1/12. urticaria as a presenting manifestation of adult-onset Still's disease.adult-onset Still's disease (AOSD) is a rare disorder of unknown aetiology, characterised by high spiking fever, an evanescent, erythematous, maculopapular rash, arthralgia or arthritis, lymphadenopathy, hepatosplenomegaly, sore throat and serositis. It is associated with marked leukocytosis, high erythrocyte sedimentation rate, increased level of serum ferritin and negative rheumatoid factor and antinuclear antibody tests. Here we report a patient in whom an urticaria-like rash was an uncommon presenting clinical feature of AOSD. To our knowledge, this association has only been reported once before.- - - - - - - - - - ranking = 1keywords = sore throat, throat (Clic here for more details about this article) |
2/12. Bilateral empyema caused by adult-onset Still's disease.Empyemas are usually of infectious origin. Noninfectious causes of empyema may be a considerable diagnostic challenge, as exemplified in the present case report. A 21-year-old male presented with high fever, sore throat and myalgias of 1 week duration. In the following days, bilateral pleural effusions developed, with cellular counts in the pleural fluid up to 117 x 10(9)/liter (98% neutrophils). Despite institution of empiric antibiotic therapy, spiking fever continued. All culture studies resulted in being negative. Following the report of a serum ferritin concentration of 6,975 microg/l, adult-onset Still's disease was diagnosed and successfully treated with anti-inflammatory drugs. This case adds adult-onset Still's disease to the list of noninfectious causes of empyema and underlies the value of measuring serum ferritin as a diagnostic tool.- - - - - - - - - - ranking = 1keywords = sore throat, throat (Clic here for more details about this article) |
3/12. Infliximab in the treatment of adult Still's disease refractory to conventional therapy.In this study we evaluated the efficacy of Infliximab in the treatment of adult Still's disease (ASD) refractory to conventional therapy. Three patients with chronic and active ASD unresponsive to corticosteroids and methotrexate were given intravenous Infliximab infusions at a dosage of 3 mg/kg at weeks 0, 2, 6 and then once every 8 weeks. methotrexate was maintained in all cases at a dosage of 15 mg/week, whereas the prednisone dose was modified according to disease activity. The follow-up lasted 50 weeks and disease activity improved in all cases during Infliximab therapy. Two patients presented arthralgias and sore throat at 20 and 28 weeks, that was rapidly controlled by Infliximab reinfusion every 4 weeks. One patient relapsed at 18 weeks and dropped out at 22 weeks due to an urticarioid rash after the beginning of the fifth infusion. Infliximab may be effective in the treatment of relapse of ASD refractory to conventional therapy and requiring continuous high dose corticosteroid medication. Further studies are needed to evaluate the long-term safety, efficacy and the optimal schedule of infusion.- - - - - - - - - - ranking = 1keywords = sore throat, throat (Clic here for more details about this article) |
4/12. miller fisher syndrome in adult onset Still's disease: case report and review of the literature of other neurological manifestations.adult-onset Still's disease (AOSD) is a multi-system inflammatory disorder characterized by high spiking fevers, evanescent salmon-coloured rash, arthralgias or arthritis, hepatosplenomegaly, lymphadenopathy and sore throat. There is no specific test or combination of tests that can establish the diagnosis of AOSD and patients may present with other systemic involvement including neurological manifestations in 7-12% of cases. We present a complex case of a patient with AOSD who developed the Miller-Fisher variant of guillain-barre syndrome. This immunological disorder of the nervous system has not been described in association with AOSD before. We also review the literature on other neurological manifestations in AOSD. AOSD mimics different disease processes and its multi-system manifestations may complicate the picture further.- - - - - - - - - - ranking = 1keywords = sore throat, throat (Clic here for more details about this article) |
5/12. liver failure in adult Still's disease during corticosteroid treatment.adult Still's disease is a well-characterized rheumatic disorder of unknown origin, which may affect multiple organs and may have a fatal course. However, liver failure has rarely been described in adult Still's disease. We present the case of a 25-year-old woman who was admitted with acute liver failure 2 years after the start of symptoms (arthritis, fever, sore throat) of a yet undefined rheumatic disease. She had been treated with prednisolone for 2 months before admission. The diagnosis of adult Still's disease was made in accordance with well-established criteria. Other causes of liver failure were excluded. Withdrawal of prednisolone did not affect the course of liver disease. ursodeoxycholic acid therapy was started when the patient slowly began to recover. To the best of our knowledge, this is the first case of adult Still's disease reported in which hepatic failure developed when other symptoms were well controlled by corticosteroid treatment.- - - - - - - - - - ranking = 1keywords = sore throat, throat (Clic here for more details about this article) |
6/12. Chronic relapsing thrombotic thrombocytopenic purpura in adult onset Still's disease.We report the first known case of chronic relapsing thrombotic thrombocytopenic purpura associated with adult-onset Still's disease. The patient presented with diffuse arthralgias, sore throat, and a maculopapular rash involving the trunk and extremities; she was hospitalized with fever and confusion. thrombocytopenia, renal failure, and microangiopathic hemolytic anemia developed within several days. After a diagnosis of thrombotic thrombocytopenic purpura was made, she responded well to a series of plasma exchanges. Evaluation for infection, autoimmune disorders, and malignancy was negative. She was discharged to home in good condition, with normal renal function and normal platelet count. Two more episodes of TTP developed 7 and 9 months after the first hospitalization. The diagnosis of adult-onset Still's disease was then determined on the basis of clinical and laboratory criteria. She was successfully treated with plasma exchange, prednisone, and azathioprine. She later had splenectomy and has subsequently been without recurrence of thrombotic thrombocytopenic purpura for 2 years.- - - - - - - - - - ranking = 1keywords = sore throat, throat (Clic here for more details about this article) |
7/12. Successful treatment with cyclosporin in adult-onset Still disease manifesting as acute hepatitis with marked hyperferritinemia.A 48-year-old woman was admitted because of spiking high fever, sore throat, and jaundice. A diagnosis was made of adult-onset Still disease (AOSD) presenting with acute hepatitis and very high serum ferritin levels (32,240 ng/mL), and she was treated with 2 courses of pulse therapy of methylprednisolone (2 g/day for 3 days) followed by 40 mg/day prednisolone. Subsequently, the serum level of ferritin decreased, but serum total bilirubin increased to 17.3 mg/dL. Therefore, cyclosporin was administered orally. Within the next 3 months, results of liver function tests, as well as serum levels of ferritin, soluble interleukin-2 receptor, interferon-gamma, interleukin-6, and tumor necrosis factor-alpha gradually returned to within normal limits, and cyclosporin administration was subsequently reduced gradually. The clinical presentation suggests that AOSD should be considered when liver dysfunction is accompanied with high fever and extreme hyperferritinemia, and that treatment with cyclosporin or other immunosuppressive drugs that selectively suppress cytokine production by helper T cells is a valuable option in the treatment of AOSD with very high serum ferritin levels.- - - - - - - - - - ranking = 1keywords = sore throat, throat (Clic here for more details about this article) |
8/12. A pruritic linear urticarial rash, fever, and systemic inflammatory disease in five adolescents: adult-onset still disease or systemic juvenile idiopathic arthritis sine arthritis?The characteristic rash of systemic juvenile idiopathic arthritis is a transient erythematous eruption associated with a quotidian spiking fever. Usually asymptomatic, it can be pruritic, with dermatographism at sites of scratching or pressure. An illness similar to this entity in adults is designated adult-onset Still disease. The relationship between the pediatric and adult disease is uncertain and differences in case definition have evolved. Specifically, a sustained arthritis for at least 6 weeks is required for a diagnosis of systemic juvenile idiopathic arthritis, whereas transient arthritis and arthralgia are accepted criteria in adult-onset Still disease. We describe five patients less than 16 years of age who presented with an acute illness characterized by fever and a distinctive skin eruption. Intense pruritus and linear erythematous lesions flared with a spiking fever, usually in the late afternoon and evening. Periorbital edema/erythema and nonlinear urticarial lesions were also seen. Two children had splinter hemorrhages of the nail beds and one girl developed a fixed, scaling, pigmented, linear eruption. Severe malaise, myalgia, arthralgia, and leukocytosis were present in every patient. Other systemic manifestations included sore throat, transient arthritis, abdominal pain, lymphadenopathy, hepatomegaly, splenomegaly, hyperferritinemia, and hepatic dysfunction. No patient had a sustained arthritis. The course of the disease was variable. One patient, diagnosed with macrophage activation syndrome, recovered on oral naproxen. Two patients responded to systemic corticosteroid therapy. One girl developed status epilepticus and died from aspiration and asphyxia. A boy with severe hepatitis developed renal failure and thrombotic thrombocytopenic purpura and was treated with plasmapheresis, dialysis, and systemic corticosteroids; he had recurrent episodes of rash and fever into adult life. These children did not fulfill the case definition of systemic juvenile idiopathic arthritis because they lacked a persistent arthritis. adolescent and adult patients with the same clinical and laboratory findings are described under the rubric of adult-onset Still disease. Recognition of the distinctive urticarial skin eruption and spiking fever is important in the diagnosis of a disease with severe morbidity and potentially life-threatening complications.- - - - - - - - - - ranking = 1keywords = sore throat, throat (Clic here for more details about this article) |
9/12. Myopericarditis revealing adult-onset Still's disease.We report the case of a 33 year-old man presenting with fever, arthralgias, sore throat, dyspnea and chest pain. Myopericarditis was diagnosed on the basis of electrocardiogram, echocardiography and cardiac magnetic resonance imaging (MRI). The dramatic increase in ferritinemia suggested the diagnosis of adult-onset Still's disease (AOSD), which could be established according to criteria of Yamaguchi. Rapid improvement occured under corticosteroids. myocarditis is very rare in AOSD, pericarditis being more frequent. We report herein the first description of AOSD associated myocarditis evidenced by cardiac MRI.- - - - - - - - - - ranking = 0.020459653667754keywords = throat (Clic here for more details about this article) |
10/12. Atypical presentation of adult Still's disease mimicking acute bacterial endocarditis.adult Still's disease is a chronic, systemic disease of unknown origin. We describe the case of an otherwise healthy man with an uncommon presentation of Still's disease. A 38-year-old man presented with sore throat, fever, rash and arthritis. Laboratory findings showed that both erythrocyte sedimentation rate and ferritin had increased. Transoesophageal echocardiography revealed a vegetation involving the aortic leaflet. The diagnosis of Still's disease was made after the exclusion of infectious endocarditis, based upon the clinical picture, the high level of ferritin and the follow-up. The patient markedly improved after treatment with prednisone 1 mg. kg-1. This controlled and then progressively reduced the disease; the drug was then withdrawn. This case illustrates that Still's disease can present with endocardial involvement mimicking acute bacterial endocarditis as a first clinical manifestation. The observation suggests that the presence of high ferritinaemia in a patient with some clinical criteria of Still's disease could lead to an early diagnosis.- - - - - - - - - - ranking = 1keywords = sore throat, throat (Clic here for more details about this article) |
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