Adult onset Still's disease is a rare inflammatory disease characterized by spiking fevers, arthritis/ arthralgias, typical salmon-colored bumpy rash, pharyngalgia, myalgia and possible involvement of visceral organs. The diagnosis is exclusively based on clinical symptoms, according to the criteria, after the exclusion of well-known infectious, neoplastic, or other autoimmune/autoinflammatory disorders. This report includes one case of adult onset Still's disease with the initial symptom of pharyngalgia.
Adult ; Humans ; Pharyngitis ; Rare Diseases ; Still's Disease, Adult-Onset ; diagnosis

OBJECTIVE: Adult onset Still s disease is an acute systemic inflammatory disorder. There are no pathognomonic symptoms or specific laboratory abnormalities. In recent reports, serum ferritin concentration is increased in active disease phase and decreased after defervescence. Our purpose was to determine the clinical significance of serum ferritin as an indicator for disease activity. METHODS: Seven patients who were diagnosed as adult onset Still s disease at Samsung Medical Center between October 1994 and March 1997, were reviewed. In these patients we checked leukocyte count, ESR, CRP and serum ferritin concentrations at the time of diagnosis and during follow-up periods and recorded febrile events during follow-up periods. RESULTS: At the time of diagnosis and during febrile periods, the concentrations of ferritin were extremely high(927ng/ml to 96,650ng/ml normal 10-290.8 ng/ml). The values were unrelated to other manifestations of the disease or laboratory findings. The ferritin concentrations decreased rapidly after adequate treatment. Eleven febrile reattacks happened in 7 patients. Serum ferritin concentrations were increased in 8 febrile attacks, while leukocyte count, ESR, and CRP were increased in 5, 5, 6 febrile attacks respectively, There were 10 events of increased serum ferritin concentrations in 7 patients during follow-up periods and 8 events were related with fever. The increases of other laboratory tests were similar. CONCLUSIONS: In all patients, serum ferritin concentrations were increased at the time of diagnosis and closely related to fever. During follow-up periods, serum ferritin concentrations are helpful in monitoring disease activity and guiding decisions about treatment.
Adult* ; Diagnosis ; Ferritins* ; Fever ; Follow-Up Studies ; Humans ; Leukocyte Count ; Still's Disease, Adult-Onset*

Adult onset Still 's disease (AOSD)is a rare,distinct clinical entity which affects predominantly young adults aged 16-35.It affects multiple organs,and at present,the etiology is still unclear.Because this disease has few diagnostic or confirmative test,the diagnosis is made by the differential diagnosis and by excluding other diseases.Several reports have suggested a viral trigger in the pathogenesis of this disease.It has some common clinical features ;abrupt onset, high fever,sore throat,transient maculopapular rash,lymphadenopathy,and hepatosplenomegaly.We describe a 17-year-old man who fulfilled the proposed diagnostic criteria of AOSD and had evidence of acute Epstein-Barr virus infection.He complained of intermittent high fever and myalgia for a week.He also had maculopapular rash,sore throat,multiple right cervical lymphadenopahty, and right ankle pain and swelling.After admission,intermittent fever persisted for a month,but diffuse myalgia and migrating arthralgia fluctuated.
Adolescent ; Adult* ; Ankle ; Arthralgia ; Diagnosis ; Diagnosis, Differential ; Fever ; Herpesvirus 4, Human* ; Humans ; Myalgia ; Still's Disease, Adult-Onset* ; Young Adult

BACKGROUND: Human neutrophil lipocalin (HNL) has been used extensively to differentiate acute bacterial infection from febrile diseases as a biomarker to reflect the activation of the neutrophil. The serum HNL levels in the adult-onset Still's disease (AOSD) patients with and without infection, as well as the healthy controls (HCs), were analyzed statistically in this study to evaluate the value of HNL for the diagnosis of AOSD. METHODS: A total of 129 AOSD patients were enrolled, from whom blood samples were drawn and the AOSD diagnosis was confirmed through the review of the medical records, where the systemic score, demographic characteristics, clinical manifestations, and laboratory parameters were also collected for the patients; in addition, a total of 40 HCs were recruited among the blood donors from the healthcare center with the relevant information collected. The HNL test was done for the blood samples with the enzyme-linked immunosorbent assay and the analyses were done for the correlations of HNL with clinical manifestations and diagnostic effectiveness. RESULTS: The serum HNL increased significantly in the patients with only AOSD as compared with that in the HCs (139.76 ± 8.99 ng/mL vs . 55.92 ± 6.12 ng/mL; P  < 0.001). The serum HNL level was correlated with the white blood cell (WBC) count ( r  = 0.335, P  < 0.001), neutrophil count ( r  = 0.334, P  < 0.001), erythrocyte sedimentation rate ( r  = 0.241, P  = 0.022), C-reactive protein ( r  = 0.442, P  < 0.0001), and systemic score ( r  = 0.343, P  < 0.0001) in the AOSD patients significantly. Patients with fever, leukocytosis ≥15,000/mm 3 , and myalgia in the HNL-positive group were observed relatively more than those in the HNL-negative group ( P  = 0.009, P  = 0.023, and P  = 0.007, respectively). HNL was a more sensitive indicator than ferritin and C-reactive protein (CRP) to differentiate the AOSD patients with bacterial infection from AOSD-only patients, and the Youden index was 0.6 for HNL and 0.29 for CRP. CONCLUSION Serum HNL can be used as a biomarker for the diagnosis of the AOSD, and HNL is also observed to be associated with the disease activity.
Adult ; Humans ; Still's Disease, Adult-Onset/diagnosis* ; C-Reactive Protein/metabolism* ; Neutrophils/metabolism* ; Clinical Relevance ; Biomarkers ; Bacterial Infections

BACKGROUND: Due to advances in various diagnostic methods, recent studies reported changes in the pattern of etiology of fever of unknown origin (FUO). To identify the current pattern of the causes of FUO, we analyzed the etiology of recently diagnosed FUO at a university hospital in Korea. MATERIALS AND METHODS: We reviewed 69 cases that fulfilled the criteria of classic FUO and retrospectively analyzed the etiology and decisive methods of diagnosis. RESULTS: The etioloies of FUO were infectious disease, non-infectious inflammatory disease, malignancy and miscellaneous cases in 22 (31.9%), 8 (11.6%), 4 (2.3%) and 21 (30.4%) patients, respectively. In 15 (21.7%) cases the cause could not be identified. Among infectious diseases, tuberculosis and suspected typhoid fever were the most common causes of infection (8 case, 11.7%) with tuberculosis being the most common confirmed infection. Adult onset Still's disease (13 cases, 4.4%) and drug-related fever (13 cases, 18.8%) were the most common cause of non-infectious inflammatory disease and miscellaneous causes, respectively. Decisive methods of final diagnosis were by observation of clinical course in 35 (64.8%), radiologic examination in 10 (18.5%), serologic or biochemical test in 5 (9.3%) and tissue biopsy in 4 (7.4%); none were diagnosed by culture. CONCLUSION: Infection remains the most common etiology of classic FUO in Korea and observing the clinical course is the most commonly used method for decisive diagnosis and its importance should be emphasized in approaching patients with FUO.
Adult* ; Biopsy ; Communicable Diseases ; Diagnosis ; Fever of Unknown Origin* ; Fever* ; Humans ; Korea* ; Retrospective Studies ; Still's Disease, Adult-Onset ; Tuberculosis ; Typhoid Fever

BACKGROUND: Due to advances in various diagnostic methods, recent studies reported changes in the pattern of etiology of fever of unknown origin (FUO). To identify the current pattern of the causes of FUO, we analyzed the etiology of recently diagnosed FUO at a university hospital in Korea. MATERIALS AND METHODS: We reviewed 69 cases that fulfilled the criteria of classic FUO and retrospectively analyzed the etiology and decisive methods of diagnosis. RESULTS: The etioloies of FUO were infectious disease, non-infectious inflammatory disease, malignancy and miscellaneous cases in 22 (31.9%), 8 (11.6%), 4 (2.3%) and 21 (30.4%) patients, respectively. In 15 (21.7%) cases the cause could not be identified. Among infectious diseases, tuberculosis and suspected typhoid fever were the most common causes of infection (8 case, 11.7%) with tuberculosis being the most common confirmed infection. Adult onset Still's disease (13 cases, 4.4%) and drug-related fever (13 cases, 18.8%) were the most common cause of non-infectious inflammatory disease and miscellaneous causes, respectively. Decisive methods of final diagnosis were by observation of clinical course in 35 (64.8%), radiologic examination in 10 (18.5%), serologic or biochemical test in 5 (9.3%) and tissue biopsy in 4 (7.4%); none were diagnosed by culture. CONCLUSION: Infection remains the most common etiology of classic FUO in Korea and observing the clinical course is the most commonly used method for decisive diagnosis and its importance should be emphasized in approaching patients with FUO.
Adult* ; Biopsy ; Communicable Diseases ; Diagnosis ; Fever of Unknown Origin* ; Fever* ; Humans ; Korea* ; Retrospective Studies ; Still's Disease, Adult-Onset ; Tuberculosis ; Typhoid Fever

PURPOSE: To investigate clinical implications of delta neutrophil index (DNI) to discriminate adult onset Still's disease (AOSD) from sepsis. MATERIALS AND METHODS: We reviewed the medical records of 13 patients with AOSD and 33 gender and age-matched patients with sepsis. In all subjects, microbial tests were performed to exclude or confirm sepsis. All laboratory data were measured two or three times during the first 3 days and represented by their mean levels. DNI was measured automatically by ADVIA 2120 for the first 3 days. RESULTS: There were no significant differences in white blood cell counts, neutrophil proportion, erythrocyte sedimentation rate and C-reactive protein between two groups. AOSD patients had notably lower DNI than sepsis patients regardless of the presence of bacteremia or not. However, both DNI and ferritin were not significant independent factors for predicting sepsis in the multivariate logistic regression analysis. Meanwhile, the area under the receiver operating characteristic curve (AUROC) of DNI was slightly higher than that of ferritin. When we set DNI of 2.75% as the cut-off value for predicting sepsis, 11 (84.6%) of AOSD patients had a DNI value below 2.75% and 2 (15.4%) of them had a DNI over 2.75% (relative risk for sepsis 176). CONCLUSION: We suggest that DNI may be a useful marker for differential diagnosis of AOSD from sepsis in the early phase as supplementary to ferritin.
Adult ; Biological Markers/*metabolism ; Diagnosis, Differential ; Female ; Humans ; Male ; Middle Aged ; Neutrophils/*metabolism ; Retrospective Studies ; Sepsis/*diagnosis/metabolism ; Still's Disease, Adult-Onset/*diagnosis/metabolism

OBJECTIVE: To analyze and compare the clinical and laboratory characteristics of macrophage activation syndrome (MAS) in patients with systemic lupus erythematosus (SLE) and adult-onset Still's disease (AOSD), and to evaluate the applicability of the 2016 European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organization classification criteria for MAS complicating systemic juvenile idiopathic arthritis (sJIA) in different auto-immune diseases contexts and to propose new diagnostic predictive indicators. METHODS: A retrospective analysis was conducted on the clinical and laboratory data of 24 SLE patients with MAS (SLE-MAS) and 24 AOSD patients with MAS (AOSD-MAS) who were hospitalized at Peking University People's Hospital between 2000 and 2018. Age- and sex-matched SLE (50 patients) and AOSD (50 patients) diagnosed in the same period without MAS episodes were selected as controls. The cutoff values for laboratory indicators predicting SLE-MAS and AOSD-MAS were determined using receiver operating characteristic (ROC) curves. Furthermore, the laboratory diagnostic predictive values for AOSD-MAS were used to improve the classification criteria for systemic juvenile idiopathic arthritis-associated MAS (sJIA-MAS), and the applicability of the revised criteria for AOSD-MAS was explored. RESULTS: Approximately 60% of SLE-MAS and 40% of AOSD-MAS occurred within three months after the diagnosis of the underlying diseases. The most frequent clinical feature was fever. In addition to the indicators mentioned in the diagnosis criteria for hemophagocytic syndrome revised by the International Society for Stem Cell Research, the MAS patients also exhibited significantly elevated levels of aspartate aminotransferase and lactate dehydrogenase, along with a significant decrease in albumin. Hemophagocytosis was observed in only about half of the MAS patients. ROC curve analysis demonstrated that the optimal discriminative values for diagnosing MAS was achieved when SLE patients had ferritin level≥1 010 μg/L and lactate dehydroge-nase levels≥359 U/L, while AOSD patients had fibrinogen levels≤225.5 mg/dL and triglyceride levels≥2.0 mmol/L. Applying the 2016 sJIA-MAS classification criteria to AOSD-MAS yielded a diagnostic sensitivity of 100% and specificity of 62%. By replacing the less specific markers ferritin and fibrinogen in the 2016 sJIA-MAS classification criteria with new cutoff values, the revised criteria for classifying AOSD-MAS had a notable increased specificity of 86%. CONCLUSION Secondary MAS commonly occurs in the early stages following the diagnosis of SLE and AOSD. There are notable variations in laboratory indicators among different underlying diseases, which may lead to misdiagnosis or missed diagnosis when using uniform classification criteria for MAS. The 2016 sJIA-MAS classification criteria exhibit high sensitivity but low specificity in diagnosing AOSD-MAS. Modification of the criteria can enhance its specificity.
Adult ; Humans ; Child ; Macrophage Activation Syndrome/complications* ; Arthritis, Juvenile/diagnosis* ; Still's Disease, Adult-Onset/diagnosis* ; Retrospective Studies ; Lupus Erythematosus, Systemic/diagnosis* ; Fibrinogen ; Ferritins

BACKGROUND: Patients with fever and rash often pose an urgent diagnostic and therapeutic dilemma for the clinician. The nonspecificity of many fever and rash syndromes mandates a systemic approach to diagnosis. OBJECTIVE: We aimed to determine the etiology of fever and rash in 100 adult patients followed-up as in- or outpatients prospectively. METHODS: All the patients, who presented with rash and fever, were followed-up prospectively and their clinical and laboratory studies were evaluated. RESULTS: The median age was 35 years (14~79 years); 45 were female and 55 were male. Patients were divided into 3 groups according to the etiology: infectious (50%), noninfectious (40%) and undiagnosed (10%). The most common type of rash was maculopapular, and the most common 5 causes were measles, cutaneous drug reactions, varicella, adult-onset Still's disease (ASD) and rickettsial disease. Viral diseases among infectious causes and cutaneous drug reactions, among the noninfectious causes, were determined as the main diseases. The mortality rate was 5% and the reasons of mortality were as follows: toxic epidermal necrolysis (2 patients), ASD (1), staphylococcal toxic shock syndrome (1) and graft-versus-host disease (1). CONCLUSION: Adult patients with fever and rash had a wide differential diagnosis. The most common type of rash was determined as maculopapular, and the most frequent five diseases were measles, drug reactions, chickenpox, ASD and rickettsial infection. Viral diseases among infectious causes and drug reactions among noninfectious causes were determined as the leading etiologies.
Adult ; Chickenpox ; Diagnosis, Differential ; Epidermal Necrolysis, Toxic ; Exanthema ; Female ; Fever ; Graft vs Host Disease ; Humans ; Male ; Measles ; Outpatients ; Prospective Studies ; Shock, Septic ; Still's Disease, Adult-Onset ; Virus Diseases

Fever of unknown etiology that occurs along with highly elevated serum ferritin concentrations (>500 ng/mL) is mostly observed in hematologic malignancies and rheumatic diseases such as systemic lupus erythematosus, temporal arteritis, and adult-onset Still's disease (AOSD), among which AOSD is a type of systemic inflammatory disorder with unknown pathophysiology and has very low incidence. AOSD presents with various nonspecific symptoms and signs such as high spiking fever, joint pain, skin rash, and increased leukocytes. Because AOSD is diagnosed after excluding the possibility of other conditions such as neoplasms, infections, and inflammations, diagnosis and treatment are generally delayed. Patients with AOSD often have high serum ferritin levels than those with other conditions, although the underlying mechanism for this is not clearly understood. In addition, decreased proportion of glycosylated ferritin are observed in most patients with AOSD. Therefore a combination of high serum ferritin and a decreased proportion of glycosylated ferritin seems to be important for the differential diagnosis of AOSD that thus may allow early diagnosis of AOSD. Here we report a case of AOSD diagnosed via extremely high serum ferritin levels and decreased glycosylated ferritin proportion.
Arthralgia ; Diagnosis, Differential ; Early Diagnosis ; Exanthema ; Ferritins ; Fever ; Giant Cell Arteritis ; Hematologic Neoplasms ; Humans ; Incidence ; Inflammation ; Leukocytes ; Lupus Erythematosus, Systemic ; Rheumatic Diseases ; Still's Disease, Adult-Onset