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.

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.

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)

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.

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.

Enfermedad de Still del adulto, a propósito de un caso: un desafío diagnóstico / Still disease of the adult, about a case: a diagnostic challenge

La enfermedad de Still del adulto (ESA) es una patología inflamatoria sistémica infrecuente, que se caracteriza por la tríada de fiebre, exantema evanescente y artralgias. Mujer de 57 años con historia de 7 meses de lesiones tipo máculas-habones asalmonadas, en relación a peaks febriles intermitentes. Asocia odinofagia, mialgias y artralgias. En los exámenes destaca anemia leve, VHS, PCR y ferritina elevadas. Estudio viral e inmunológico negativo. La tomografía computada de tórax, abdomen y pelvis (TC-TAP) reveló múltiples adenopatías axilares, pélvicas y esplenomegalia. La biopsia cutánea y de un linfonodo axilar, descartó patología vasculítica y neoplásica respectivamente, apoyando el diagnóstico de ESA. Se trató con prednisona 40 mg/día, con buena respuesta y sin recidivas. La ESA constituye un verdadero desafío diagnóstico, pues su clínica inespecífica y la ausencia de marcadores propician un diagnóstico tardío. Su pronóstico se relaciona con un tratamiento precoz, por lo que es fundamental un diagnóstico oportuno.

Adult-onset Still's disease (ESA) is an infrequent systemic inflammatory pathology, characterized by the triad of febrile syndrome, evanescent rash and arthralgias. A 57-year-old woman with a 7-months history of salmon-colored rash in relation to intermitent febrile peaks, asociated with odynophagia, myalgias and arthralgias. General exams showed mild anemia, elevated ESR, CRP and ferritin. Viral and immunological studies were negative. Computed tomography of the chest, abdomen and pelvis revealed multiple axillary and pelvic lymph nodes and splenomegaly. The skin and axillary lymph node biopsies, ruled out vasculitic and neoplastic pathologies respectively, supporting the diagnosis of ESA. Prednisone 40 mg daily was administered with good response and no evidence of recurrence. No evidence of relapse in subsequent controls. ESA reprsents a great diagnostic challenge, since its nonspecific clinic and the absence of pathognomonic studies lead to a late or erroneous diagnosis. Its prognosis is related to an early treatment; therefore it is essential to make en early diagnosis.

Miopericarditis como manifestación inicial de enfermedad de Still del adulto / Myopericarditis as initial manifestation of adult-onset Still's disease

Se presenta el caso de un hombre de 25 años de edad con cuadro febril persistente, leucocitosis, mialgias y artralgias, en quien se documenta miopericarditis con falla cardiaca aguda asociada. Tras un exhaustivo estudio de fiebre de origen desconocido se hace diagnóstico de enfermedad de Still del adulto.

We report the case of a 25 year-old man with persistent fever, leukocytosis, myalgias and arthralgias; in whom myopericarditis associated with acute heart failure was documented. After an exhaustive study of fever of unknown origin he was diagnosed as adult Still's disease.

Enfermedad de Still del adulto asociada a endocarditis marántica: reporte clínico y revisión de literatura / Marantic endocarditis associate to adult Still's disease

Varón de 52 años, 9 meses de enfermedad, caracterizado por artralgias, fiebre alta, exantema evanescente, diaforesis, faringitis, astenia y baja de peso. Al examen: linfadenomegalia, taquicardia, soplo sistólico III/VI mitral y aórtico, Catalogado como fiebre de origen desconocido, Ecografía abdominal: hepato-esplenomegalia leve; ecocardiografía transesofágica: masa calcificada de 4,5 x 8 mm en válvula aortica y leve efusión pericárdica; tomografía abdominal: hepatoesplenomegalia leve, ganglios paraaórticos abdominales y una imagen captadora de contraste en hilio renal derecho de 15 x 25 mm, de bordes definidos. Leucocitosis neutrofílica, anemia moderada, ferritina > 2 000 ng/ ml., FR, ANA Y ANCA negativos. Ganglio cervical: reacción inflamatoria inespecífica; histopatología del tumor renal: carcinoma renal de células claras. A pesar de exéresis del tumor renal, la fiebre persistía; por exclusión, se diagnosticó enfermedad de Still del adulto (ESA) asociado a endocarditis trombótica no bacteriana (ETNB); recibió prednisona 1 mg/kg, con respuesta favorable. CONCLUSION. La ESA es un trastorno inflamatorio inmune caracterizado por la triada de fiebre, exantema evanescente y artralgias, asociado a otros signos y síntomas y pruebas de laboratorio inespecíficas, pero muy sugestivas. El diagnóstico es por exclusión. El tumor renal sed a la causa de ETNB y desencadenante de la ESA.

This is a 52-year-old male, with a 9-month of illness, characterized by arthralgias, high fever, evanescent rash, pharyngitis, weight loss. Examination, lymphadenopathy; tachycardia, III/VI systolic murmur in mitral and aortic areas. It was considered as fever of unknown origin. Abdominal ultrasound: mild hepato-splenomegaly; transesophageal echocardiography: calcified mass of 4,5 x 8 mm in the aortic valve and mild pericardial effusion; abdominal tomography: mild hepato-splenomegaly, abdominal para-aortic lymph nodes and an image with contrast uptake at the level of the right renal hilum of 15 x 25 mm, with defined borders. Moderate leukocytosis with neutrophilia, moderate anemia, ferritin > 2000 ng/mL, negative for RF, ANA and ANCA. Cervical lymph node: unspecific inflammatory reaction; histopathology from the renal tumor: clear-cell renal carcinoma. Despite removal of the renal tumor, the fever persisted; by exclusion, was diagnosed as adult Still's disease (ASD) associated with non-bacterial thrombotic endocarditis (NBTE); treatment was initiated with prednisone at 1 mg/kg, obtaining positive result. Conclusion. Adult Still's disease (ASD) is an immune disorder characterized by the trio fever, evanescent rash and arthralgia or arthritis, accompanied others signs and symptom and laboratory essay. The diagnosis is made by exclusion. The renal tumor would have been the cause of the NBTE and unchain of the ASD.

Enfermedad de Still del adulto asociado a compromiso pulmonar / Adult Honest Still disease with pulmonary involvement

La enfermedad de Still del adulto es una patología sistémica poco frecuente, de etiología aún no clara que afecta principalmente a adultos jóvenes y que se caracteriza por presentar fiebre, rash evanescente color rosa salmón, odinofagia, artralgia o artritis y en algunos casos hepatoesplenomegalia y adenomegalias. En el siguiente artículo presentamos el caso de una paciente de 21 años que presentó dichas manifestaciones asociadas a compromiso pulmonar, elevación de transaminasas y ferritina, leucocitosis con anticuerpos anticitoplasma de neutrófilos (ANCAS) y anticuerpos antinucleares (ANAs) positivos que hacen dudar del diagnóstico de la enfermedad

Doença de Still do Adulto associada à neoplasia de ovário: relato de caso / Adult Still's disease associated with ovarian cancer: case report

Relatamos um caso de diagnóstico de Doença de Still do Adulto (DSA) em paciente feminina com febre, mialgia, rash cutâneo fugaz e linfonodomegalia inguinal bilateral, após extensa investigação para exclusão de outras doenças reumatológicas, infecciosas e neoplásicas. A paciente inicialmente apresentou resposta ao tratamento com prednisona, porém evoluiu com aumento de volume de linfonodos inguinais, cuja biópsia revelou adenocarcinoma seroso de ovário. De acordo com nosso conhecimento, esse é o primeiro relato de neoplasia ovariana associada ao diagnóstico de DSA.

We report a case of adult-onset Still's disease in a female patient with fever, myalgia, vanishing rash and bilateral inguinal lymphadenopathy, diagnosed after extensive workup to exclude other rheumatic, infectious and neoplastic diseases. The patient initially responded to corticosteroid therapy, but progressed to increased lymph nodes size that when biopsied, revealed serous ovarian adenocarcinoma. To our knowledge, this is the first report of ovarian neoplasm associated with adult-onset Still's disease.

Enfermedad de still del adulto: presentación de un caso y revisión de la literatura / Adult still's disease: a case report and literature review

La enfermedad de Still del adulto es una enfermedad inflamatoria con compromiso sistémico de origen desconocido, de poca frecuencia en la población general y evolución variable,desde un curso relativamente benigno, hasta enfermedad severa con complicaciones que pueden ser mortales. Se presenta un caso de un paciente de 49 años quien ingresó por un cuadro febril prolongado de un mes de evolución, y dentro de los estudios para fiebre de origen desconocidose descartaron diferentes etiologías, cumpliendo criterios para el diagnóstico de enfermedad de Still del adulto. Posteriormente se hace una revisión de la literatura, evaluando la epidemiología, etiología, clínica, hallazgos de laboratorio, diagnóstico, tratamiento y pronóstico de la enfermedad de Still del adulto.

Adult Still’s disease is an inflammatory disease with systemic involvement of unknown origin, not frequent in the general population and with variable evolution, from a relatively benigncourse to a severe disease with potentially fatal complications. We present a case of 49-yearoldpatient who consulted due to a 1-month history of prolonged febrile illness, and among the diagnostic work-up for fever of unknown origin, different etiologies were ruled out while fulfillingthe criteria for diagnosis of adult Still’s disease. Furthermore, we review the literature on the epidemiology,etiology, clinical manifestations, laboratory findings, diagnosis, treatment and prognosis of adult Still’s disease.