Enfermedad de Still del adulto: serie de casos / Adult Still's disease: case series

Resumen La enfermedad de Still del adulto (ESA) es una enfermedad reumática e inflamatoria, infrecuente, de etiología desconocida. Su presentación clínica es variada y sus síntomas más frecuentes son fiebre, artralgias, exantema asalmonado evanescente, odinofagia, adenopatías, hepatoesplenomegalia, serositis y presencia en laboratorio de leucocitosis (neutrofilia), hiperferritinemia asociado a ausencia de anticuerpos. El diagnóstico es clínico y por exclusión. Existen criterios clasificatorios como son los de Yamaguchi1, Cush y Fautrel2,3 que ayudan a la orientación diagnóstica. El tratamiento se define según la presentación y la evolución clínica1. Se describen las características clínicas, diagnósticas, tratamiento y evolución de siete pacientes con ESA.

Abstract Adult Still's disease (ASD) is an uncommon rheumatic and inflammatory disorder of unknown etiology. The major clinical features include fever, arthralgia, transient salmon-pink rash, odynophagia, lymphadenopathy, hepatosplenomegaly, serositis, and laboratory findings such as leukocytosis with neutrophilia, hyperferritinemia, and negative immunologic laboratory testing. ASD diagnosis is reached by exclusion. Different classification criteria such as Yamaguchi, Cush and Fautrel have been developed for the identification of ASD. Treatment is based on the degree of disease activity and clinical response1. We describe clinical manifestations, diagnosis, treatment, and prognosis of seven patients with adult's Still disease.

Increased human neutrophil lipocalin and its clinical relevance in adult-onset Still's disease / 中华医学杂志(英文版)

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.

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.

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.

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.

Fiebre de origen desconocido. Presentación de un caso clínico / Fever of uknown origin: a case report

La fiebre de origen desconocido (FOD) constituye una forma de presentación atípica o infrecuente de una patología común, que se manifiesta como una alteración rara. No se ha consensuado el diagnóstico de FOD de manera sistemática, sino que se establece a medida que evoluciona el cuadro sin un hallazgo etiológico cierto. El tratamiento es muy discutido y no existe consenso al respecto. Caso clínico: se presenta el caso de un paciente de sexo masculino de 45 años, que consulta a la guardia médica del Hospital Naval Puerto Belgrano (HNPB) por fiebre persistente con 6 días de evolución, acompañada de cefalea frontal y dolor abdominal generalizado. Refiere haber tomado levofloxacina (500mg/día). Al exámen físico presenta lucidez, signos vitales conservados, hemodinámicamente estable sin particularidades en la semiología cardiovascular y respiratoria. Estudios de laboratorio, ecográficos y colangio pancreatografía por resonancia magnética descartaron un proceso obstructivo y/o infeccioso (bacteriológico/viral) y tumoral. Por tomografía axial computada de cerebro, tórax, abdomen y pelvis se informó una lesión parietal en ángulo esplénico del colon y se indicó una colonoscopía sin hallazgo de patología neoplásica. El cuadro febril persistente se trató con antibióticos de forma empírica y con corticoides (meprednisona). Conclusiones: La evolución del paciente fue favorable. Fue definido como enfermedad de Still por exclusión, considerando los resultados negativos de los diferentes estudios impartidos. Se otorgó alta hospitalaria sin secuelas. (AU)

Fever of unknown origin (FUO) is an atypical or infrequent presentation of a common pathology, which manifests itself as a rare disorder. Currently, it has not been possible to formulate the diagnosis of FUO in a systematic way, with the difficulties that it entails in order to address a behavior in this regard. The diagnosis is made as the picture evolves without a true etiological finding. Treatment is much discussed with no consensus about it. Clinical Case: the case of a 45-year-old male patient is presented, who consults the doctor on-call at the Puerto Belgrano Naval Hospital for persistent fever for 6 days, accompanied by frontal headache and generalized abdominal pain. He refers having taken levofloxacin (500mg/ day). At the physical examination he shows alertness, preserved vital signs, hemodynamic stability without particularities in cardiovascular and respiratory semiology. Laboratory, ultrasound and magnetic resonance cholangio-pancreatography studies ruled out an obstructive and / or infectious (bacteriological/viral) disease and tumor process. Computed axial tomography of the brain, thorax, abdomen and pelvis reported a parietal lesion in the splenic angle of the colon, and a colonoscopy was reported without findings of neoplastic pathology. The persistent febrile condition was treated with antibiotics empirically and with corticosteroids (meprednisone). Conclusions: The evolution of the patient was favorable. A diagnosis of Still's disease was reached by exclusion, considering the negative results of the different studies carried out. Hospital discharge was granted without sequelae. (AU)

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.

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.

Enfermedad de Still del adulto asociada a neoplasia gástrica / Adult-onset Still's disease associated with gastric cancer

Resumen El diagnóstico de enfermedad de Still del adulto requiere la exclusión de cuadros infecciosos, tumorales y autoinmunes. Sin embargo, un proceso neoplásico poco expresivo clínicamente y en las pruebas complementarias puede pasar desapercibido al diagnóstico o comenzar con posterioridad, habiéndose descrito numerosos casos de enfermedad de Still asociada a tumores. Presentamos el caso de una paciente de 84 años con diagnóstico previo de enfermedad de Still del adulto, que desarrolló un tumor gástrico de evolución fatal 2 años después del diagnóstico del cuadro reumatológico.

Abstract The diagnosis of Adult-onset Still's disease (AOSD) requires the exclusion of infectious, malignant, and autoimmune diseases. However, a poorly symptomatic neoplastic process can easily be overlooked, or even onset later during the course of the disease. Therefore, numerous cases of Adult-onset Still's disease associated with malignancy have been reported. The case is reported of an 84-year old woman with previous diagnosis of AOSD who developed a gastric tumour with fatal outcome 2 years after the diagnosis of her rheumatic disease.

Clinical characteristics and treatment outcomes of macrophage activation syndrome in adults: A case series of 67 patients / 北京大学学报(医学版)

OBJECTIVE@#To described the clinical and laboratory features and outcome of 67 macrophage activation syndrome (MAS).@*METHODS@#A total of 67 MAS patients from three centers from January 2007 to December 2017 were enrolled. Clinical and laboratory features, and response to therapy were analyzed. Predictive factors for remission and survival were explored.@*RESULTS@#We identified a mean age of (36.1±16.3) years at diagnosis of MAS and a median connective tissue disease (CTD) duration of 8 months prior to MAS development. Among 67 MAS patients identified, underlying diseases included adult-onset Still's disease (AOSD) in 56.7% and systemic lupus erythematosus (SLE) in 30.0%. Fever and splenomegaly were found in 100.0% and 82.1% of the patients, respectively. Ferritinemia and elevation of serum soluble interleukin-2 receptor was seen in 100.0% and 93.2% of the patients. Serum levels of alanine aminotransferase, D-dimer, ferritin and C reactive protein were significantly higher in MAS associated with the AOSD patients than in MAS associated with the SLE patients. A significant decrease of erythrocyte sedimentation rate was found in MAS associated with AOSD, as compared with MAS associated with SLE. The most commonly used therapy was corticosteroids, which were initially administered in 100.0% of the patients. Intravenous immunoglobulin (IVIG) was administered in 91.0%, cyclosporine A in 64.2%, and etoposide in 46.3% of the patients, respectively. The induction therapy yielded a complete remission (CR) at the end of week 8 in 47.8% of the MAS patients. The overall mortality rate at the end of week 16 was 22.4%. The median serum levels of gamma-glutamyltransferase, alkaline phosphatase, total bilirubin and direct bilirubin were significantly lower in the patients who achieved complete remission at the end of week 8 than in those who did not, and splenomegaly was significantly less frequent (71.9% vs.91.4%, P=0.037). Both the mean age at diagnosis of MAS and the mean age at diagnosis of underlying CTD of the deceased patients were elder than those of the survived population (P=0.014 and P=0.017, respectively). The platelet count was significantly less in the deceased population as compared with the living population (P=0.018). No addition of cyclosporine A (P=0.004) was identified as risk factors associated with death in Logistic regression analysis.@*CONCLUSION@#MAS secondary to connective tissue disease is most common with AOSD and SLE. In terms of laboratory findings, there were considerable differences between the patients with underlying SLE and those with AOSD. Advanced age and low platelet counts are significant predictive factors for death, while treatment with cyclosporine may reduce the risk.

Afectación articular en un paciente joven con enfermedad de Still del adulto: a propósito de un caso / Joint involvement in a young patient with adult Still's disease: in relation to a case

Introducción: La enfermedad de Still del adulto es una enfermedad reumática, inflamatoria, sistémica y crónica que generalmente comienza sobre los 40 años de edad, pero puede aparecer en edades más tempranas. Se caracteriza por gran variedad de manifestaciones generales, articulares, fiebre y rash principalmente. Etiopatogénicamente, el proceso inflamatorio crónico que se genera durante el curso de la enfermedad es el responsable de las manifestaciones clínicas y del daño articular que se produce. Objetivo: dar a conocer los elementos básicos para el diagnóstico de la enfermedad de Still del adulto. Caso clínico: se presenta el caso de un paciente masculino, de 21 años de edad, que acude a consulta con manifestaciones clínicas que permiten realizar el diagnóstico de una Enfermedad de Still del Adulto. Conclusiones: La enfermedad de Still del Adulto, a pesar de no tener un patrón de deformidades tan amplio como la artritis reumatoide, también produce daño articular que puede comprometer la función de la articulación afectada. Resulta importante identificar precozmente las manifestaciones clínicas que hacen sospechar su diagnóstico para minimizar, retrasar o prevenir la afectación de las articulaciones(AU)

Introduction: adult Still's disease is a rheumatic, inflammatory, systemic and chronic disease that usually begins around 40 years of age, but may appear at younger ages. It is characterized by a great variety of general, joint, fever and rash manifestations. Etiopathogenically, the chronic inflammatory process that is generated during the course of the disease is responsible for the clinical manifestations and joint damage that occurs. Objective: to present the basic elements for the diagnosis of adult Still's disease. Clinical case: the case of a male patient, 21 years of age, who comes to the clinic with clinical manifestations that allow the diagnosis of an Adult Still's Disease. Conclusions: adult Still's disease, despite not having a pattern of deformities as extensive as rheumatoid arthritis, also produces joint damage that can compromise the function of the affected joint. It is important to identify early clinical manifestations that make your diagnosis suspect to minimize, delay or prevent the involvement of the joints(AU)

Manifestación atípica de enfermedad de Still: Reporte de un caso / Atypical manifestation of Still disease: Case report

La enfermedad de Still del adulto (ESA) es un proceso inflamatorio sistémico, de etiología desconocida, que se caracteriza por fiebre, artritis y eritema evanescente, además de valores elevados de ferritina sérica. Sin embargo, hasta la fecha, no hay una prueba definitiva de laboratorio o de imagen disponible para su diagnóstico, por lo tanto la ESA es un diagnóstico de exclusión. Presentamos el caso de una mujer de 44 años con manifestación cutánea atípica de ESA y cuadro clínico de 1 año de evolución caracterizado por fiebre de 40°C, linfadenopatía, hiperferritinemia, y que en la sistemática de estudio presentó positividad para anti-CCP (anticuerpo antipéptido cíclico citrulinado).

Adult Still's disease (ASD) is a systemic inflammatory process, of unknown etiology, characterized by fever, arthritis and evanescent erythema, in addition to elevated serum ferritin values. However, to date, there is no definitive laboratory or imaging test available to diagnose it, therefore ASD is a diagnosis of exclusion. We present the case of a 44-year-old woman with atypical cutaneous manifestation of ESA and positivity of ACPA (anti-cyclic citrullinated peptide antibody).

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.

Doença de Still como causa de febre de origem indeterminada: relato de caso / Still's disease as a cause of fever of undetermined origin: case report

POS, 22 anos, sexo feminino, foi admitida na enfermaria de Clínica Médica para investigação diagnóstica de quadro consumptivo. Queixava-se perda ponderal (45 kg no total), febre vespertina, artralgias e hiporexia.Relatava internações recorrentes devido a fraqueza. Na história pregressa constava aborto espontâneo, tendo sido aventada a hipótese de infecção pelo zika vírus, porém não foram realizados exames sorológicos confirmatórios.Tinha contato com irmão portador de paracoccidioidomicose e tio com tuberculose. Apresentava-se emagrecida,com flacidez notável, sarcopenia, palidez cutânea mucosa grave, linfadenomegalia generalizada e hepatoesplenomegalia. Diante do quadro clínico, suspeitou-se inicialmente de doença linfoproliferativa. Apresentou anemia microcítica e hipocrômica, sorologias negativas para HIV, sífilis, leishmaniose e hepatites virais, FAN positivo, padrão nuclear pontilhado, PCR e VSH elevados, leucocitose e ferritina maior que 2000 em dois exames. O PPD era não reator. Os anatomopatológicos apresentavam pesquisa negativa para fungos e parasitas e sugeriam doença linfoproliferativa,entretanto as imunohistoquímicas revelaram padrão de linfonodos reacionais. Devido à suspeita inicial de doença linfoproliferativa foi iniciado profilaxia para lise tumoral com hidratação e alopurinol.Além disso devido as diversas linfonodomegalias foi iniciado tratamento empírico com Prednisona 80 mg/dia, com melhora do quadro clínico, remissão da febre e artralgia. Como os anatomopatológicos e imunohistoquímicas descartaram doenças proliferativas, foi iniciado desmame do corticoide, com ressurgimento da febre, dessa vez acompanhada de rash cutâneo evanescente em membros e artrite em joelho direito. Após afastar doenças infecciosas, neoplásicas e outras etiologias, considerou-se o diagnóstico de doença de Still, uma vez que a paciente apresentava quadro clínico compatível e preenchia os critérios de Yamaguchi. Iniciada terapia com metotrexate, com melhora do quadro clínico e alta hospitalar. (AU)

POS, 22 years old, female, was admitted for a diagnostic investigation of the consumptive condition. Weight loss (45 in total), afternoon fever, arthralgia and hyporexia were noted. Reported recurrent hospitalizations due to weakness. Previous history consisted of spontaneous abortion, having been hypothesized to be infected by zika virus, but no confirmatory serological tests were performed. He had contact with a brother with paracoccidioidomycosis and uncle with tuberculosis. He was emaciated, with remarkable flaccidity, sarcopenia, severe mucosal skin paleness, generalized lymphadenomegaly and hepatosplenomegaly. In the clinical picture, lymphoproliferative disease was initially suspected. It presented microcytic and hypochromic anemia, HIV negative serology, syphilis, leishmaniasis and viral hepatitis, (PPS) was not a reactor. The anatomopathologicals presented negative research for fungi and parasites and suggested lymphoproliferative disease. However, immunohistochemistry revealed a pattern of reactional lymph nodes. Due to the initial suspicion of lymphoproliferative disease, prophylaxis was started for tumoral lysis with hydration and allopurinol. In addition, due to the various lymph node metastasis, empiric treatment with Prednisone 80 mg / day was initiated, with improvement of the clinical picture, remission of fever and arthralgia. and immunohistochemistry discarded proliferative diseases, weaning of the corticosteroid was started, with a resurgence of fever, this time accompanied by evanescent cutaneous rash in limbs and right knee arthritis. After eliminating infectious, neoplastic and other etiologies diseases, it was considered the diagnosis of Still, once the patient presented a compatible clinical picture and fulfilled Yamaguchi criteria. Initiated therapy with methotrexate, with improvement of the clinical picture and hospital discharge. (AU)

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 en el adulto: relato de caso y revisión literaria / Still's disease in adults: case report and literary review

La enfermedad de Still se caracteriza por ser una enfermedad inflamatoria, sis-témica, rara, sin etiología y patogénesis establecidas. Su tríada clásica de manifesta-ción es fiebre alta de origen desconocido, artralgia persistente y erupción cutánea de color salmón, además de manifestaciones sistémicas. El diagnóstico es clínico y requiere la exclusión de enfermedades infecciosas, neoplásicas y otras enfermeda-des autoinmunes, pues no posee marcadores biológicos específicos. Los exámenes de laboratorio son inespecíficos y muestran una actividad inmunológica exacerbada. El tratamiento incluye medicamentos antiinflamatorios no esteroideos, corticosteroi-des, inmunosupresores, gammaglobulina intravenosa y agentes biológicos. Describi-mos el caso de un paciente de 25 años con fiebre vespertina diaria sin foco conocido y artralgia migratoria, sin erupciones cutáneas.

Still's disease is characterized by being a rare systemic inflammatory disease with no established etiology and pathogenesis. Its classic manifestation triad is high fever of unknown origin, persistent arthralgia and salmon-colored rash, in addition to systemic manifestations. The diagnosis is clinical and requires the exclusion of infec-tious, neoplastic and other autoimmune diseases, as it does not have specific biologi-cal markers. Laboratory tests are non-specific and show exaggerated immune activity. Treatment includes non-steroidal anti-inflammatory drugs, corticosteroids, immu-nosuppressants, intravenous gammaglobulin and biological agents. We describe the case of a 25-year-old patient with daily afternoon fever with no known focus and migratory arthralgia without rash.

Síndrome ASIA: prótesis mamarias y enfermedad de Still / ASIA syndrome: breast implant and Still's disease

Las enfermedades del tejido conectivo vinculadas a implantes mamarios de siliconas han sido tema de discusión. En la última década, la siliconosis ha sido incluida dentro del síndrome autoinmune/inflamatorio inducido por adyuvante (ASIA) junto al síndrome de la guerra del Golfo, síndrome de miofascitis macrofágica y fenómenos post vacunales. El ASIA puede manifestarse como lupus, artritis reumatoidea, o más raramente como enfermedad de Still del adulto. Presentamos el caso de una paciente con fiebre prolongada y criterios clínicos compatibles con ASIA y enfermedad de Still. Se resecaron las prótesis y la anatomía patológica descartó linfoma anaplásico ALK (-) vinculado a prótesis. Los médicos debemos estar alertas ante la aparición de estas nuevas entidades asociadas a los implantes mamarios de siliconas.

Connective tissue diseases associated with silicone breast implants have been widely discussed. In the last decade, siliconosis has been included in the autoimmune/inflammatory syndrome induced by adjuvants (ASIA) next to Gulf War syndrome, macrophage myofascitis and postvaccination phenomena. The ASIA syndrome may appear as lupus, rheumatoid arthritis, or more rarely, as adult Still's disease. We discuss the case of a patient with prolonged fever and clinical criteria for ASIA and Still's disease. The prostheses were resected and pathology showed absence of breast implant associated anaplastic lymphoma ALK (-). Physicians should be alert to these new entities linked to silicone breast implants.

A case of adult-onset Still's disease accompanied by pseudo-gray platelet syndrome

No abstract available.

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.