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.

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.

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.

Adult Onset Stills Disease: A Case Report.

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

Enfermedad de Still del adulto: una gran simuladora: experiencia clínica basada en 20 casos / Adult Still's disease: retrospective review of 20 patients

Background: Adult Still's disease is an inflammatory disorder characterized by quotidian fevers, and an evanescent rash. Its presentation can be acute or subacute. Aim: To report our experience with Adult Still's disease. Material and methods: Systematic retrospective review of medical records of nine men and 11 women aged between 17 and 57 years, with Still's disease, followed in two public hospitals of Metropolitan Santiago. Results: Eighty percent of patients had a prior different diagnosis. All presented with fever and joint involvement. Eighty percent had malaise, 80 percent had odynophagia, 80 percent had an evanescent rash, 70 percent had myalgias, 50 percent had lymph node enlargement and 40 percent had splenomegaly. Laboratory showed ¡eukocytosis in 80 percent and a high erythrocyte sedimentation rate in all. High ferritin levels were detected in 80 percent, and became an important diagnosis clue. Initial treatment was based on non steroidal antiinflammatory drugs, however 80 percent required steroids and 35 percent required methotrexate. Azathioprine, sulphalazine, hydroxychloroquine and ¡eflunomide were used occasionally. Eleven patients had a single episode, nine had a relapsing disease and four had a chronic or persistent mode. Conclusions: Adult Still's disease must be suspected in patients with fever of unknown origin. An early diagnosis and adequate treatment of the disease are associated with a favorable evolution and prognosis.

[ adult onset still's disease. 11 cases]

The stills' disease of the adult is a systemic disease that the couse is unknown. If the pediatric forms are frequent, the affection of the adult is rare and it's diagnosis is difficult. The purpose of this study is to analysis the clinical and evaluative features of the adult still's disease and to compare our results with the literature it is retrospective study about 21 years that permitted to collect 11 cases according the criteria of yamaguchi. It is matter of 6 men and 5 women that the mean age is 31 years old [age range: 16 to 48 years old] the fever was constant. The skin rash noticed in 8 patients [72.7%] a polyarthritis was noticed in all the patients. Chronic in 10 cases [90.9%] which 2 erosion forms adenopathies were present in 5 patients, a splenomegaly and hepatomegly were noticed respectively in 4 and 2 cases. Inflammation syndrome with hyperleukocytosis was constant - and a hepatic cytolysis was noticed in 80% the total ferritinemia titrated in 8 patients was constantly high. The hemocultures realized in all the patients were sterile and the complete immunology examination was negative. The strong dose of corticoid was prescrited with sucess in all the patients. The immediate evolution was favourable in 10 patients. We depolore one death after astate of deep denutrution. The still's disease of the adult is rare, it's diagnosis is difficult, sensible to the corticotherapy and it's clinicoevolution aspect in our country is comparable to the literature

Osteoporosis vertebral inducida por corticoides y ciclosporina en una paciente con enfermedad de Still / Vertebral osteoporosis induced by corticoids and cyclosporine in a patient with Still diseases

Se presenta el caso de una paciente de 29 años quien recibió prednisona y ciclosporina en altas dosis como tratamiento de la enfermedad de Still. Consultó por dolores invalidantes. Se comprobaron fracturas vertebrales múltiples, densidad mineral ósea lumbar descendida en rango de osteoporosis, alta remodelación ósea e hipercalciuria. Fue tratada con pamidronato por vía endovenosa (225 mg en tres meses), calcitonina nasal (200-400 UI/día), tiazidas (25 mg/día) y kinesioterapia. Se obtuvo una rápida disminución del dolor y la paciente se movilizó por sus propios medios. Se discuten estas indicaciones en el marco de los conocimientos actuales sobre enfermedades metabólicas del hueso.

Enfermedad de Still del adulto / Still's disease of the adult

Se revisa el cuadro clínico de la enfermedad de Still del adulto, destacando que básicamente es una poliartritis simétrica, seronegativa, de evolución febril. Es una afección poco frecuente pero no excepcional que hay que tener presente frente a síndromes febriles prolongados de etiología no infecciosa. Es mucho menos frecuente que la artritis reumatoídea, el lupus y la esclerosis sistémica pero se equipara con la polidermatomiositis, los síndromes de sobreposición y la sarcoidosis

Corticotherapie et maladies systemiques

Corticosteroid are an essential mean to overcome systemic disorders. Thirty years ago they changed the prognosis of most of these diseases. Adverse effects are numerous and dangerous, especially infectious diseases and bone disorders. Prednisone is the most used drug. The pulse of methyprednisone is less used. In this study, we discuss some principles of the use of corticosteroid and the problems encountered using corticotherapy in systemic disorders