We report the first case demonstrating an association between Kawasaki disease (KD) and erythema nodosum (EN). A 3-year-old girl presented with EN as an initial manifestation of KD. At the initial visit, she showed high fever of 40℃, injection of the oropharynx, cervical lymphadenopathy, and red-purple cutaneous nodules, particularly on the lower limbs. She complained of severe pain in the neck and cutaneous lesions. Initially, the development of EN was attributed to Salmonella spp infection, which was detected in stool culture. However, the patient did not respond to high-dose ampicillin/sulbactam to which the Salmonella spp is sensitive. Echocardiography performed as screening for fever of unknown origin revealed medium-sized aneurysms of the left anterior descending artery. EN masked the diagnosis of KD, and the patient developed a coronary artery lesion. KD should be considered in the differential diagnosis of refractory EN in pediatric patients.
Aneurysm ; Arteries ; Bacterial Infections ; Child, Preschool ; Coronary Vessels ; Diagnosis ; Diagnosis, Differential ; Echocardiography ; Erythema Nodosum ; Erythema ; Exanthema ; Female ; Fever ; Fever of Unknown Origin ; Humans ; Lower Extremity ; Lymphatic Diseases ; Masks ; Mass Screening ; Mucocutaneous Lymph Node Syndrome ; Neck ; Oropharynx ; Salmonella ; Skin ; Subcutaneous Tissue

Adult-onset Still's disease (AOSD) is a rare systemic febrile disorder of unknown etiology. AOSD is characterized by spiking fever, arthralgia, sore throat, skin rash, and multi-organ involvement but is difficult to diagnose due to the lack of specific clinical features. AOSD is most frequently observed among young adults. We describe the case of an 18-year-old male patient with multiple cerebral infarction who was diagnosed with AOSD based on the clinical features observed during rehabilitation treatment after the diagnosis of ischemic stroke. Clinical symptoms of the patient was controlled with steroid therapy. If cerebral infarction is accompanied by unusual clinical features as non-infectious fever, rash, and arthralgia, AOSD should be considered in young stroke patient.
Adolescent ; Arthralgia ; Cerebral Infarction* ; Diagnosis ; Exanthema ; Fever ; Fever of Unknown Origin ; Humans ; Male ; Pharyngitis ; Rehabilitation ; Still's Disease, Adult-Onset* ; Stroke ; Young Adult

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

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

PURPOSE: Fever is one of the most common symptoms in children. In previous studies, infectious disease was the most common cause of pediatric fever of unknown origin (FUO). The aim of this study is to investigate the etiology, clinical characteristics and prognosis of pediatric FUO in 21 century with more diagnostics available and to analyze the factors for certain disease categories. METHODS: Among the children under 18 years old who were hospitalized at Samsung Medical Center from January 2000 to December 2014, the patients who met the criteria including fever of ≥38.0℃ for longer than ≥14 days and failure to reach a diagnosis after one week of investigations were included. RESULTS: Total 100 patients were identified. Confirmed diagnosis was achieved in 57 patients (57%). Among them, infectious diseases (n=19, 19%) were most common, followed by connective tissue diseases (n=15, 15%), necrotizing lymphadenitis (n=8, 8%), and malignancies (n=7, 7%). Children with fever duration over 28 days had a trend for higher frequency of connective tissue diseases (28.3%) except undiagnosed etiology. The symptoms such as arthritis, lymph node enlargement and only fever without other symptoms were significantly related with connective tissue diseases, necrotizing lymphadenitis and undiagnosed respectively (P<0.001). Ninety-two patients have become afebrile at discharge and 1 patient died (1%). CONCLUSION: Almost half of our patients were left without diagnosis. Although it has been known that infectious disease was most common cause of pediatric FUO in the past, undiagnosed portion of FUO have now increased due to development of diagnostic techniques for infectious diseases.
Arthritis ; Child* ; Communicable Diseases ; Connective Tissue Diseases ; Diagnosis ; Fever of Unknown Origin* ; Fever* ; Humans ; Lymph Nodes ; Lymphadenitis ; Prognosis

PURPOSE: Infection and malignancy represent two common complications after solid organ transplantation, which are often characterized by poorly specific clinical symptomatology. Herein, we have evaluated the role of 18F-fluoro-2-deoxy-Dglucose (FDG) positron emission tomography/computed tomography (PET/CT) in this clinical setting.METHODS: Fifty-eight consecutive patients who underwent FDG PET/CT after kidney, lung or heart transplantation were included in this retrospective analysis. Twelve patients underwent FDGPET/CT to strengthen or confirma diagnostic suspicion of malignancies. The remaining 46 patients presented with unexplained inflammatory syndrome, fever of unknown origin (FUO), CMVor EBV seroconversion during post-transplant follow-up without conclusive conventional imaging. FDG PET/CT results were compared to histology or to the finding obtained during a clinical/imaging follow-up period of at least 6 months after PET/CT study.RESULTS: Positive FDG PET/CT results were obtained in 18 (31 %) patients. In the remaining 40 (69 %) cases, FDG PET/CT was negative, showing exclusively a physiological radiotracer distribution. On the basis of a patient-based analysis, FDG PET/CT's sensitivity, specificity, PPV and NPV were respectively 78 %, 90 %, 78 % and 90 %, with a global accuracy of 86 %. FDG PET/CT was true positive in 14 patients with bacterial pneumonias (n = 4), pulmonary fungal infection (n = 1), histoplasmosis (n = 1), cutaneous abscess (n = 1), inflammatory disorder (sacroiliitis) (n = 1), lymphoma (n = 3) and NSCLC (n = 3). On the other hand, FDG PET/CT failed to detect lung bronchoalveolar adenocarcinoma, septicemia, endocarditis and graft-versus-host disease (GVHD), respectively, in four patients. FDG PET/CT contributed to adjusting the patient therapeutic strategy in 40 % of cases.CONCLUSIONS: FDG PET/CT emerges as a valuable technique to manage complications in the post-transplantation period. FDG PET/CT should be considered in patients with severe unexplained inflammatory syndrome or FUO and inconclusive conventional imaging or to discriminate active from silent lesions previously detected by conventional imaging particularly when malignancy is suspected.
Abscess ; Adenocarcinoma ; Diagnosis ; Electrons ; Endocarditis ; Fever of Unknown Origin ; Follow-Up Studies ; Graft vs Host Disease ; Hand ; Heart Transplantation ; Herpesvirus 4, Human ; Histoplasmosis ; Humans ; Kidney ; Lung ; Lung Diseases, Fungal ; Lymphoma ; Organ Transplantation ; Pneumonia, Bacterial ; Positron-Emission Tomography and Computed Tomography ; Retrospective Studies ; Sensitivity and Specificity ; Sepsis ; Seroconversion ; Transplants

Familial Mediterranean fever (FMF) is the most common Mendelian autoinflammatory disease, characterized by uncontrolled activation of the innate immune system that manifests as recurrent brief fever and polyserositis (e.g., peritonitis, pleuritic, and arthritis). FMF is caused by autosomal recessive mutations of the Mediterranean fever gene, MEFV which encodes the pyrin protein. Although FMF predominantly affects people from Mediterranean and Middle Eastern ethnic origins, 3 cases of FMF have been reported in Korea since 2012. We report another case of FMF in Korea in which the patient presented with a month-long fever without serositis. After treatment with colchicine was initiated, the patient’s symptoms quickly subsided. The response to colchicine was helpful for diagnosis. We compare the FMF genotypes in Korea with in other countries. Studying FMF cases in Korea will help establish the best MEFV exons to use for screening and diagnosis of Korean FMF.
Colchicine ; Diagnosis ; Exons ; Familial Mediterranean Fever* ; Fever of Unknown Origin* ; Fever* ; Genotype ; Humans ; Immune System ; Korea* ; Mass Screening ; Peritonitis ; Serositis

A 61-year-old man was referred to our hospital due to a 3-month history of fever of unknown origin, and with right knee and ankle joint pains. At another hospital, extensive investigations had produced negative results, including multiple sterile cultures of blood and joint fluids, and negative autoantibodies. His serum uric acid level was not elevated. However, after admission to our hospital, we performed right knee arthrocentesis, which revealed uric acid crystals. These findings, combined with the results of imaging tests, which showed joint degeneration, led to a diagnosis of advanced erosive gout. After receiving a therapeutic non-steroidal anti-inflammatory drug and a maintenance dose of colchicine for prophylaxis against recurrence, the patient's symptoms subsided and did not return. Advanced erosive gout should be considered a possible cause of fever of unknown origin and diagnostic arthrocentesis should be performed in patients with unexplained arthritis.
Ankle Joint ; Arthritis ; Autoantibodies ; Colchicine ; Diagnosis ; Fever of Unknown Origin* ; Gout* ; Humans ; Joints ; Knee ; Middle Aged ; Recurrence ; Uric Acid

Extra-nodal malignant lymphoma is often characterized by a lack of typical symptoms and positive results of auxiliary examinations, which make diagnosis difficult. In some cases, fever can be the only clinical manifestation. For the lymphoma patients presenting with persistent fever with a duration over 3 weeks, characteristics of fever including time of fever attack, fever type and effects of drugs may have significant value in the diagnosis, especially in the early stage of the disease or in rare cases.
Fever of Unknown Origin ; Humans ; Lymphoma ; diagnosis ; Muscles ; pathology

OBJECTIVETo study the clinical value of combined measurement of C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), lactate dehydrogenase (LDH), and serum ferritin (SF) in the etiological diagnosis of fever of unknown origin (FUO) in children.

METHODSThe clinical data of 154 hospitalized children who had FUO for at least two weeks were retrospectively analyzed, and they were classified into infection (n=54), rheumatism (n=67), and tumor (n=33) groups according to the diagnosis at discharge. The levels of CRP, ESR, LDH, and SF were compared between the three groups, and the diagnostic values of the four indices alone or together were analyzed using the receiver operating characteristic (ROC) curve.

RESULTSSerum CRP and ESR levels were elevated in all the three groups, and increased most significantly in the rheumatism group. Serum LDH level was increased most significantly in the tumor group. SF level was significantly increased in the rheumatism and tumor groups. The area under the ROC curve (AUC) of LDH for diagnosing rheumatism and the AUC of ESR and CRP for diagnosing tumors were lower than 0.7 (P>0.05). The AUC of CRP for diagnosing infection and rheumatism was 0.861 and 0.782, respectively. The AUC of ESR for diagnosing infection and rheumatism was 0.770 and 0.743, respectively. LDH had relatively low AUC, sensitivity, specificity, and Youden's index in diagnosing infection and tumors. SF had the highest AUC, sensitivity, and Youden's index in diagnosing infection, but had the lowest specificity. SF had relatively high AUC, sensitivity, specificity, and Youden's index in diagnosing rheumatism, but had relatively low AUC in diagnosing tumor. The four indices had higher AUC, sensitivity, and specificity in diagnosing rheumatism and tumors when measured together than when measured alone.

CONCLUSIONSIn the etiological diagnosis of FUO in children, CRP, ESR, LDH, and SF have certain clinical significance in the preliminary diagnosis of rheumatic diseases, but have limited value in the diagnosis and differentiation of infectious diseases and malignant tumors. Combined measurement of the four indices is superior to the determination of each one for the etiological diagnosis of FUO in children.

Adolescent ; Animals ; Area Under Curve ; Blood Sedimentation ; C-Reactive Protein ; analysis ; Child ; Female ; Ferritins ; blood ; Fever of Unknown Origin ; blood ; diagnosis ; Humans ; Infant ; L-Lactate Dehydrogenase ; blood ; Male ; Rabbits