Clinical characteristics and diagnostic indicators of macrophage activation syndrome in patients with systemic lupus erythematosus and adult-onset Still's disease / 北京大学学报(医学版)

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.

The role of platelet to mean platelet volume ratio in the identification of adult-onset still's disease from sepsis

OBJECTIVES: Inflammatory factors exert a significant role in the development of adult-onset Still's disease (AOSD) and sepsis. Although platelet counts and platelet parameters have long served as indicators for inflammatory diseases, their role in the differential diagnosis between adult-onset stilĺs disease and sepsis remains unclear. We designed this retrospective study to explore whether the platelet to mean platelet volume (MPV) ratio (PMR) can help to distinguish AOSD from sepsis. METHODS: A total of 110 AOSD patients and 84 sepsis patients were enrolled in the study. Seventy-three AOSD patients and 56 sepsis patients between January 2010 and June 2017 were enrolled in the test cohort to analyze PMR values, which was then validated in the validation cohort (37 AOSD patients and 28 sepsis patients between June 2017 and December 2019). RESULTS: The values of PMR were significantly higher in AOSD patients than in sepsis patients (test cohort, validation cohort, and entire cohort), In the test cohort, logistic regression analysis showed that PMR was an independent risk factor of AOSD (odds ratios [OR]: 9.22, 95% confidence interval [CI] 2.15-39.46, p=0.003). Further receiver operating characteristic curve (ROC) analysis showed that the area under the ROC curve was 0.735 (95% CI 0.631-0.839, p<0.001) for PMR alone and 0.925 (95% CI 0.869-0.980, p<0.001) for the combination of PMR and serum ferritin. Consistently, the validation cohort exhibited analogous results. CONCLUSIONS: PMR could be used as a single indicator or a complementary indicator to distinguish AOSD from sepsis.

Adult-onset Still's disease successfully treated with Chinese herbal medicine: A case report with 15-month follow-up / 中西医结合学报

Adult-onset Still's disease (AOSD) is a rare but clinically well-known, polygenic, and systemic autoinflammatory disease, which is characterized by spiking fever, evanescent skin rash, arthralgia, and sore throat. The application of non-steroidal anti-inflammatory drugs and glucocorticoids, which are first-line therapies of AOSD, is limited due to their side effects such as liver injury or disorder of blood glucose. Therefore, patients who suffer from systemic diseases in China prefer to seek help from Chinese herbal medicine (CHM), which is an important part of complementary and alternative medicine. In this case, we report a 28-year-old male badminton coach presenting with a 15-day history of fever and skin rash, accompanied by sore throat, fatigue, myalgia and chills. Additionally, hepatosplenomegaly, multiple lymphadenopathies, aminotransferase abnormality, and elevated inflammatory factor levels were observed during hospitalization. Infectious diseases, solid tumors, hematological diseases, and common autoimmune diseases were excluded. Not benefitting from antibiotic therapy, the patient was finally diagnosed with AOSD, after a careful examination, then showed rapid remission after a six-week treatment with CHM granules based on Xiaochaihu Decoction and Yinqiao Powder. After stopping the treatment, there was no relapse within a 15-month follow-up period. To the best of our knowledge, this is the first well-documented case of this successful treatment. The present case report suggests that CHM is a reliable choice for complementary and alternative therapy for AOSD, but confirming the utility of CHM for AOSD requires further support from prospective studies.

Rapid responses to golimumab in refractory adult-onset Stills disease: A case report and review of the literature

ABSTRACT Introduction: Adult onset Still's disease (AOSD) is a systemic inflammatory disorder of unknown etiology. AOSD is generally a mild and self-limiting disease, but it could progress to become chronic. The anemia of chronic diseases may occur in patients with acute or chronic immune activation, and is associated with the production of pro-inflammatory cytokines. Case report: A 61-year-old woman with several pharyngitis episodes, spiking fever, evanescent salmon-pink skin rash, normocytic normochromic anemia, leucocytosis, thrombocytopenia, polyarthritis, liver dysfunction, marked elevated erythrocyte sedimentation rate and C-reactive protein and, notably high ferritin levels. AOSD was diagnosed after secondary diseases were ruled out. Despite eight month on treatment with high-dose corticosteroids and methotrexate the clinical course the patient worsened, with significant synovitis, joint deformities leading to a worse quality of life and requiring help with activities of daily living. A rapid response to the anti-TNFα golimumab (50 mg/month) was observed from the third month of treatment. Conclusion: Golimumab improved anemia, serum C-reactive protein levels, polyarthritis and quality of life in a refractory AOSD.

Adult onset still’s disease: a diagnostic dilemma

Adult onset still’s disease is a rare systemic inflammatory disorder of unknown aetiology, characterized by clinical triad (high spiking fever, evanescent rash and arthritis) and biological triad with lack of serological markers as a true gold standard makes diagnosis difficulty. Here is a case of 32year old male presented with high grade fever for 2 months, joint pains and swelling for 1month rash for 3 days. O/E: pallor and B/L tender, swollen ankle and knee joints, and P/A: splenomegaly. Investigations showed-Hb%. 8 gms, neutrophilic leucocytosis with thrombocytosis, ESR:72 mm/1h, CRP elevated. ASO-titre, RA factor and Anti CCP antibodies are negative. Adult onset still’s disease is a heterogenous and rare systemic inflammatory disorder of unknown aetiology with lack of serological diagnostic modalities.

Red blood cell distribution width is useful in discriminating adult onset Still's disease and sepsis within 24 hours after hospitalization

BACKGROUND/AIMS: Red blood cell distribution width (RDW) is a value representing the heterogeneity in the size of red blood cell, and it is usually used in distinguishing types of anaemia. Recently, it was reported that it could reflect the burden of inflammation in diverse diseases and their prognosis. Hence, in this study, we investigated whether RDW may contribute to discriminating adult onset Still’s disease (AOSD) from sepsis in serious febrile patients within 24 hours after hospitalization. METHODS: We reviewed the medical records and enrolled 21 AOSD patients, 27 sepsis patients and 30 matched healthy controls. We collected at least two laboratory results of variables including RDW within 24 hours after hospitalization, and we calculated their mean values. RESULTS: Sepsis patients showed the significantly increased median white blood cell count, compared to AOSD patients (14,390.0/mm3 vs. 12,390.0/mm3 , p = 0.010). The median RDW in sepsis patients was higher than that in AOSD patients (15.0% vs. 13.3%, p = 0.001), and furthermore, the median RDW in both patient-groups was significantly higher than that in healthy controls. In contrast, the median ferritin level in sepsis patients was lower than that in AOSD patients (544.0 mg/dL vs. 3,756.6 mg/dL, p = 0.001). In multivariate analysis, RDW ≥ 14.8% (odds ratio, 17.549) and ferritin < 2,251.0 mg/dL (odds ratio, 32.414) independently suggested sepsis more than AOSD in patients initially presenting with fever requiring hospitalization. CONCLUSIONS: RDW might be a rapid and helpful marker for a differential diagnosis between AOSD from sepsis at an early phase.

Adult-onset Still's Disease as the First Manifestation of Cerebral Infarction: a Case Report

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.

Enfermedad de Still del Adulto, a propósito de un caso / Still's disease, a clinical case

La enfermedad de Still del Adulto es una variante sistémica del universo llamado "artritis idiopática juvenil", cuya diferencia es la edad de aparición, recibiendo este nombre cuando el cuadro se manifiesta en personas mayores de 15 años. Es una enfermedad autoinflamatoria caracterizada por fiebre intermitente, artritis, rash evanescente y linfadenopatías, asociado a manifestaciones de compromiso sistémico. Es una patología de baja prevalencia, pero es importante considerarla como uno de los principales diagnósticos etiológicos de la fiebre de origen desconocido.

Adult-onset Still's disease is a systemic variant of Still's disease distinguished from juvenile idiopathic arthritis by its onset age of over 15. It is an auto-inflammatory disease characterized by intermittent fever, arthritis, short-lived rash and lymphadenopathies. It has low prevalence but is important to consider as one of the principle etiological diagnoses of fever of unknown origin.

Diagnosis and treatment for one case of elderly diabetes complicated with Still's disease / 中南大学学报(医学版)

Adult onset Still's disease (AOSD) is a clinical syndrome with multiple organ failure.The patients normally show intermittent high fever for a long time,a transient rash,arthritis or joint pain as the main performance,accompanied by an increase in granulocytes and enlargement in liver,spleen and lymph node.A 71-years-old female patient with type 2 diabetes admitted hospital because of high fever,skin rash,joint pain and increased granulocyte.After review of the iron protein,she was diagnosed as AOSD.We found that clinicians need to improve the understanding for this disease in order to make the early diagnosis,especially in elderly patients with diabetes mellitus.In such patients,ferritin may not be high at early time.However,when the symptoms and signs are consistent with clinical manifestations,and anti-infection treatment effect is poor,we should pay attention to the disease,and repeated review of ferritin is necessary to assist the early diagnosis.

Clinical characteristics of patients with macrophage activation syndrome secondary to adult-onset Still's disease / 上海交通大学学报（医学版）

Objective· To investigate the clinical features of macrophage activation syndrome (MAS) associated with adult-onset Still's disease (AOSD),and provide the basis for clinical diagnosis and treatment of the disease.Methods· The clinical data of 42 patients with AOSD,including 14 patients with AOSD-induced MAS (the MAS group) and 28 AOSD patients paired by age and sex (the non-MAS group),diagnosed in Department of Rheumatology,Renji Hospital,Shanghai Jiao Tong University School of Medicine from October 2013 to June 2016 were collected and then retrospectively analyzed.Results· There was no significant difference in age,sex and duration of AOSD between two groups.The mortality rate of patients in MAS group was significantly higher than that of patients in non-MAS group,as well as the rates of rash,splenomegaly and hemophagocytosis.The levels of ALT and serum ferritin in MAS group were higher than those in non-MAS group,while the level of FDP is lower.Glucocorticoids were used in all 42 patients,and the dosage of glucocorticoids was significantly higher in MAS group than non-MAS group.Only 1 patient with AOSD-induced MAS received MTX,the percentage of patients receiving MTX was significantly lower in MAS group than non-MAS group.Five patients with AOSD-induced MAS received IVIG,the percentage of patients receiving IVIG was significantly higher in MAS group than non-MAS group.Two patients with AOSD-induced MAS received VP-16.Conclusion · The mortality rate of patients in MAS group was significantly higher than that of patients in non-MAS group,as well as the rates of rash,splenomegaly and hemophagocytosis.The levels of ALT and serum ferritin in patients with AOSD-induced MAS were higher than patients without MAS,while the level of FDP was lower.Early diagnosis and active treatment is the key point to improve clinical outcome.

Adult‑onset Still’s disease with myocarditis and hemophagocytic lymphohistiocytosis: Rare manifestation with fatal outcome.

Adult‑onset Still’s disease (AOSD) is a rare inflammatory disorder of unknown etiology characterized by fever, evanescent pink salmon rash, arthritis, and multiorgan involvement. Here, we report an unusual manifestation of AOSD in a 40‑year‑old male who presented to our hospital with pyrexia of unknown origin and rash of 3 weeks duration. All his serological investigations and imaging studies were unremarkable. He was fulfilling clinical and laboratory criteria as per Yamaguchi for AOSD and was managed for the same. Our patient did not respond well to the treatment, had a downhill course, and succumbed to his illness. Autopsy confirmed myocarditis and florid bone marrow reactive hemophagocytosis as the cause of his death.

A Case of Atypical Adult-onset Still's Disease That Presented with Unique Histopathologic Findings / 대한피부과학회지

Adult-onset Still's disease (AOSD) is an uncommon disorder characterized by fever, polyarthralgia, elevated white blood cell count, and maculopapular rash, the histologic features of which have not been well known. A 54-year-old woman presented with fever, arthritis, and persistent pruritic lichenified rash. These cutaneous lesions showed unique histological features such as dyskeratosis with a peculiar, distinctive distribution in the upper epidermis and cornified layers with hyperkeratosis. In addition, dermal mucin deposition was increased, with inflammation and minimal fibroblast proliferation. The patient was diagnosed with AOSD according to the diagnostic criteria of Yamaguchi. Herein, we present an atypical form of AOSD showing distinctive clinical and pathological manifestations.

Adult Onset Stills Disease: A Case Report.

Adult onset still’s disease (AOSD) is a rare inflammatory disorder of unknown etiology. Because of lack of a defined diagnostic test the diagnosis of AOSD can only be made after excluding infectious, malignant, and autoimmune diseases. A 21-year-old female patient was admitted in our hospital with intermittent high-grade fever, polyarthritis and rash. On further history, examination and laboratory investigation she was found to be fulfilling all the Yamaguchi criteria for AOSD. All other causes consisting of acute or chronic infections, hematological malignancies, and other rheumatic disorders were excluded by laboratory investigations. Patient was treated as AOSD with non-steroidal anti-inflammatory drugs and steroids and is completely free of symptoms.

Adult Onset Still's Disease Developed in Chronic Urticaria Patient / 대한피부과학회지

No abstract available.

A Case of Adult Onset Still's Disease with Severe Pneumonitis Treated with Anakinra / 대한내과학회지

Adult onset Still's disease (AOSD) is a systemic autoinflammatory disorder that presents with recurrent fever, extreme fatigue, and joint pain. Pulmonary involvement is not uncommon and, although rare, severe pneumonitis can progress to respiratory failure. Still's disease-associated pneumonitis is generally treated with immunosuppressive agents, but improvement in our understanding of systemic inflammatory processes led us to explore alternative agents. Anakinra is an interleukin-1 receptor antagonist used to treat autoinflammatory disorders resistant to immunosuppressive therapy. Several case reports have demonstrated efficacy of anakinra in treating AOSD, but its relevance in cases complicated with severe pneumonitis has not been examined. Our patient's disease activity was not controlled with systemic steroids and cyclophosphamide. Treatment with anakinra led to a dramatic clinical response. This is the first reported case of AOSD with severe pneumonitis refractory to conventional therapy successfully treated with anakinra.

Delta Neutrophil Index as an Early Marker for Differential Diagnosis of Adult-Onset Still's Disease and Sepsis

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.

Multiple Gastric Ulcers as a Manifestation of Cytomegalovirus Infection in a Patient with Adult-onset Still's Disease

Cytomegalovirus (CMV) is a relatively common viral pathogen, and CMV infection is generally assumed asymptomatic in general hosts. In immunologically compromised patients, CMV infection can cause further serious diseases such as pneumonitis, retinitis, encephalitis, and enterocolitis. A 40-year-old man is being presented with acute fever, myalgia, and sore throat. Laboratory findings have revealed elevated ESR, CRP, and ferritin levels. The patient was being treated for adult-onset Still's disease (AOSD). Three weeks later, although AOSD activity was under control, the patient began to complain about oral soreness, epigastric pain, and diarrhea. Endoscopy revealed multiple round ulcers with white patches in the esophagus and the stomach, sparing the colon. Anti-fungal agent is being administered but failed to bring improvements after 2 weeks of therapy. CMV infection is confirmed with pathology, antiviral agents were initiated after the ulcers subsided. Currently, clinical associations between CMV infection and AOSD are suggested. CMV infection may be considered as a differential diagnosis when multiple upper gastrointestinal ulcerative lesions develop within patients whom have been treated AOSD with immunosuppressive agents.

A Case of Adult-Onset Still's Disease Presenting with Periorbital Edema

Adult-onset Still's disease (AOSD) is a systemic inflammatory disorder of unknown etiology that is characterized by high-spiking fever, arthralgia, sore throat, and skin rash. The typical rash of AOSD is an evanescent, salmon-colored erythema, which is considered to be the major diagnostic criterion. Recently, other cutaneous manifestations of AOSD, such as persistent plaque and urticaria, have been reported. Here, we report a rare case of AOSD presenting with periorbital swelling and erythema. A 47-year-old woman was presented with periorbital swelling, erythema, high fever, arthritis, and a sore throat. One year prior to admission, she was diagnosed with AOSD based on the diagnostic criteria of Yamaguchi. The patient's periorbital swelling and erythema may not have been associated with periorbital cellulitis because they did not respond to antibiotics but did improve after treatment with steroids. Considering all of her signs and symptoms with a history of AOSD, periorbital lesion was suspected as atypical cutaneous manifestation of AOSD.

A Case Report of Renal Involvement in Adult-Onset Still's Disease over Age 70

Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disease characterized by spiking fever, arthralgia, salmon pink rash, neutrophilic leukocytosis, and multi-organ involvement. Although renal involvement may appear in some cases of adult Still's disease, onset over 70 years of age with renal involvement has not been described. We report a 73-years-old woman whose illness manifested with fever of unknown origin, massive proteinuria, and multiple lymph nodes enlargement. With proteinuria of 2,650 mg/day, a renal biopsy was performed, and histopathological evaluation yielded the diagnosis of chronic glomerulonephritis (CGN). After excluding infectious disease, malignancy, and other rheumatic disease, AOSD was diagnosed with symptoms including fever over 39.0degrees C for more than a week, leukocytosis, generalized lymphadenopathy, and negative autoantibodies. Proteinuria and fever were improved markedly by high dose glucocorticoids and methotrexate therapy.

A Case of Adult Onset Still's Disease / 대한피부과학회지

No abstract available.