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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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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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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.

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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.

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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.

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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.

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Still's disease is a subset of juvenile idiopathic arthritis (JIA) that usually presents with intermittent fever, rash, and arthritis. Extra-articular flares can occur several years after disease onset. We report two cases of adult Still's disease with myocarditis after several years of being in remission. A 34-year-old Caucasian man with history of systemic juvenile arthritis in remission since age 13 was admitted in hospital with 10 days history of fever, odynophagia, and arthralgias. Chest X-ray and cardiac ultrasound showed cardiac enlargement. An endomyocardial biopsy revealed acute myocarditis. He was treated with methylprednisolone and intravenous gammaglobulin, with improvement of his general condition and cardiac parameters. A 16-year-old Caucasian male patient with history of systemic JIA in remission for the last 7 years was admitted with 7 days history of fever, odynophagia, arthralgias, and myalgias. Two days after admission, he developed chest pain and pericardial rubbing was found on examination. Cardiac ultrasound showed left ventricular dilatation with impaired systolic function, and posterior, inferior and apical-septal wall hypokinesia. Blood test showed elevated creatine phosphokinase levels. He was treated with IV methylprednisolone with normal follow-up cardiac ultrasound. Cardiac involvement in patients with systemic JIA can be the first symptom of disease reactivation, even after many years of disease remission.

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Hemophagocytic Lymphystiocytosis is a rare and fatal complication of rheumatic diseases, particularly Adult Onset Still's Disease (AOSD). It may be precipitated with immunosuppressive drugs and with viral and bacterial infections. A diagnosis depends on a high index of suspicion associated to certain clinical manifestations (fever, rash, Splemomegaly, any cytology blood dyscrasia, hipertrigliceridemia, hiperfibrinogenemia, and others), as well as pathologic evidence of hemophagocitosis from bone marrow biopsy or tissue samples of affected organs. Therapy consists of high dose corticosteroids and immunosuppressive drugs. We present a 42 year old woman with AOSD in remission who developed HLH in spite of receiving therapy with high dose steroids and immunosuppressive drugs. She had 2 negative bone marrow aspirates. Evidence of Hemophagocytosis was detected in both bone marrow biopsies. Timely evaluation and recognition of the signs and symptoms of HLH is crucial for the prompt management and a decrease in the mortality associated with this disease.

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