Adult-onset Still's disease after ChAdOx1 nCoV-19 vaccine: a possible association

ABSTRACT With emergent Sars-Cov-2, a highly transmissive virus that caused millions of deaths worldwide, the development of vaccines became urgent to combat COVID-19. Although rare, important adverse effects had been described in a hypothetical scenario of immune system overstimulation or overreaction. Still's disease is a rare inflammatory syndrome of unknown etiology. It manifests as a cytokine storm, mainly IL-18 and IL-1β, and presents itself with fever spikes, joint pain, maculopapular evanescent salmon-pink skin rash, and sore throat, among other symptoms. Here, we report a case of a 44-year-old healthy male who developed adult-onset Still's disease (AOSD) with atypical symptoms after both doses of ChAdOx1 nCoV-19 vaccine with 3 months of dose interval. The medical team suspected Still's disease and started prednisone 1 mg/kg (40mg). The next day the patient showed a marked improvement in articular and chest pains and had no other fever episodes. Therefore, he was discharged to continue the treatment in outpatient care. On the six-month follow-up, the patient was free of complaints, and the progressive corticoid withdrawal plan was already finished.

Recommendations of diagnosis and treatment of adult-onset Still′s disease in China / 中华内科杂志

Adult-onset Still′s disease (AOSD) is a rare systemic autoinflammatory disorder. In China, standardized diagnosis and treatment for AOSD is insufficient. Based on the evidence from China and other countries, Chinese Rheumatology Association developed standardization of diagnosis and treatment of AOSD in China. The purpose is to standardize the methods for diagnosis of AOSD, treatment strategies, and reduce misdiagnosis, missed diagnosis and irreversible damage.

Role of mean platelet volume in differential diagnosis of adult-onset Still's disease and sepsis

SUMMARY OBJECTIVES: Mean platelet volume is a simple biomarker for inflammatory disease. The purpose of this study is to evaluate the role of mean platelet volume in distinguishing adult-onset Still's disease from sepsis. METHODS: We retrospectively selected 68 patients with adult-onset Still's disease and 55 patients with sepsis between January 2015 and December 2019. Related laboratory data were collected and analyzed. RESULTS: There were no significant differences in white blood cell counts, neutrophils, lymphocytes, and C-reactive protein between adult-onset Still's disease group and sepsis group. However, patients in adult-onset Still's disease group showed higher ferritin and platelets and lower mean platelet volume and platelet distribution width than those in sepsis group (p<0.01 for both). Receiver operating characteristic curve analysis was performed to distinguish adult-onset Still's disease and sepsis. The area under the curve of mean platelet volume was 0.761 (95%CI 0.673-0.849), with a sensitivity of 79.1%, a specificity of 63.3%, and a cutoff value of 10.9 fL. In contrast, the area under the curve of combined ferritin and mean platelet volume was 0.90l (95%CI 0.837-0.965), with higher sensitivity (82.8%) and specificity (96.2%). Therefore, mean platelet volume could be used as a supplementary indicator to distinguish adult-onset Still's disease from sepsis. CONCLUSION: We suggest that mean platelet volume could be used as a supplementary biomarker for differential diagnosis of adult-onset Still's disease and sepsis in addition to ferritin.

Adult onset still's disease (aosd): a rare condition with a classic clinical presentation. case report

ABSTRACT Introduction: Adult Onset Still's Disease (AOSD) is a rare systemic inflammatory disease of unclear etiology, with low incidence and prevalence among the general population. AOSD is a common cause of fever of unknown origin (FUO) in up to 20% of cases. Due to the scarce knowledge about this disease and its diagnosis, it is usually unrecognized in the differential diagnoses, worsening the prognosis and increasing complications in some patients. Case presentation: This is the case of a 32-year-old female patient with prolonged febrile illness, who did not respond to the antimicrobial treatments previously established. She was diagnosed with AOSD according to the Yamaguchi criteria after an extensive exclusion process. She was treated with first-line treatment with corticosteroids, achieving satisfactory results Conclusions: The diagnosis of AOSD is an exhaustive process. Regardless of the availability of cutting-edge diagnostic tools, the medical history of the patient and an adequate physical examination are the most important aspects to consider.

RESUMEN Introducción. La enfermedad de Still del adulto (ESA) es una enfermedad inflamatoria sistémica de baja incidencia y prevalencia en población general y cuya etiología aún no es clara. La ESA puede causar fiebre de origen desconocido hasta en el 20% de los casos, pero suele pasar inadvertida dentro de los diagnósticos diferenciales iniciales debido a su desconocimiento, lo que empeora el pronóstico y aumenta las complicaciones en los pacientes. Presentación del caso. Paciente femenina de 32 años con síndrome febril prolongado que no respondía a tratamientos antimicrobianos instaurados previamente y en quien, finalmente, se diagnosticó ESA aplicando los criterios clasificatorios de Yamaguchi. La mujer recibió tratamiento de primera línea con corticosteroides y obtuvo buenos resultados. Conclusiones. La ESA requiere un exhaustivo proceso para su diagnóstico, en el cual, a pesar de la disponibilidad de herramientas diagnósticas avanzadas, la verificación de la historia clínica y la realización de un adecuado examen físico son los aspectos más importantes a tener en cuenta.

Enfermedad de Still del adulto: características clínicas y pronóstico de una cohorte de pacientes colombianos / Adult Still disease: Clinical features and prognosis in a cohort of Colombian patients

Resumen Introducción. La enfermedad de Still del adulto (ESA) es una condición médica poco frecuente que puede presentar complicaciones graves; sin embargo, aún no se conocen con claridad sus factores de riesgo, sus factores pronósticos, ni los aspectos asociados a las recaídas y a la refractariedad a esteroides en individuos con esta condición. Objetivos. Describir el comportamiento clínico de la ESA y determinar los factores asociados a la refractariedad a esteroides, a recaídas y a complicaciones en pacientes con esta enfermedad. Materiales y métodos. Estudio retrospectivo de cohortes que incluyó 45 pacientes diagnosticados con ESA entre enero de 2007 y enero de 2017 en 2 hospitales de referencia. Se presentan medidas de resumen. Se realizaron dos análisis de regresión logística para identificar posibles factores que expliquen la refractariedad a esteroides, las recaídas y el desarrollo de complicaciones en la población de estudio. Resultados. La edad promedio de los participantes fue 42.13±15.8 años. De los 45 pacientes incluidos, 23 (51.1%) eran mujeres, a 42 (93.3%) se le prescribieron esteroides, y, de estos, 13 (32%) se consideraron refractarios a esteroides. Se presentaron complicaciones en 12 (26.7%) individuos y su frecuencia fue mayor en pacientes con temperatura máxima >39°C. Finalmente, 33 (73.3%) pacientes tuvieron un seguimiento mayor a 1 año, de los cuales, 17 (37.8%) presentaron recaídas, las cuales fueron más frecuentes en individuos refractarios a esteroides o con esplenomegalia. Conclusión. Los pacientes que recibieron cualquier tipo de terapia biológica fueron más refractarios a esteroides; por su parte, la refractariedad a esteroides y la esplenomegalia se asociaron a un mayor número de recaídas, y la temperatura >39°C se asoció a al desarrollo de complicaciones.

Abstract Introduction: Adult Still disease (ASD) is a rare disorder that can lead to serious complications; however, risk factors associated with the development of this disease, its prognostic factors, and aspects related to relapse and steroids refractoriness in individuals with this condition are not yet clearly known. Objectives: To describe the clinical behavior of ASD and determine factors associated with steroid refractoriness, relapse, and complications in patients with this condition. Materials and methods: A retrospective cohort study was conducted in 45 patients diagnosed with ASD between January 2007 and January 2017 in 2 reference hospitals. Summary statistics are presented. In addition, two logistic regression analysis were conducted in order to identify possible factors explaining steroid refractoriness, relapses, and the development of complications in the study population. Results: Participants' average age was 42.13 ± 15.8 years. Out of the 45 patients, 23 (51.1%) were women, 42 (93.3%) were prescribed steroids, and of these, 13 (32%) were considered as steroid refractory. Complications were observed in 12 individuals (26.7%), and their frequency was higher in patients whose maximum body temperature was higher than 39°C. Finally, 33 (73.3% patients had a follow-up greater than 1 year, of which 17 (37.8%) suffered relapses; in addition, relapses were more frequent in patients who were steroid refractory or those with splenomegaly. Conclusion: Patients who were prescribed any type of biological therapy were more steroid refractory. On the other hand, steroid refractoriness and splenomegaly were associated with a higher frequency of relapses, while having a body temperature >39°C was associated with developing complications.

Doença de Still do adulto: um desafio diagnóstico / Adult-onset Still's disease: a diagnostic challenge

A doença de Still do adulto é uma rara condição inflamatória, cujo diagnóstico é um desafio, por se tratar de diagnóstico de exclusão, após vasta investigação. Manifesta-se com febre alta diária, amigdalite não supurativa, artrite, rash evanescente, leucocitose e hiperferritinemia. O presente caso demonstra a doença de Still do adulto e sua vasta investigação, motivando a realização de revisão bibliográfica sobre inovações na fisiopatologia, no diagnóstico e no tratamento.

Adult onset Still's disease is a rare inflammatory condition, the diagnosis of which is a challenge, because it is a diagnosis of exclusion, and demands extensive investigation. It manifests with high daily fever, nonsuppurative tonsillitis, arthritis, evanescent rash, leukocytosis, and hyperferritinemia. The present case de­monstrates adult-onset Still's disease and its extensive inves­tigation, motivating literature review on innovations of its pathophysiology, diagnosis, and treatment.

Clinical features of DRESS， AOSD， HLH with liver injury: An analysis of 58 cases / 临床肝胆病杂志

ObjectiveTo investigate the clinical features of adult liver injury patients with drug reaction with eosinophilia and systemic symptoms (DRESS), adult-onset Still’s disease (AOSD) or hemophagocytic lymphohistiocytosis (HLH) and the correlation of the degree of liver injury with inflammatory indices and immune indices. MethodsRelated clinical data were collected from 58 patients with liver injury caused by the above three systemic inflammatory diseases who were admitted to Peking University First Hospital from January 2008 to April 2019, among whom 7 had DRESS, 29 had AOSD, and 22 had HLH. General information, liver biochemical parameters, inflammatory indices, and immune indices were collected before treatment. The t-test was used for comparison of normally distributed continuous data between two groups; a one-way analysis of variance was used for comparison between multiple groups, and the least significant difference t-test was used for further comparison between two groups. The Mann-Whitney U test was used for comparison of non-normally distributed continuous data between two groups; the Kruskal-Wallis H test was used for comparison between multiple groups, and the Bonferroni method was used for further comparison between two groups. Four inflammatory indices were compared between the groups with different alanine aminotransferase (ALT) levels (≤200 U/L or ＞200 U/L), and a Spearman correlation analysis was used to investigate the correlation of ferritin and immune indices with ALT level. ResultsThe median age of the patients with DRESS or AOSD was 38 years, and the median age of the patients with HLH was 34 years. DRESS patients were mostly male (5/7, 71%), while most of AOSD patients (20/29, 69%) and HLH patients (12/22, 55%) were female. For liver injury indices, there were no significant differences between the three groups in ALT peak, aspartate aminotransferase level, and alkaline phosphatase level (all P＞0.05). For the indices for the synthetic function of the liver, there were no significant differences in blood glucose, albumin, and prothrombin activity between the three groups (all P＞0.05). For inflammatory indicators, there were significant differences between the three groups in erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) (all P＜0.001), while there were no significant differences between the three groups in lactate dehydrogenase, ferritin, and procalcitonin (all P＞0.05); the AOSD group had significantly higher median CRP and ESR than the other two groups (all P＜0.05). For the patients with the three diseases, there were no significant differences in the four inflammatory indices between any two the groups with different ALT levels (all P＞0.05), and the level of ferritin was positively correlated with ALT level (R2=0.702 1, P＜0000 1). As for immune indices, there were no significant differences in IgG, IgA, C4, and the counts of NK and B cells between the three groups (P＞0.05), and CD8+ T cells were positively correlated with ALT level in HLH patients (R2=0.969 6, P＜0.000 1). ConclusionVarying degrees of liver injury are observed in patients with DRESS, AOSD or HLH. Ferritin and CD8+ T cells are well correlated with ALT level and can reflect liver injury, systemic inflammation, and immune status in patients with the three diseases, and therefore, they may become important indices for evaluating disease condition, guiding treatment, and judging treatment outcome and prognosis.

Clinical analysis and literature review of adult onset Still's disease with interstitial lung diseases / 中华风湿病学杂志

Objective To analyze the clinical characteristics of patients with adult onset Still's disease (AOSD) with interstitial lung diseases (ILD),and review the literature.Methods The medical records of inpatients with AOSD and ILD from January 2000 to October 2017 were retrospectively analyzed,and papers were searched and summarized with the key words "adult onset Still's disease" and "interstitial lung diseases".Kolmogorov-Smirnov test was used to test if variables met normal distribution.Measurement data which was normally distributed was described as Mean ±SD.Measurement data which was not normally distributed was described as median and interquartile range.Results Among the 15 patients included in the study,six were male,and nine were female,and the mean age was (50±12) years.All of the 15 patients had fever,and the average temperature was (39.4±0.4) ℃.Eleven patients had rash,and 12 patients had arthralgia,seven patients presented with cough,and eight patients presented with short of breath.The high resolution computed tomography of the chest presented as ground glass opacity in nine patients,grid shadow in three patients and consolidation in three patients.All the 15 patients received glucocorticoids,and 10 patients received immunosuppressants at the same time.One patient was lost to follow up,four patients died (three patients died of respiratory failure and one patient died of myocardial infarction),10 patients improved.Conclusion Patients with AOSD can also have ILD,which should be alerted by clinicians.Early treatment with glucocorticoids and immunosuppressants if infection is excluded may bring good prognosis,and it is easy to relapse when glucocorticoids is tapered off.

Expression of procalcitonin in polygenic auto-inflammatory diseases / 中华风湿病学杂志

Objective To detect the levels of procalcitonin in multiple genes autoinflammatory disease (adult Still disease,systemic juvenile idiopathic arthritis,crohn's Crohn's disease),and to explore the relationship between erythrocyte sedimentation rate (ESR),C-reactive protein (CRP),ESR/CRP and disease or complicated infection combined disease.Methods One hundred and fifty-three patients were en-rolled,88 patients with multiple genes autoinflammatory disease,including 32 cases of adult Still disease,27 cases of systemic juvenile idiopathic arthritis,29 cases of Crohn's disease.In addition,30 cases of healthy controls,35 patients with systemic lupus erythematosus (SLE) were included into this study.Electroche-miluminescence was used to test the value of serum PCT,erythrocyte sedimentation rate was tested by blood sedimentation instrument method,the CRP level was tested by lmmunoturbidimetry,and the data was handed managed and analysised by matlab software and One-way analysis of variance (ANOVA) was used to compare the differences of PCTs between groups.Results ① In the non-infection condition,the PCT value of the autoimmune inflammatory diseases [(0.36±0.74) μg/L,95％confidence interval (CI) (0.174 9,0.550 9) μg/L)] was signifieantly higher than that of the healthy control group [(0.06±0.06) μg/L,95％CI (0.035 1,0.081 7 μg/L)],the difference was statistically significant (F=5.03,P=0.027 4),but there was no statistically significant (F=1.03,P=0.475 5) when comparing with SLE group.② The PCT level of the non-infected inflammatory enteric arthritis [(0.20±0.32),95％CI(0.042 7,0.364 3) μg/L] was different compared with the healthy group,the difference was statistically significant (F=5.77,P=0.020 4),at the same time,the difference was not statistically significant when comparing with the SLE group (F=0.22,P=0.647 6).When the PCT value in non-infected adults Still disease [(0.60±1.02) 95％CI(0.048 4,1.153 6) μg/L] compared with the healthy group,the difference was statistically significant (F=7.22,P=0.01) but the difference was not statistically different when compared with the SLE group (F=2.65,P=0.114 3).The PCT level difference was statistically significant (F=2.23,P＜0.01)when comparing infection-free juvenile idiopathic arthritis [(1.52±2.02) μg/L,95％CI(0.054 8,4.591 9) μg/L] and the healthy group,the difference was statistically significantly different (F=8.34,P=0.004 7) when compared with the PCT of the non-infected SLE group.③ In the case of autoinflammatory diseases without infection,the 95％CI of ESR/CRP ratio was between 1.121 2 and 3.589 4.In the case of co-infection,the 95％ CI of ESR/CRP ratio was between 1.502 2 and 8.718 8,so we considered autoimmune inflammatory diseases might had a high possibility of co-infection when the ESR/CRP ratio was higher than 3.5.Conclusion ① The multiple genes autoinflammatory disease group has a higher value of PCT level than healthy controls even without infection.② The mean and 95％CI range of PCT of the inflammatory bowel disease arthritis,adult Still disease and the juvenile id-iopathic arthritis is significantly higher than the healthy controls,partially higher than SLE group.In addition,the PCT level in the juvenile idiopathic arthritis is the highest.③ In clinical,to estimate whether the multiple genes autoinflammatory disease has bacterial infection,we can't just simply rely on PCT to estimate whether the multiple genes autoinflammatory disease has bacterial infection,we may consider the ratio of the ESR/CRP,when the value is higher than 3.5,we may consider patients has strong probability with infection.

Enfermedad de Still del adulto. Presentación de un caso / Still´s disease in adults. Case presentation

La enfermedad de Still del adulto es una entidad de posible origen autoinmune, de etología desconocida y presentación infrecuente. Se caracteriza por fiebre alta, artritis, leucocitosis neutrofílica y exantema evanescente. Los valores de ferritina sérica por encima de 3 000 ng/mL son de gran valor para establecer el diagnóstico. En el presente artículo se describe el caso de un paciente de 28 años de edad, masculino, con un síndrome febril crónico, erupción cutánea, odinofagia y artritis. Se realizaron exámenes complementarios mediante los que se demostró la existencia de leucocitosis, elevación de los niveles de enzimas hepáticas y de ferritina sérica. La baja incidencia de la enfermedad, su complicada forma de presentación, así como la inexistencia de algún complementario confirmatorio para su diagnóstico, motivó a los autores a describir un paciente con enfermedad de Still del adulto. 

Still´s disease in adults is an entity with a possible autoimmune origin, of unknown etiology and infrequent presentation. It is characterized by high fever, netrophylic leukocytosis, and transient exanthema. The values of seric ferritin over 3 000 ng/mL are of great value to determine the diagnosis. It is described the case of a 28 year old male patient with chronic febrile syndrome, cutaneous rash, odynophagia and arthritis. Laboratory tests were performed which showed leukocytosis, high level of hepatic enzymes and seric ferritin. The low incidence of this disease, its complicated form of presentation, so as the non- existence of any confirming test for its diagnosis, motivated the authors to describe a patient with Still´s disease of the adult.

Oral mucosa lesions as atypical manifestation of adult-onset Still´s disease

Abstract: Adult-onset Still's disease is a systemic inflammatory disorder of unknown etiology, characterized by skin rash, spiking fever, arthralgias or arthritis, and leukocytosis. The typical skin rash is evanescent, salmon-pink, nonpruritic and maculopapular, predominantly on the extremities. It is considered one of the major Yamaguchi's criteria in adult-onset Still's disease. However, atypical skin lesions are also described. Here, a 61-year-old woman with sore throat, spiking fever, polyarthritis and evanescent salmon-pink nonpruritic maculopapular skin rash on the extremities was diagnosed with adult-onset Still's disease. In addition, atypical brown macules on oral mucosa, localized on the inner lips and tongue were also observed. Biopsy revealed a neutrophilic infiltrate. Despite treatment and improvement of the adult-onset Still's disease, the atypical oral mucosal lesions persisted.

Progress on pathogenesis and treatment of adult-onset Still disease / 中华全科医师杂志

Adult-onset Still disease (AOSD) is a polygenic autoinflammatory disease with uncertain etiology.The clinical manifestations are complex,including high-spiking fever,rash,arthritis,arthralgia,lymphadenopathy,and leukocytosis.The pathogenesis of AOSD is still unclear,whereas it has been confirmed that many inflammatory cells and cytokines take part in the development of AOSD.The treatment of AOSD is mainly based on corticosteroids and immunosuppressive agents.Recently,with the research progress of autoinflammatory diseases and the emerging biological agents,we have new knowledge on the pathogenesis and therapeutic strategies of AOSD.This article reviews the current progress in pathogenesis and therapy of AOSD.

Application of the 2016 European League Against Rheumatism/American College of Rheumatology/Pediatric Rheumatology International Trials Organization classification criteria for macrophage activation syndrome complicating systemic juvenile idiopathic arthritis in adult-onset Still disease / 中华风湿病学杂志

Objective In 2016,European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR)/Pediatric rheumatology international trials organization (PRINTO) released the classification criteria for macrophage activation syndrome (MAS) in patients with systemic juvenile idiopathic arthritis (sJIA).Due to the similarities of both clinical manifestations and pathogenesis between adult-onset Still dsease (AOSD) and sJIA,we hope to evaluate the 2016 sJIA-AMS classification in AOSD patients.Methods A total of 169 AOSD patients who were hospitalized in Renji Hospital were enrolled in this study.AOSD patients were divided into AOSD with MAS and AOSD without MAS,using the 2016 sJIA-MAS criteria.The data of the two groups were analyzed by Chi-square test,Mann-Whitney U test and binary Logistic analysis,and factors influencing the prognosis of patients were analyzed by Kaplan-Meier and COX regression analysis.Results According to sJIA-MAS criteria,56 AOSD patients with MAS were identified in all the 169 AOSD cases.In AOSD patients,the incidence of splenomegaly and pericarditis/myocarditis was significantly higher in patients with MAS than in AOSD without MAS [42.9％ vs 14.2％,OR(95％CI)=4.50(2.13,9.51),P＜0.01;10.7％ vs 0.9％,OR(95％CI)=13.21 (1.56,113.57),P＜0.01],also the incidence of liver dysfunction was higher in AOSD with MAS [67.8％ vs 11.5％,OR(95％CI)=0.18(7.26,36.33),P＜0.01].Among the AOSD with MAS,62.5％(35/56) of these patients received large-dose glucocorticoid therapy,5.4％ (3/56) received the glucocorticoid pulse therapy,48.2％(27/56) were treated with IVIG,and 26.8％(15/56) were treated with calcium phosphatase inhibitors.The mortality rates of AOSD with MAS was 8.9％(5/56),which was significantly higher than 1.8％(2/113) (OR =5.44,P＜0.05),the mortality rate of the AOSD without MAS.Patients who fulfilled the sJIA-MAS criteria suggested poor prognosis (OR=0.041,P=5.44),and the platelet count ≤ 181× 109/L (OR=12.17,P=0.002),alanine aminotransferase ＞48 U/L (OR=9.43,P=9.040) were also highly suggestive of poor prognosis.Conclusion The 2016 sJIA-MAS classification criteria are particularly valuable for early recognization of MAS in AOSD patients,and convenient to use.AOSD patients fulfilled sJIA-MAS criteria are more severe,and require larger doses of glucocorticoid and more immunosuppression therapy compared to patients without MAS,and the prognosis of these patients is also poor.

Low dose efficacy of etoposide on conventional treatment-refractory adult-onset Still disease / 中华风湿病学杂志

Objective To assess the efficacy of low-dose etoposide in patients with adult-onset Still disease (AOSD) refractory to conventional treatment.Methods This was a retrospective study of etoposid treatment in 24 patients with conventional treatment-refractory AOSD.Mann-Whitney U-test,Student's t test and chi-squared test were used for analysis.Results The age of the patients was (38±13) years.The median duration of AOSD before etoposide initiation was 2.5 months [interquartile range (IQR)] 1 month to 14 years).The median dosageof etoposide was 575 mg (IQR 150-1 400 mg).The median treatment course was 4 weeks (IQR 2 weeks to 10 months).Etoposide treatment resulted in rapid and maintained improvement in both clinical and laboratory parameters.The median dosage of methylprednisolone was also reduced.The most common side effectwas infection,and other side effects were mild leukopenia or neutropenia,gastrointestinal effects and hair loss.Two patients died and 22 patients survived.With an average follow-up of 14 months (IQR 1-32 months),4 of which were treated with corticosteroid alone,and 18 patients were treated with corticosteroid plus immunosuppressive agents.The patient's condition was stable without disease flare.Conclusion Etopo-side treatment is associated with rapid and maintained clinical and laboratory improvement in patients with refractory AOSD.Infection is the most common side effect.It is necessary to carry out large samples and longterm follow-up clinical studies to evaluate its exact effect and safety.

Skin symptoms as diagnostic clue for autoinflammatory diseases

ABSTRACT Autoinflammatory disorders are immune-mediated diseases with increased production of inflammatory cytokines and absence of detectable autoantibodies. They course with recurrent episodes of systemic inflammation and fever is the most common symptom. Cutaneous manifestations are prevalent and important to diagnosis and early treatment of the syndromes. The purpose of this review is to emphasize to dermatologists the skin symptoms present in these syndromes in order to provide their early diagnosis.

Imaging of 18F-FDG PET/CT in Adult Onset Still's Disease / 中国医学影像学杂志

Purpose The diagnosis of adult onset Still's disease (AOSD) is usually difficult due to the lack of specific clinical manifestation.This paper summarizes the manifestations of 18F-FDG PET/CT in adult onset Still's disease and investigates the value of PET/CT in diagnosis and differential diagnosis of AOSD.Materials and Methods Fiftyfour patients who was diagnosed as AOSD were selected and underwent 1 8F-fluorodeoxyglueosepositron emission tomography/computed tomography (18F-FDG PET/CT).The clinical features,laboratory examination and the maximum standard uptake value (SUVmax) of liver,spleen,bone marrow,lymph node were collected.Then the main PET/CT manifestations of patients with AOSD,the influence factor of SUVmax and correlation between SUVmax and laboratory indexes were analyzed.Results FDG accumulation occurred mainly in bone marrow (88.89％;SUVmax:3.91 ± 1.16),spleen (79.63％,SUVmax:3.24±0.89) and lymph node (77.78％;SUVmax:3.83± 1.97).FDG accumulation can also occurred in joints,parotid gland,submandibular gland,pleural and other organs.Compared with the nonglucocorticoid group,SUVmax of the spleen,bone marrow and lymph node were significantly decreased in the glucocorticoid group with or without fever (P＜0.05),whereas the SUVmax of liver,spleen,bone marrow and lymph node between the two glucocorticoid groups were not statistically different (P＞0.05).The SUVmax of liver,spleen,bone marrow and lymph node between two groups with or without disease-modifying anti-rheumatic drugs were not statistically different (P＞0.05).Correlation analysis showed that spleen SUVmax and lactate dehydrogenase,bone marrow SUVmax and C reactive protein were weakly correlated (r=0.33 and 0.30,P＜0.05).Conclusion The main manifestations of 18F-FDG PET/CT of AOSD are FDG accumulation in spleen,bone marrow and lymph nodes.Glucocorticoid can reduce the SUVmax.18F-FDG PET/CT can help to rule out malignancy,guide biopsy and assist in definite diagnosis of AOSD.

Clinical analysis of eight cases of adult-onset Still's disease / 中华皮肤科杂志

Objective To investigate characteristic skin lesions and typical histopathological changes of adult-onset Still's disease(AOSD) for its early diagnosis and treatment.Methods Clinical data were collected from 8 patients with AOSD,and analyzed retrospectively.Results All the patients had transient rashes and persistent papules/plaques during the course of disease.Of the 8 patients,1 had urticaria-like rashes,3 had dermatomyositis-like rashes,and 1 had prurigo pigmentosa-like rashes.Biopsies were carried out at the sites where transient rashes or persistent papules/plaques occurred.Histopathological findings showed necrotic keratinocytes in the upper prickle cell layer,and perivascular infiltration of neutrophils and lymphocytes in the upper dermis.Conclusion The skin lesions and histopathological changes of AOSD are characteristic,which can provide clues to the early diagnosis of AOSD.

The role of ¹⁸F-fluorodeoxyglucose positron emission tomography in the assessment of disease activity of adult-onset Still’s disease

BACKGROUND/AIMS: ¹⁸F-fluorodeoxyglucose positron emission tomography/computed tomography (¹⁸F-FDG PET/CT) has been suggested as a reliable imaging technique for monitoring of disease activity in patients with adult-onset Still’s disease (AOSD). Therefore, we investigated the clinical significance of ¹⁸F-FDG PET/CT in Korean AOSD patients. METHODS: Thirteen AOSD patients were included in the study. The PET/CT images were evaluated with visual and semiquantitative method using standardized uptake values (SUVs). RESULTS: The presence of increased ¹⁸F-FDG uptake was noted in 90% of clinically active AOSD patients. ¹⁸F-FDG uptake was located in the lymph node, spleen, and bone marrow. Visual grade and SUV intensity of lymph node was significantly correlated with the systemic score of AOSD. Visual grade of spleen was significantly correlated with the systemic score, erythrocyte sedimentation rate (ESR), and ferritin. Additionally, visual grade and SUV intensity of bone marrow was significantly correlated with the systemic score, ESR, leukocyte, and neutrophil. CONCLUSIONS: Visual grade and SUV intensity of lymph node, spleen, and bone marrow on ¹⁸F-FDG PET/CT scan showed significant correlations with known disease activity markers. The data suggest that ¹⁸F-FDG PET/CT scan may be a useful imaging technique for evaluation of disease activity in AOSD patients.

Adult onset Still disease: A rare condition. Clinical experience with 17 cases / Una patología poco frecuente: la enfermedad de Still del adulto. Experiencia clínica con 17 casos

OBJECTIVE: To present current clinical experience and the clinical outcomes in the management of patients with adult Still disease. MATERIALS AND METHODS: A retrospective study of a case series was conducted on of 17 cases diagnosed with adult Still disease during a period between 2009 and 2014 in 2 tertiary hospitals in Santiago de Cali. RESULTS: Of the 17 cases diagnosed and treated, it was found that 50% of patients had an elevated ferritin five times its normal value, being useful in the diagnosis. Less than 50% of cases were under 40 years, and about 50% of the population required combined treatment, with two patients requiringbiological therapy to control their symptoms. Yamaguchi criteria were used for diagnostic correlation. CONCLUSIONS: Adult Still's disease is a rare disease that requires a high index of suspicion, but it must be a disease to rule out, and it always requires management generally combined with schemes to improve the quality of life of patients.

OBJETIVO: Dar a conocer la experiencia clínica en el mundo real y los resultados clínicos del manejo de los pacientes con enfermedad de Still del adulto. MATERIALES Y MÉTODOS: Se realiza un estudio retrospectivo de serie de casos, de 17 casos con diagnóstico de enfermedad de Still del adulto, en 2 clínicas de nivel 3 de Santiago de Cali, Colombia, en el periodo comprendido entre 2009 y 2014. RESULTADOS: De los 17 casos diagnosticados y tratados se encontró que en el 50% de los pacientes había una elevación de ferritina de 5 veces su valor normal, siendo útil en el diagnóstico, menos del 50% de los casos fueron en menores de 40 anos, alrededor del 50% de la población requirió tratamiento combinado y 2 pacientes requirieron terapia biológica para el control de sus síntomas. Se usaron los criterios de Yamaguchi para la correlación diagnóstica. CONCLUSIONES: de descarte y, generalmente, siempre requiere manejo con esquemas combinados para mejorar la calidad de vida del paciente.

Clinical features of adult-onset Still's disease patients with pulmonary complications / 中华全科医师杂志

Objective To review the clinical characteristics of adult-onset Still's disease (AOSD) patients with special pulmonary complications.Methods Medical records of 506 AOSD inpatients,who were admitted in Peking Union Medical Hospital from October 1997 through March 2015,were retrospectively reviewed.The clinical features,biochemical parameters,radiology,pulmonary function tests,ultrasound,treatments and prognosis of AOSD patients with special pulmonary complications were analyzed.Results The incidence of special pulmonary complications in AOSD inpatients was 2.2％ (11/ 506),including 3 cases of interstitial lung disease and 8 cases of pulmonary artery hypertension.All 11 patients had common manifestations of AOSD,including fever (11/11),arthralgia/arthritis (11/11),rash (9/11),lymphadenopathy and/or splenomegaly (8/11),serositis (10/11),abnormal liver function (9/ 11),neutrophilia (11/11),and hyperferritinemia (11/11).The patients with interstitial lung disease had no or mild symptom,and were diagnosed by HRCT and pulmonary function test.The patients with pulmonary artery hypertension had various symptoms including dyspnea of exertion (8/8),cough (4/8),chest suppression (2/8),and chest pain (2/8).Four patients died from severe infection,including 3 cases of pulmonary artery hypertension and 1 case of interstitial lung disease.Conclusions Interstitial lung disease and pulmonary artery hypertension are life-threatening complications of AOSD patients.Earlier recognition and prompt treatment should be made to improve patient outcomes.