RESUMO
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.
Assuntos
Doença de Still de Início Tardio , Adulto , Feminino , Humanos , Masculino , População do Caribe/estatística & dados numéricos , Etnicidade , Glucocorticoides/uso terapêutico , Martinica/epidemiologia , Estudos Retrospectivos , Doença de Still de Início Tardio/diagnóstico , Doença de Still de Início Tardio/tratamento farmacológico , Doença de Still de Início Tardio/epidemiologia , Doença de Still de Início Tardio/etnologia , Índias Ocidentais/epidemiologiaRESUMO
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.
Assuntos
Humanos , Feminino , Adulto , Adulto Jovem , Doença de Still de Início Tardio/diagnóstico , Aspartato Aminotransferases/sangue , Fator Reumatoide/sangue , Esplenomegalia , Sedimentação Sanguínea , Proteína C-Reativa/análise , Faringite , Doenças Reumáticas/diagnóstico , Doença de Still de Início Tardio/tratamento farmacológico , Corticosteroides/uso terapêutico , Artralgia , Antirreumáticos/uso terapêutico , Doenças Raras/diagnóstico , Diagnóstico Diferencial , Alanina Transaminase/sangue , Exantema , Febre , Hiperferritinemia/sangue , Infecções/diagnóstico , Leucocitose/sangue , Neoplasias/diagnósticoRESUMO
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.
Assuntos
Humanos , Masculino , Adulto , Doença de Still de Início Tardio/diagnóstico , Doença de Still de Início Tardio/tratamento farmacológico , Corticosteroides/uso terapêutico , Artralgia , Exantema , Febre , Febre de Causa Desconhecida , Anti-Inflamatórios/uso terapêuticoRESUMO
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.
Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Doença de Still de Início Tardio/diagnóstico , Doença de Still de Início Tardio/tratamento farmacológico , Prognóstico , Prednisona/uso terapêutico , Resultado do TratamentoRESUMO
Adult-onset Still's disease (AOSD) patients typically present with arthralgia, fever, lymphadenopathy and a transient salmon maculopapular rash. Only approximately 25 cases of AOSD with urticaria were described in the literature. In this article, the authors report three additional cases of AOSD with urticarial and dermographic lesions who had a good clinical response to glucocorticoid and antihistamines. A review of the literature concerning this issue is also herein written.
Assuntos
Pele/patologia , Doença de Still de Início Tardio/complicações , Urticária/etiologia , Adulto , Diagnóstico Precoce , Feminino , Glucocorticoides/uso terapêutico , Antagonistas dos Receptores Histamínicos/uso terapêutico , Humanos , Masculino , Valor Preditivo dos Testes , Pele/efeitos dos fármacos , Doença de Still de Início Tardio/diagnóstico , Doença de Still de Início Tardio/tratamento farmacológico , Resultado do Tratamento , Urticária/diagnóstico , Urticária/tratamento farmacológicoRESUMO
Still's disease (SD) is a rare chronic inflammatory disease characterized by polyarthritis, systemic symptoms, and elevated inflammatory markers. Of note, 74 SD cases were reported with anti-tumoral necrosis factor (TNF) therapy and the experience of switching is limited to five patients. During a 3-year period, SD cases were 1.9% of 319 rheumatic patients that received anti-TNF agents in the infusion center of our University Hospital. In this manuscript, the authors add six new cases of refractory SD who had clinical and laboratory response to TNF blockers and report the outcome of switching in five of them. Partial or complete response was achieved by four of six (66.7%) patients and three of four (75%) required switching. Regarding safety, five of six (83.3%) had adverse events. Anti-TNF treatment with switching seems to be a valid approach for refractory SD patients.
Assuntos
Anticorpos Monoclonais/efeitos adversos , Antirreumáticos/efeitos adversos , Substituição de Medicamentos , Doença de Still de Início Tardio/tratamento farmacológico , Fator de Necrose Tumoral alfa/antagonistas & inibidores , Adulto , Resistência a Medicamentos , Feminino , Humanos , Masculino , Resultado do Tratamento , Adulto JovemRESUMO
In this case report, the authors described the first case of a patient with adult-onset Still's disease (AOSD) who presents advanced periodontal infection. AOSD is a rare systemic inflammatory disorder of unknown aetiology, characterised by spiking fever, usually exceeding 39°C, an evanescent salmon pink rash, arthritis and multiorgan involvement. Periodontal infection is a pathogen-induced oral inflammatory disease affecting the supporting tissues of teeth and is currently considered as a risk factor for cardiovascular disease. Several cytokines capable of inducing systemic effects are produced during the course of this infection and the values of serum markers of inflammation, such as C reactive protein (CRP), may significantly decrease after periodontal treatment. Although AOSD can produce elevations in CRP, similar increase may be produced by periodontal infection, suggesting the need for medical and dental diagnosis when evaluating the sources of acute-phase responses in systemic autoimmune disease patients.
Assuntos
Doenças Periodontais/etiologia , Doença de Still de Início Tardio/complicações , Anti-Inflamatórios/administração & dosagem , Anti-Inflamatórios/uso terapêutico , Biomarcadores/sangue , Proteína C-Reativa/análise , Prótese Dentária , Quimioterapia Combinada , Feminino , Humanos , Metotrexato/administração & dosagem , Metotrexato/uso terapêutico , Doenças Periodontais/terapia , Prednisona/administração & dosagem , Prednisona/uso terapêutico , Doença de Still de Início Tardio/diagnóstico , Doença de Still de Início Tardio/tratamento farmacológico , Adulto JovemRESUMO
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% had odynophagia, 80% had an evanescent rash, 70% had myalgias, 50% had lymph node enlargement and 40% had splenomegaly. Laboratory showed leukocytosis in 80% and a high erythrocyte sedimentation rate in all. High ferritin levels were detected in 80%, and became an important diagnosis clue. Initial treatment was based on non steroidal antiinflammatory drugs, however 80% required steroids and 35% required methotrexate. Azathioprine, sulphalazine, hydroxychloroquine and leflunomide 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.
Assuntos
Doença de Still de Início Tardio/diagnóstico , Adolescente , Adulto , Anti-Inflamatórios não Esteroides/uso terapêutico , Biomarcadores/sangue , Feminino , Ferritinas/sangue , Febre de Causa Desconhecida/diagnóstico , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Doença de Still de Início Tardio/sangue , Doença de Still de Início Tardio/tratamento farmacológico , Adulto JovemRESUMO
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.
Assuntos
Adolescente , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Adulto Jovem , Doença de Still de Início Tardio/diagnóstico , Anti-Inflamatórios não Esteroides/uso terapêutico , Biomarcadores/sangue , Ferritinas/sangue , Febre de Causa Desconhecida/diagnóstico , Estudos Retrospectivos , Doença de Still de Início Tardio/sangue , Doença de Still de Início Tardio/tratamento farmacológico , Adulto JovemRESUMO
Still's disease is very rare in elderly patients. We report a case of Still's disease in an elderly patient that had an atypical initial presentation. A 76-year-old woman developed unexplained chronic anemia and thrombocytosis. Three years later she had acute onset of high fever, arthritis, maculopapular rash, pleuritic chest pain, abdominal pain, lymphadenopathy and elevated erythrocyte sedimentation rate. Rheumatoid factor and antinuclear antibodies were negative. She responded favorably to prednisone and methotrexate treatment. Anemia and thrombocytosis as well as Still's disease manifestations resolved.