudinal reviews, KFD occurs before systemic lupus erythematosus (SLE). Herein, the case of a 28-year-old Kuwaiti female without any relevant past medical history, who was simultaneously diagnosed with KFD and SLE following an Ebstein-Barr virus infection, is reported. The patient was treated with oral prednisolone, hydroxychloroquine, cyclosporin, and belimumab and her response was clinically and biochemically favourable. Although KFD is prevalent in Asian populations, it may affect all races. Early diagnosis of KFD is difficult, particularly when simultaneously diagnosed with SLE, but crucial to preventing inappropriate therapy. Clinicians need to know about this rare disease, especially when patients present with fever and swollen lymph nodes, due to a risk of misdiagnosis with tuberculosis or lymphoma, as these are more often thought to be the cause of such symptoms.
Asunto(s)
Linfadenitis Necrotizante Histiocítica , Lupus Eritematoso Sistémico , Adulto , Femenino , Humanos , Árabes , Linfadenitis Necrotizante Histiocítica/diagnóstico , Linfadenitis Necrotizante Histiocítica/tratamiento farmacológico , Linfadenitis Necrotizante Histiocítica/patología , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/tratamiento farmacológico , Prednisolona/uso terapéutico , Prednisolona/administración & dosificación , KuwaitRESUMEN
Kikuchi-Fujimoto disease (KFD) is an inflammatory disease of unknown aetiology characterised by fever and cervical lymphadenopathy. Although KFD is a self-limiting disease, patients with severe or long-lasting course require glucocorticoid therapy. We presently report a 17-year-old boy with KFD who had seven relapses since the onset at 4 years old. He suffered from hypothermia, bradycardia, and hypotension during the treatment with prednisolone or methylprednisolone. All of his vital signs recovered after cessation of the drug in addition to fluid replacement and warming. Thus, glucocorticoid was effective but could not be continued because of the adverse event. Although hypothermia developed during the treatment with 5 mg/kg/day of cyclosporine A (CsA) at his second relapse, he was successfully treated with lower-dose CsA (3 mg/kg/day). Thereafter, he had five relapses of KFD until the age of 12 years and was treated by 1.3-2.5 mg/kg/day of CsA. Hypothermia accompanied by bradycardia and hypotension developed soon after concomitant administration of ibuprofen at his fifth and sixth relapses even during low-dose CsA therapy. Conclusively, glucocorticoid, standard dose of CsA, or concomitant use of non-steroidal anti-inflammatory drugs may cause hypothermia, bradycardia, and hypotension and needs special attention. Low-dose CsA could be a choice for such cases with KFD.
Asunto(s)
Bradicardia , Ciclosporina , Glucocorticoides , Linfadenitis Necrotizante Histiocítica , Hipotensión , Hipotermia , Humanos , Masculino , Bradicardia/inducido químicamente , Bradicardia/diagnóstico , Bradicardia/etiología , Ciclosporina/efectos adversos , Ciclosporina/uso terapéutico , Ciclosporina/administración & dosificación , Adolescente , Glucocorticoides/uso terapéutico , Glucocorticoides/efectos adversos , Glucocorticoides/administración & dosificación , Hipotensión/inducido químicamente , Hipotensión/etiología , Hipotermia/inducido químicamente , Hipotermia/diagnóstico , Linfadenitis Necrotizante Histiocítica/diagnóstico , Linfadenitis Necrotizante Histiocítica/complicaciones , Linfadenitis Necrotizante Histiocítica/tratamiento farmacológico , Inmunosupresores/efectos adversos , Inmunosupresores/uso terapéutico , Metilprednisolona/administración & dosificación , Metilprednisolona/uso terapéutico , Metilprednisolona/efectos adversos , Prednisolona/administración & dosificación , Prednisolona/uso terapéutico , Prednisolona/efectos adversos , RecurrenciaRESUMEN
Kikuchi-Fujimoto disease (KFD) is a benign and self-limited disorder that usually presents with regional cervical lymphadenopathy and fever. We report a case of a 12-year-old female who complained of fever, night sweating, significant weight loss, and tender right cervical lymph node enlargement for 2 months. A full workup including laboratory tests and imaging studies, an excisional biopsy, and histopathological analysis were done, and the diagnosis of KFD was confirmed. The patient was treated with analgesia and oral prednisolone, resulting in good improvement. A high degree of clinical suspicion is imperative for physicians, given the rarity of the disease and the associated diagnostic challenges.
Asunto(s)
Linfadenitis Necrotizante Histiocítica , Linfadenopatía , Femenino , Humanos , Niño , Linfadenitis Necrotizante Histiocítica/complicaciones , Linfadenitis Necrotizante Histiocítica/diagnóstico , Linfadenitis Necrotizante Histiocítica/tratamiento farmacológico , Linfadenopatía/complicaciones , Prednisolona/uso terapéutico , Fiebre/etiología , DolorRESUMEN
Kikuchi-Fujimoto disease, or histiocytic necrotizing lymphadenitis, is in most cases a benign disease which affects lymph nodes in the cervical region. Cervical adenopathy and fever are the most common symptoms, and young adults are mostly affected. Lymph node biopsy is the mode of diagnosis with demonstration of paracortical areas of apoptotic necrosis with abundant karyorrhectic debris and a proliferation of histiocytes, plasmacytoid dendritic cells, and CD8+ T cells in the absence of neutrophils. In most cases, the disease is self-limiting but it can be recurrent or evolve to SLE. Treatment varies from symptomatic to more systemic with cortisone and intravenous immunoglobulin.
Asunto(s)
Linfadenitis Necrotizante Histiocítica , Linfadenitis , Adulto Joven , Humanos , Linfadenitis Necrotizante Histiocítica/diagnóstico , Linfadenitis Necrotizante Histiocítica/tratamiento farmacológico , Linfadenitis Necrotizante Histiocítica/patología , Linfadenitis/diagnóstico , Biopsia , Necrosis/diagnóstico , Necrosis/patología , Diagnóstico Diferencial , Ganglios Linfáticos/patologíaRESUMEN
A 13-year-old Black male patient with a history of Kikuchi-Fujimoto disease (KFD) and sickle cell trait presented with acute painless vision loss and no light perception vision (NLP) in his left eye. The examination was indicative of occlusive retinal vasculitis with near total central retinal artery occlusion (CRAO). He was started on oral steroids with dramatic reperfusion and improvement of the retinal hemorrhages. However, his vision remained at NLP. Oral steroids were tapered, and rituximab infusion was initiated. While ocular involvement is uncommon in KFD, vision-limiting complications, such as occlusive retinal vasculitis, ophthalmic artery occlusion, and CRAO can occur. Early systemic immunosuppression is key in achieving rapid remission. [Ophthalmic Surg Lasers Imaging Retina 2024;55:235-239.].
Asunto(s)
Angiografía con Fluoresceína , Linfadenitis Necrotizante Histiocítica , Vasculitis Retiniana , Rasgo Drepanocítico , Humanos , Masculino , Linfadenitis Necrotizante Histiocítica/diagnóstico , Linfadenitis Necrotizante Histiocítica/complicaciones , Linfadenitis Necrotizante Histiocítica/tratamiento farmacológico , Rasgo Drepanocítico/complicaciones , Rasgo Drepanocítico/diagnóstico , Vasculitis Retiniana/diagnóstico , Vasculitis Retiniana/etiología , Adolescente , Angiografía con Fluoresceína/métodos , Agudeza Visual , Oclusión de la Arteria Retiniana/diagnóstico , Oclusión de la Arteria Retiniana/etiología , Fondo de Ojo , Glucocorticoides/uso terapéutico , Glucocorticoides/administración & dosificaciónRESUMEN
BACKGROUND: Histiocytic necrotizing lymphadenitis (HNL) is a self-limited inflammatory disease of unknown pathogenesis. A very small fraction of patients with HNL could develop hemophagocytic lymphohistiocytosis (HLH), a hyperinflammatory disorder. These patients are diagnosed as HNL with HLH (HNL-HLH). HNL-HLH in the pediatric population has been systemically studied, however, the clinical, laboratory, and radiological features and outcomes of adult patients with HNL-HLH remain to be explored. We aimed to explore the clinical, laboratory, and radiological features and outcomes of adult patients with HNL-HLH. METHODS: We collected the clinical data of patients with HNL-HLH admitted to the First Affiliated Hospital of Nanjing Medical University from October 2010 to June 2015. All the patients underwent lymph node biopsy and have a pathological diagnosis of HNL. The age, gender, clinical presentation, lymph node signs, laboratory findings and imaging data, and pathological findings of the patients were collected. RESULTS: In this study, we reported five adult patients with HNL-HLH. All five patients showed enlarged lymph nodes and prolonged fever. Laboratory findings were consistent with the diagnosis of HLH. 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) showed enlarged lymph nodes with increased FDG uptake and splenic hypermetabolism could be present. All the patients responded well to corticosteroids and had a good prognosis. Two of the five patients were diagnosed with systemic lupus erythematosus during the follow-up. CONCLUSIONS: Our study demonstrated that adult patients with HNL-HLH showed distinct clinical, laboratory, and radiological features. And the prognosis is good and patients could be managed with steroids and supportive care.
Asunto(s)
Linfadenitis Necrotizante Histiocítica , Linfohistiocitosis Hemofagocítica , Adulto , Humanos , Niño , Linfadenitis Necrotizante Histiocítica/complicaciones , Linfadenitis Necrotizante Histiocítica/diagnóstico , Linfadenitis Necrotizante Histiocítica/tratamiento farmacológico , Linfohistiocitosis Hemofagocítica/complicaciones , Linfohistiocitosis Hemofagocítica/diagnóstico , Linfohistiocitosis Hemofagocítica/tratamiento farmacológico , Tomografía Computarizada por Tomografía de Emisión de Positrones/efectos adversos , Ganglios Linfáticos , Biopsia/efectos adversosRESUMEN
Immune-mediated necrotizing myopathy (IMNM) is a distinct type of idiopathic inflammatory myositis, pathologically characterized by myofiber necrosis and degeneration in the absence of lymphocyte infiltration. Herein, we present a case of IMNM with concomitant development of Kikuchi-Fujimoto disease (KFD), characterized by histiocytic necrotizing lymphadenitis, in a 36-year-old woman who had a treatment history for rheumatoid arthritis (RA). Treatment with oral prednisolone and tacrolimus as immunosuppressants resulted in the remission of the skeletomuscular involvement and lymphadenopathy. To the best of our knowledge, this is the first report of IMNM and KFD developing concomitantly during the clinical course of RA.
Asunto(s)
Artritis Reumatoide , Enfermedades Autoinmunes , Linfadenitis Necrotizante Histiocítica , Miositis , Femenino , Humanos , Adulto , Linfadenitis Necrotizante Histiocítica/complicaciones , Linfadenitis Necrotizante Histiocítica/diagnóstico , Linfadenitis Necrotizante Histiocítica/tratamiento farmacológico , Prednisolona/uso terapéutico , Enfermedades Autoinmunes/tratamiento farmacológico , Inmunosupresores/uso terapéutico , Miositis/complicaciones , Miositis/diagnóstico , Miositis/tratamiento farmacológico , Artritis Reumatoide/tratamiento farmacológicoRESUMEN
BACKGROUND: Kikuchi-Fujimoto disease is an uncommon systemic disease that mostly affects young women. Kikuchi-Fujimoto disease typically manifests as necrotizing lymphadenopathy, which frequently follows by a fever; however, Kikuchi-Fujimoto disease occurs rarely in extranodal regions. One of the most important accompaniments of Kikuchi-Fujimoto disease is its connection with autoimmune diseases such as systemic lupus erythematosus. This case presents a simultaneous occurrence of Kikuchi-Fujimoto disease with liver involvement and systemic lupus erythematosus in a young female patient. CASE PRESENTATION: We present a rare case of a 20-year-old white woman who presented with fever, joint pains, myalgia, and shortness of breath. Initial hospitalization and treatment for fever of unknown origin did not yield improvement. Physical examination revealed cervical and supraclavicular lymphadenopathy, and laboratory investigations showed abnormal blood counts, elevated inflammatory markers, and positive autoimmune serologies. Imaging studies revealed bilateral pleural effusion and liver lesions. Lymph node biopsy confirmed the diagnosis of Kikuchi-Fujimoto disease, and liver biopsy showed extranodal involvement. The patient was diagnosed with Kikuchi-Fujimoto disease-associated systemic lupus erythematosus and treated with hydroxychloroquine and corticosteroids. The patient showed gradual resolution of symptoms and lymphadenopathy with treatment. CONCLUSION: Kikuchi-Fujimoto disease is a rare systemic condition primarily impacting young females. It is characterized by necrotizing lymphadenopathy, often accompanied by fever. Although Kikuchi-Fujimoto disease is predominantly seen in the lymph nodes, occurrences in non-nodal areas are infrequent. When diagnosing Kikuchi-Fujimoto disease, it is essential to screen patients for systemic lupus erythematosus. In this particular case, we observed liver involvement along with the presence of both Kikuchi-Fujimoto disease and systemic lupus erythematosus.
Asunto(s)
Linfadenitis Necrotizante Histiocítica , Lupus Eritematoso Sistémico , Linfadenopatía , Femenino , Humanos , Adulto Joven , Fiebre/etiología , Linfadenitis Necrotizante Histiocítica/complicaciones , Linfadenitis Necrotizante Histiocítica/diagnóstico , Linfadenitis Necrotizante Histiocítica/tratamiento farmacológico , Hidroxicloroquina/uso terapéutico , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/tratamiento farmacológico , Ganglios Linfáticos/patología , Linfadenopatía/etiología , Linfadenopatía/patologíaRESUMEN
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.
Asunto(s)
Infecciones por Virus de Epstein-Barr , Linfadenitis Necrotizante Histiocítica , Femenino , Masculino , Humanos , Niño , Linfadenitis Necrotizante Histiocítica/diagnóstico , Linfadenitis Necrotizante Histiocítica/tratamiento farmacológico , Anticuerpos Antinucleares , Estudios Retrospectivos , Herpesvirus Humano 4 , Pronóstico , EsteroidesRESUMEN
Patients from countries, endemic with tuberculosis, who present with febrile lymphadenopathy refractory to first line antibiotics are often empirically treated for extra-pulmonary tuberculosis. However, Kikuchi-Fujimoto Disease (KFD) or histiocytic necrotizing lymphadenitis, a self-limiting and benign condition, presents with similar clinical symptoms. We present an adolescent with febrile lymphadenopathy, who was initially treated for tubercular lymphadenopathy, before a diagnosis of KFD was made.
Asunto(s)
Linfadenitis Necrotizante Histiocítica , Linfadenitis , Linfadenopatía , Tuberculosis Ganglionar , Adolescente , Humanos , Diagnóstico Diferencial , Linfadenitis/diagnóstico , Linfadenitis Necrotizante Histiocítica/diagnóstico , Linfadenitis Necrotizante Histiocítica/tratamiento farmacológico , Tuberculosis Ganglionar/diagnóstico , Tuberculosis Ganglionar/tratamiento farmacológico , Linfadenopatía/diagnóstico , Fiebre/diagnósticoRESUMEN
We present the case of a patient diagnosed with ankylosing spondylitis (AS) along with Kikuchi-Fujimoto disease (KFD), possibly triggered by infection or autoimmunity. Both AS and KFD involved a similar immune pathway. Hence, clinicians should consider the possibility of KFD when lymphadenopathy is observed, especially in patients with infection or underlying autoimmune diseases.
Asunto(s)
Linfadenitis Necrotizante Histiocítica , Espondilitis Anquilosante , Humanos , Linfadenitis Necrotizante Histiocítica/complicaciones , Linfadenitis Necrotizante Histiocítica/diagnóstico , Linfadenitis Necrotizante Histiocítica/tratamiento farmacológico , Espondilitis Anquilosante/complicaciones , Espondilitis Anquilosante/diagnóstico , Espondilitis Anquilosante/tratamiento farmacológico , AutoinmunidadRESUMEN
The inflammatory response to viral infection is an important component of the antiviral response, a process that involves the activation and proliferation of CD8+ T, CD4+ T, and dendritic cells; thus, viral infection disrupts the immune homeostasis of the organism, leading to an increased release of inflammatory factors. Kikuchi-Fujimoto disease (KFD) is an inflammatory self-limited disorder of unknown etiology, and it is generally believed that the pathogenesis of this disease includes two aspects: viral infection and autoimmune response. Various immune cells, such as CD8+ T lymphocytes, CD4+ T lymphocytes, and CD123+ plasmacytoid dendritic cells, as well as the cytokines they induce and secrete, such as interferons, interleukins, and tumor necrosis factors, play a crucial role in the pathogenesis of KFD. In this article, we present a case study of a young female patient from China who exhibited typical symptoms of lymph node inflammation and fever. The diagnosis of KFD was confirmed through a lymph node biopsy. She presented with elevated ESR, IL-6, and IFN-γ. Viral markers showed elevated IgG and IgM of cytomegalovirus (CMV) and elevated IgG of Epstein-Barr virus (EBV), while changes occurred in the CD4+ T and CD8+ T cell counts. Eventually, the patient achieved disease relief through steroid treatment. Based on these findings, we conducted a comprehensive review of the involvement of viral infection-induced inflammatory response processes and autoimmunity in the pathogenesis of Kikuchi-Fujimoto disease.
Asunto(s)
Infecciones por Virus de Epstein-Barr , Linfadenitis Necrotizante Histiocítica , Linfadenopatía , Fiebre Recurrente , Femenino , Humanos , Diagnóstico Diferencial , Infecciones por Virus de Epstein-Barr/patología , Herpesvirus Humano 4 , Linfadenitis Necrotizante Histiocítica/diagnóstico , Linfadenitis Necrotizante Histiocítica/tratamiento farmacológico , Linfadenitis Necrotizante Histiocítica/patología , Inmunoglobulina G , Ganglios Linfáticos/patología , Linfadenopatía/diagnósticoRESUMEN
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.
Asunto(s)
Femenino , Masculino , Humanos , Niño , Linfadenitis Necrotizante Histiocítica/tratamiento farmacológico , Anticuerpos Antinucleares , Infecciones por Virus de Epstein-Barr , Estudios Retrospectivos , Herpesvirus Humano 4 , Pronóstico , EsteroidesRESUMEN
The Kikuchi-Fujimoto disease (histiocytic necrotizing lymphadenitis) is an autolimited process, which can be caused by viral agents like Epstein-Barr virus, human herpes virus, HIV, B19 parvovirus, paromyxoviruses, SARS-CoV-2, Toxoplasma and Yersinia. The correct diagnosis is based on histological findings. A 45-year-old female patient presented in our ambulatory room with a unilateral neck mass, fever, dysphonia and dysphagia. The patient was double-tested positive for SARS-CoV-2 approximately 1 month before the symptoms started. Before examination, the nasopharyngeal rapid test for SARS-CoV-2 resulted negative. After hospitalization, intravenous antibiotic (Augmentin, 3 x 1.2 g; Klion, 2 x 100 mg) and steroid (Solu-Medrol, 2 x 125 mg) therapy were administered. The neck and chest CT described a right-sided retropharyngeal abscess with bilateral neck lympadenopathy. Urgent tracheotomy, neck dissection and lymph node biopsy were made. The intraoperative findings excluded the presence of the abscess. The histological findings confirmed the necrotizing histiocytic lymphadenitis. Despite of the fact that the Kikuchi-Fujimoto disease is autolimited, we can see that, in this particular case, the lymphadenopathy after the SARS-CoV-2 infection caused a life-threatening situation. The formed extratissular liquid imitated the image of a retropharyngeal abscess. In the searched worldwide literature, similar intervention for this type of process was not described. Tracheotomy, neck dissection and removing the lymph nodes as ,,trigger zones used the full recovery of the patient. In the future, we consider important proving and clarifying the correlation between SARS-CoV-2 and Kikuchi-Fujimoto disease.
Asunto(s)
COVID-19 , Infecciones por Virus de Epstein-Barr , Linfadenitis Necrotizante Histiocítica , Absceso Retrofaríngeo , COVID-19/complicaciones , COVID-19/diagnóstico , Diagnóstico Diferencial , Infecciones por Virus de Epstein-Barr/diagnóstico , Infecciones por Virus de Epstein-Barr/patología , Femenino , Herpesvirus Humano 4 , Linfadenitis Necrotizante Histiocítica/complicaciones , Linfadenitis Necrotizante Histiocítica/diagnóstico , Linfadenitis Necrotizante Histiocítica/tratamiento farmacológico , Humanos , Ganglios Linfáticos , Persona de Mediana Edad , Absceso Retrofaríngeo/diagnóstico , Absceso Retrofaríngeo/patología , SARS-CoV-2RESUMEN
A 34-year-old man presented to our hospital with a 5-day history of progressive abdominal pain and fever. A CT scan identified extensive mesenteric lymphadenopathy. Initial diagnostic tests were inconclusive. Abdominal lymph node biopsy showed histiocytic necrotising lymphadenitis, compatible with Kikuchi-Fujimoto disease (KFD). This benign and self-limiting disease generally resolves following supportive treatment. In this case, remission occurred within 3 weeks of initial presentation. KFD is a very uncommon cause of lymphadenopathy, and selective mesenteric involvement is rare. Definitive diagnosis often requires lymph node biopsy. It is important to exclude more common and serious differential diagnoses associated with mesenteric lymphadenopathy, while maintaining a minimally invasive diagnostic approach, before progressing to nodal biopsy.
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Linfadenitis Necrotizante Histiocítica , Linfadenopatía , Adulto , Diagnóstico Diferencial , Linfadenitis Necrotizante Histiocítica/complicaciones , Linfadenitis Necrotizante Histiocítica/diagnóstico , Linfadenitis Necrotizante Histiocítica/tratamiento farmacológico , Humanos , Ganglios Linfáticos/diagnóstico por imagen , Ganglios Linfáticos/patología , Linfadenopatía/patología , Masculino , Enfermedades Raras/diagnósticoRESUMEN
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.
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Linfadenitis Necrotizante Histiocítica/diagnóstico , Linfadenitis Necrotizante Histiocítica/fisiopatología , Niño , Diagnóstico Diferencial , Femenino , Fiebre , Linfadenitis Necrotizante Histiocítica/tratamiento farmacológico , Linfadenitis Necrotizante Histiocítica/patología , Humanos , Hidroxicloroquina/uso terapéutico , Linfadenopatía/diagnóstico , Linfadenopatía/fisiopatología , Resultado del TratamientoRESUMEN
ABSTRACT: Histiocytic necrotizing lymphadenitis (HNL) is a rare, benign, and self-limiting inflammatory disease that mainly involves the lymph nodes. There is a lack of large sample studies concerning the clinical manifestations and imaging features of 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) of HNL.The clinical symptoms, laboratory examination results, 18F-FDG PET/CT imaging features, and treatment outcome were investigated in this retrospective study.A total of 40 HNL patients were recruited. The onset age was between 14 and 65âyears, with a median of 25âyears. The white blood cell count was 3.9 (2.9, 7.1)â×â109/L, C-reactive protein level was 20.2 (6.6, 63.8) mg/L, erythrocyte sedimentation rate was 29.0 (18.0,45.0) mm/h, and ferritin was 616.5 (205.6, 2118.1) ng/mL. An abnormal liver function was observed in 23 patients. 18F-FDG PET-CT showed that an abnormal lymph node metabolism was observed in 38 patients, among which the highest 18F-FDG maximal standard uptake value (SUVmax) of the lymph nodes ranged between 3.4 and 41.9; the nodes were mainly distributed in the neck and axilla regions. Meanwhile, a total of 2502 lymph nodes (721 lymph nodes with a short axis greater than 10âmm) were found in the 38 patients, including 1837 lymph nodes with an 18F-FDG SUVmax ≥ 2.5. The 18F-FDG SUVmax of the spleen ranged from 2.5 to 9.2 in 20 patients, while that of central and peripheral bone marrow ranged from 2.7 to 36.0 in 30 patients. After follow-up for an average period of 1 month, the symptoms improved after prednisone treatment.HNL often occurs in adolescents. Scanning with 18F-FDG PET/CT showed that most patients had multiple involved lymph nodes that were hypermetabolic, and only few lymph nodes are enlarged. Besides, the spleen or central and peripheral bone marrow could sometimes be hypermetabolic. Glucocorticoid treatment for the HNL patients is effective.
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Fluorodesoxiglucosa F18 , Linfadenitis Necrotizante Histiocítica/diagnóstico por imagen , Tomografía Computarizada por Tomografía de Emisión de Positrones , Adolescente , Adulto , Anciano , Antiinflamatorios/administración & dosificación , Femenino , Linfadenitis Necrotizante Histiocítica/tratamiento farmacológico , Linfadenitis Necrotizante Histiocítica/patología , Humanos , Ganglios Linfáticos/patología , Masculino , Persona de Mediana Edad , Prednisona/administración & dosificación , Estudios Retrospectivos , Adulto JovenRESUMEN
A 35-year-old woman presented with a constellation of systemic symptoms: rashes, weight loss, arthralgia and mouth ulcers. Six months afterwards, she experienced bilateral and sequential reduction in vision, and was found to have bilateral vaso-occlusive retinopathy, with critical macular ischaemia in the left eye. Her serological markers were consistent with a diagnosis of lupus. A lymph node biopsy confirmed Kikuchi-Fujimoto disease, a benign condition of unknown cause characterised by fever, cervical and axillary lymphadenopathy. Given that this overlap syndrome was associated with a number of systemic features and had affected the eyes, an immunosuppressive regime with rituximab was considered prudent. This rendered her vasculitis stable and non-progressive, and there were signs of partial retinal microvasculature recovery on optical coherence tomography angiography. There is increasing evidence of an overlap between Kikuchi-Fujimoto disease and systemic lupus erythematosus, which is associated with vaso-occlusive retinopathy. In these instances, a multidisciplinary approach is warranted, with consideration of appropriate treatment in order to prevent harmful sequelae of vasculitis. Our treatment with rituximab abated the disease process, although close follow-up is paramount to monitor results and side-effects of treatment.
Asunto(s)
Linfadenitis Necrotizante Histiocítica , Lupus Eritematoso Sistémico , Enfermedades de la Retina , Adulto , Biopsia , Femenino , Fiebre , Linfadenitis Necrotizante Histiocítica/complicaciones , Linfadenitis Necrotizante Histiocítica/diagnóstico , Linfadenitis Necrotizante Histiocítica/tratamiento farmacológico , Humanos , Lupus Eritematoso Sistémico/complicacionesRESUMEN
We present a 47-year-old, South-African origin, woman with a background of stable monoclonal gammopathy of unknown significance (MGUS) who attended A&E with a history of coryzal symptoms associated with persistent fever, lymphadenopathy and a new onset of rash, not responding to antibiotics and paracetamol. A trial of high-dose steroids resolved symptoms. Bone marrow biopsy confirmed a progression of MGUS into multiple myeloma and her axillary lymph node biopsy analysis supported a diagnosis of Kikuchi-Fujimoto disease (KFD). This is an unusual presentation where KFD has been noted alongside MGUS progression to multiple myeloma. Haematology follow-up is underway.