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Introducción: La enfermedad de Still del adulto es una enfermedad reumática, inflamatoria, sistémica y crónica cuya prevalencia en la población caucásica se estima en 1 caso por cada 100 000 adultos Objetivo: Presentar un paciente adulto joven, con una fiebre de origen desconocido como forma de presentación de la enfermedad de Still del adulto. Caso clínico: Paciente de 29 años de edad con antecedente de fiebre reumática, con un ingreso hospitalario anterior; que presentó un cuadro febril no infeccioso, de 50 días de duración, al cual no se le determinó la causa. Un año después reapareció la fiebre, de similares características, asociada a poliartralgia, hepatoesplenomegalia, anemia, hiperferritinemia, neutrofilia, factor reumatoideo negativo y se constató un cuadro de pericarditis durante el ingreso. Se realizó el diagnóstico de enfermedad de Still del adulto, por exclusión. Se inició tratamiento con esteroides, desapareció la fiebre en las primeras 24 horas y el paciente tuvo una evolución favorable. Conclusiones: La enfermedad de Still del adulto puede presentarse como una fiebre de origen desconocido y se diagnostica por exclusión, ya que no existen manifestaciones clínicas ni pruebas de laboratorio, patognomónicas. La hiperferritinemia es útil para la sospecha diagnóstica(AU)
Introduction: Adult Still's disease is a rheumatic, inflammatory, systemic and chronic disease whose prevalence in the Caucasian population is estimated at 1 case per 100,000 adults. Objective: To present a young adult patient with a fever of unknown origin as the presentation of adult Still's disease. Clinical case: 29-year-old patient with a history of rheumatic fever, with a previous hospital admission; who presented a non-infectious febrile illness lasting 50 days, for which the cause was not determined. A year later, the fever reappeared, with similar characteristics, associated with polyarthralgia, hepatosplenomegaly, anemia, hyperferritinemia, neutrophilia, negative rheumatoid factor, and pericarditis was noted during admission. The diagnosis of adult Still's disease was made by exclusion. Treatment with steroids was started, the fever disappeared in the first 24 hours and the patient had a favorable evolution. Conclusions: Adult Still's disease can present as a fever of unknown origin and is diagnosed by exclusion, since there are no pathognomonic clinical manifestations or laboratory tests. Hyperferritinemia is useful for diagnostic suspicion(AU)
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Humanos , Adulto , Enfermedades Reumáticas , Enfermedad de Still del Adulto/diagnóstico , Enfermedad de Still del Adulto/etiología , Fiebre de Origen Desconocido/etiología , Hiperferritinemia , Esteroides/uso terapéuticoRESUMEN
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.
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Enfermedad de Still del Adulto , Enfermedades Reumáticas , FiebreRESUMEN
OBJECTIVES: To describe the epidemiology, characteristics, response to initial treatment, and outcomes of Adult-Onset Still's disease (AOSD) in the Afro-Caribbean population of Martinique with free and easy access to specialised care. METHODS: We conducted a retrospective study from 2004 to 2022 in the island of Martinique, French West-Indies which total population was 354 800 in 2021. Patients were identified from multiple sources including standardised databases. To be included, patients had to be residents of the island and fulfilled Yamaguchi and/or Fautrel's criteria for AOSD, or have a compatible disease course, without a diagnosis of cancer, auto-immune disease or another auto-inflammatory disorder. Date of diagnosis, clinical and biological characteristics, treatments, and outcomes were collected. RESULTS: The prevalence was 7.6/100 000 inhabitants in 2021. The mean incidence was 0.4/100 000 during study period. Thirty-three patients (70.6% females) with a median follow-up of 35 months [7.5 to 119] were included. Twenty-six patients (78.8%) had a systemic pattern. Patients with a systemic monocyclic pattern had significantly more polyarticular involvement than patients with systemic polycyclic pattern (p = 0.016). Pulmonary involvement occurred in 51.5% of patients at diagnosis and systemic Pouchot score has been identified as an independent predictive factor for pulmonary involvement; OR of 3.29 [CI 95% 1.20; 9.01]. At first flare, all patients but one received oral glucocorticoids, 11 patients (32.4%) received intravenous glucocorticoids pulse and 12 patients (33%) received anti-IL1 therapy. Nineteen patients (57%) relapsed in a median time of 9 months [6 to 12] Three patients (9%) developed hemophagocytosis lymphohistiocytosis, fatal in 1 case. All deceased patients (n = 4, 11.76%) belonged to the systemic polycyclic pattern, with an event-free survival of 13.6 months [IQR 5.7; 29.5] CONCLUSION: AOSD in the Afro-Caribbean population of Martinique shares some similarities with other ethnic groups, but exhibit differences, such as a high proportion of lung involvement. Comparative studies are needed to confirm these results.
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Enfermedad de Still del Adulto , Adulto , Femenino , Humanos , Masculino , Pueblos Caribeños/estadística & datos numéricos , Etnicidad , Glucocorticoides/uso terapéutico , Martinica/epidemiología , Estudios Retrospectivos , Enfermedad de Still del Adulto/diagnóstico , Enfermedad de Still del Adulto/tratamiento farmacológico , Enfermedad de Still del Adulto/epidemiología , Enfermedad de Still del Adulto/etnología , Indias Occidentales/epidemiologíaRESUMEN
RESUMEN La enfermedad de Still es un desorden inflamatorio infrecuente, con múltiples formas de presentación; sin embargo, el debut con miocarditis o pericarditis es aún menos común (prevalencia del 7%). Se presenta el caso de un hombre de 30 años, previamente sano, que consultó por dolor torácico. Durante su evolución clínica fue diagnosticado con miopericarditis, se indagaron diversas etiologías, pero luego de múltiples estudios se diagnosticó enfermedad de Still del adulto. Se instauró tratamiento con corticoesteroides, el cual tuvo una adecuada respuesta.
A B S T R A C T Adult-onset Still's disease is a rare inflammatory disorder with multiple forms of manifestation; however, the onset with myocarditis or pericarditis is even less common (7% prevalence). Herein, we present a case of a previously healthy 30-year-old man, who consulted for chest pain. During his clinical evolution, he was diagnosed with myopericarditis. Various aetiologies were evaluated and after multiple studies adult-onset Still's disease was diagnosed. Treatment with corticosteroids was established evidencing an adequate response.
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INTRODUCTION: The distribution of causes of hyperferritinemia in international series is heterogeneous. Also, the association between ferritin and prognosis is controversial. This study aims to describe the diagnosis associated with hyperferritinemia in a retrospective cohort at an academic healthcare network in Chile. METHODS: A retrospective review of adult patients admitted to our academic medical center from June 2014 to February 2017 with ferritin ≥3,000 ng/mL. All patients were classified into nine diagnostic categories. Then, the association between ferritin level and disease category, as well as mortality, was evaluated. RESULTS: Ninety-nine patients were identified. The mean age was 50.8 ± 19.9 years, 54.5% were men. The most frequent categories were "inflammatory and autoimmune diseases" (21.2%) and "hematological malignancies" (19.2%). The average ferritin was 10,539 ± 13,016.9 ng/mL, while the higher mean was 16,707 ng/mL in the "inflammatory and autoimmune diseases" category. There was a statistically significant association between the ferritin value and age but not between ferritin and diagnostic categories. In the group over 50, hematologic neoplasms (19%) and infections (19%) were more frequent. In those under 50, inflammatory and autoimmune diseases were more frequent (26.8%). There was no association between the ferritin level and mortality at 1, 3, and 12 months. CONCLUSIONS: The most frequent categories were "inflammatory and autoimmune diseases" and "hematological malignancies", but ferritin level was similar in both. Further research could validate a prognostic role.
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Humanos , Masculino , Femenino , Adulto , Persona de Mediana Edad , Anciano , Adulto Joven , Ferritinas/sangre , Hiperferritinemia/sangre , Pronóstico , Enfermedades Autoinmunes/sangre , Chile/epidemiología , Estudios Retrospectivos , Centros Médicos Académicos/estadística & datos numéricosRESUMEN
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-1ß, 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.
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INTRODUCTION Actually, there are more than 200 different causes of unknown fever, it is necessary for the doctor to identify the most prevalent causes of unknown fever in our Clinical boar. PRESENTATION A 52-year-old male patient with no history of chronic diseases, which was received in the Emergency Service of the Hospital San Pablo, Coquimbo, on 02/03/20 began a clinical picture characterized by fever up to 39 ºC associated with diaphoresis, evaluated several times in primary health care without response to symptomatic treatment. He denies dyspnea, cough, sputum production, headache, muscle weakness, myalgia, fainting, decreased visual acuity, nausea, vomiting, chest pain, abdominal pain, diarrhea, constipation, altered bowel movements, weight loss, dysuria, bladder tenesmus, pain lumbar, skin lesions. As relevant information, the patient reports having taken amoxicillin/clavulanate for 2 days on his own account. DISCUSSION Physicians should be aware of the rare extent of an unusual presentation of knowledge origin fever, probably associated with Still disease, as well as medical options for treatment. The literature does not conclude on a gold standard for the method of approach. CONCLUSION In our case, the etiological agent that could cause Fever of unknown origin (FUO) was Sars cov-2 given the presence of elevated inflammatory factors and acute phase proteins and the presence of neutrophilic infiltration
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Humanos , Masculino , Persona de Mediana Edad , Fiebre de Origen Desconocido/etiología , COVID-19/complicaciones , Exantema Súbito , Fiebre de Origen Desconocido/diagnósticoRESUMEN
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.
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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.
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Humanos , Enfermedad de Still del Adulto/diagnóstico , Sepsis/diagnóstico , Estudios Retrospectivos , Diagnóstico Diferencial , Volúmen Plaquetario MedioRESUMEN
RESUMEN La artritis idiopática juvenil sistémica, también conocida como enfermedad de Still, se considera un trastorno autoinflamatorio y suele ser la más compleja y grave entre todas las formas clínicas de la enfermedad. Cursa generalmente en forma de brotes de actividad repetidos, intercalados por periodos de remisión. Se presenta el caso de una paciente femenina de 4 años de edad, con diagnóstico de enfermedad de Still a los 2 años. Actualmente tiene tratamiento con triple terapia de inducción: cloroquina, metotrexato y salazosulfapiridina con actividad de la enfermedad persistentemente alta por JADAS 27. Acudió a consulta por presentar fiebre, toma del estado general y manifestaciones respiratorias de tres días de evolución que se interpretó como una infección respiratoria baja. Se prescribió tratamiento con antibióticos sin signos de mejoría. A los 7 días se agravó el cuadro clínico y se planteó el diagnóstico de síndrome de activación macrofágica. Se comenzó protocolo de tratamiento con esteroides en combinación con otros fármacos de probada eficacia para esta situación clínica (etopósido, ciclosporina, metotrexato). Se revaloró política de antibióticos sin lograrse respuesta satisfactoria y se decidió introducir el rituximab que aporta excelentes resultados. Después de 3 meses de difícil manejo, la paciente egresó del hospital recuperada de esta complicación y con bajo nivel de actividad de la enfermedad de base.
ABSTRACT Systemic Juvenile Idiopathic Arthritis, also known as Still's disease, is considered an autoinflammatory disorder and is often the most complex and severe of all clinical forms of the disease. It usually takes the form of repeated bouts of activity, interspersed with periods of remission. We present the case of a 4-year-old female patient, diagnosed with Still's disease at 2 years of age. Currently undergoing treatment with triple induction therapy: chloroquine, methotrexate and salazosulfapyridine with persistently high disease activity due to JADAS 27. He comes to the clinic due to fever, general condition, and respiratory manifestations of three days of evolution interpreted as an infectious respiratory process under. Antibiotic treatment is started without signs of improvement. At 7 days the clinical picture worsens, and the diagnosis of Macrophage Activation Syndrome is raised. A steroid treatment protocol is started in combination with other drugs of proven efficacy for this clinical situation (ethopside, cyclosporine, methotrexate). Antibiotic policy was reassessed without achieving a satisfactory response and it was decided to introduce rituximab, which provides excellent results. After three months of difficult management, the patient was released from the hospital recovered from this complication and with a low level of activity of the underlying disease.
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Humanos , Femenino , Preescolar , Artritis Juvenil/tratamiento farmacológico , Cloroquina/uso terapéutico , Metotrexato/uso terapéuticoRESUMEN
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.
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Humanos , Adulto , Enfermedad de Still del Adulto/diagnóstico , Sepsis/diagnóstico , Biomarcadores , Estudios Retrospectivos , Volúmen Plaquetario MedioRESUMEN
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.
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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.
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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.
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Humanos , Femenino , Adulto , Adulto Joven , Enfermedad de Still del Adulto/diagnóstico , Aspartato Aminotransferasas/sangre , Factor Reumatoide/sangre , Esplenomegalia , Sedimentación Sanguínea , Proteína C-Reactiva/análisis , Faringitis , Enfermedades Reumáticas/diagnóstico , Enfermedad de Still del Adulto/tratamiento farmacológico , Corticoesteroides/uso terapéutico , Artralgia , Antirreumáticos/uso terapéutico , Enfermedades Raras/diagnóstico , Diagnóstico Diferencial , Alanina Transaminasa/sangre , Exantema , Fiebre , Hiperferritinemia/sangre , Infecciones/diagnóstico , Leucocitosis/sangre , Neoplasias/diagnósticoRESUMEN
Cardiac involvement in adult-onset Still's disease (AOSD) usually manifests as a pericardial disease. Myocarditis is uncommon (prevalence of 7%). However, the cardiocirculatory failure is the second cause of life-threatening AOSD. Herein, we report the case of a 38-year-old man who was diagnosed with myocarditis caused by AOSD. He was treated medically with steroids and methotrexate, and his course was favorable. A literature search in PubMed/MEDLINE and Scopus databases from 1971 to 2019 identified 47 additional cases of myocarditis and AOSD. The main features found in these reports were reviewed and are the following: (i) myocarditis is a rare complication of AOSD manifested by fever, chest pain, dyspnea, and tachycardia; (ii) cardiac biomarkers, electrocardiogram (ECG), transthroracic echocardiography (ECHO), and cardiac magnetic resonance imaging (MRI) are useful noninvasive diagnostic tools; and (iii) myocarditis is a potentially life-threatening complication of AOSD but responds positively to steroids and other immunomodulatory drugs. This review suggests that this entity should be suspected in cases of acute febrile myocarditis after ruling out other causes since a prompt treatment results in a good prognosis.
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Miocarditis/etiología , Enfermedad de Still del Adulto/complicaciones , Adulto , Ecocardiografía , Electrocardiografía , Humanos , Imagen por Resonancia Magnética , Masculino , Metotrexato/uso terapéutico , Miocarditis/diagnóstico , Miocarditis/tratamiento farmacológico , Esteroides/uso terapéutico , Resultado del TratamientoRESUMEN
BACKGROUND: In Mexico, adult-onset Still's disease (AOSD) is one of the causes of fever of unknown origin (FUO). The aim of this study is to describe a series of AOSD cases from a FUO cohort in order to know the clinical and biochemical characteristics of the cases, as well as to describe the neutrophil-lymphocyte index (NLI), which is a clinical marker of inflammation in autoimmune diseases. CASE REPORT: An observational study of 24 cases with AOSD; 72 % of them were women, the median age was 43 years (IQR 37.7-59.7), and the most frequent manifestations were classic rash (84 %) and arthralgia (100 %). All of them had tested negative for rheumatoid factor, antinuclear antibodies, and hyperferritinemia; 83 % had NLI > 3.08. The most used treatment was the combination of methotrexate with corticosteroids; seven patients required biological therapy, and one of them presented a hypersensitivity reaction. CONCLUSION: When there's FUO, the existence of AOSD should be suspected; also in the presence of rash, arthralgia, hyperferritinemia, and NLI > 3.08.
Antecedentes: En México, la enfermedad de Still del adulto (ESA) es una causa de fiebre de origen desconocido (FOD). El objetivo de este informe fue describir una serie de casos de ESA de una cohorte de FOD para conocer las características clínicas y bioquímicas, así como describir el índice neutrófilo/linfocito (INL), marcador clínico de inflamación en enfermedades autoinmunes. Caso clínico: Estudio observacional de 24 casos con ESA; 72 % fue del sexo femenino, la edad fue de 43 años (37.7-59.7) y las manifestaciones más frecuentes fueron rash clásico (84 %) y artralgias (100 %). Todos tuvieron factor reumatoide, anticuerpos antinucleares negativos e hiperferritinemia; 83 % tuvo INL > 3.08. El tratamiento más empleado fue la combinación de metotrexato y corticosteroides; siete pacientes ameritaron terapia biológica, uno presentó reacción de hipersensibilidad. Conclusión: Ante fiebre de origen desconocido, debe sospecharse ESA si, además, existe rash, artralgias, hiperferritinemia e INL > 3.08.
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Fiebre de Origen Desconocido , Enfermedad de Still del Adulto , Adulto , Femenino , Humanos , Linfocitos , México/epidemiología , Neutrófilos , Enfermedad de Still del Adulto/complicaciones , Enfermedad de Still del Adulto/diagnósticoRESUMEN
INTRODUCCIÓN: Cuando inicia en el adulto, la enfermedad de Still es de mayor prevalencia en caucásicos y entre los 16 y 35 años. De etiología desconocida, se asocia a HLA-II, DR2, 4 y 7, y Bw35. El comienzo de los síntomas es agudo, con fiebre en agujas, asociada a exantema en las extremidades y el tronco, maculopapular, eritematoso y evanescente, pruriginoso, con fenómeno de Koebner. Presenta artralgias y artritis con un patrón poliarticular, simétrico y migratorio, mialgias y adenopatías. El 90% de los pacientes presentan anemia, leucocitosis con neutrofilia y trombocitosis asociada a la actividad, así como elevación de transaminasas y ferritina > 2000. El factor reumatoide y los anticuerpos antinucleares son negativos. El objetivo del presente trabajo es presentar un caso de enfermedad de Still que se sale del patrón habitual de manifestación. CASO CLÍNICO: Mujer de 56 años, inicia con exantema -maculopapular, eritematoso, pruriginoso, en zona periorbitaria -bilateral, tórax anterior, región glútea bilateral y zonas de extensión de codos y rodillas, que respetan el abdomen. Fiebre vespertina de 39 °C, con artralgias de codos, muñecas y rodillas, y mialgias, con faringe hiperémica. Después de descartar procesos infecciosos, neoplásicos y autoinmunitarios, y de acuerdo con los criterios de Yamaguchi y Fautrel, se diagnosticó enfermedad de Still. CONCLUSIONES: Este caso se presenta por la baja prevalencia de la enfermedad de Still y porque en el grupo etario de nuestra paciente no es habitual su presentación. Los -antecedentes familiares y el cuadro clínico sugestivo obligaron a descartar la presencia de otros procesos mórbidos, toda vez que el diagnóstico de enfermedad de Still es de exclusión. BACKGROUND: Adult-onset on Still's disease is common in Caucasians, between 16 and 35 years. Its cause is unknown, but it is associated with HLA-II, DR2, 4, 7 and Bw35. First symptoms are acute; fever in needles associated with exanthema in extremities and maculopapular trunk, erythematous and evanescent, pruritic with Koebner phenomenon. Patients present arthralgias and arthritis with a polyarticular, symmetrical and migratory pattern; myalgias and adenopathies. 90% of patients have anemia, leukocytosis with neutrophilia and thrombocytosis associated with the activity. Elevation of transaminases and ferritin greater than 2000. The rheumatoid factor and antinuclear antibodies are negative. The aim of this article is to present a case report of Still's Disease whose pattern of appearance is uncommon. CASE REPORT: A 56-year-old woman presented papular macular, erythematous, pruritic exanthema, in the bilateral peri-orbital area, anterior thorax, bilateral gluteal region, and elbow and knee extensions, while respecting abdomen. In addition, evening fever of 39 °C with arthralgias in elbows, wrists, and knees, myalgias, and hyperemic pharynx were manifested. According to the criteria of Yamaguchi and Fautrel, and after ruling out infectious, neoplastic, autoimmune processes, Still's disease was concluded. CONCLUSIONS: This case is presented due to the low prevalence of Still's disease and its presentation is not usual in the age group of our patient. The family history and very indicative clinical pictures forced us to rule out the presence of other morbid processes, while reinforcing the diagnosis of Still's disease, since it is by exclusion.
RESUMEN
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.
Asunto(s)
Humanos , Femenino , Persona de Mediana Edad , Citocinas , Enfermedad de Still del Adulto , Calidad de Vida , Actividades Cotidianas , Enfermedad CrónicaRESUMEN
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.
Asunto(s)
Humanos , Femenino , Anciano de 80 o más Años , Neoplasias Gástricas , Enfermedad de Still del Adulto , Enfermedades Autoinmunes , Diagnóstico , NeoplasiasRESUMEN
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)