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1/126. adult onset Still's disease presenting as aseptic meningitis in a young healthy female.

    A 20-year-old white female presented with symptoms of upper respiratory tract infection, meningeal signs, rash, and fever. Initial laboratory data revealed a leukocytosis and abnormal CSF. An initial working diagnosis of the Aseptic meningitis Syndrome was made. She did not respond to antimicrobial therapy. All culture results and viral titers were negative. One week into her hospital course, the diagnosis of adult Onset Still's disease (AOSD) was made. Antibiotics were discontinued and nonsteroidal anti-inflammatory drugs (NSAIDS) begun. The patient showed marked improvement within 24 hours. This case reveals that AOSD is an important consideration in the differential diagnosis of Aseptic meningitis. Meningeal signs and abnormal cerebrospinal fluid (CSF), both detected in this patient, are very rare occurrences in Still's disease. ( info)

2/126. Severe systemic inflammatory response syndrome with shock and ARDS resulting from Still's disease: clinical response with high-dose pulse methylprednisolone therapy.

    adult-onset Still's disease, the adult variant of the systemic form of juvenile arthritis, is an uncommon systemic inflammatory disorder of unknown etiology characterized by high spiking fevers, neutrophilic leukocytosis, arthritis, and an evanescent rash. There is often a delay in reaching a firm diagnosis. Differential diagnoses include infection, malignancy, and various immunologic disorders. Increased ferritin levels are of particular value in establishing the diagnosis. Clinical response to high-dose corticosteroids may be dramatic. We report a case of a 29-year-old woman who had recently been investigated for fever of unknown origin, and who presented to our hospital with high fever and hypotension. Her condition rapidly deteriorated with the development of ARDS, disseminated intravascular coagulation, and shock. The patient had a markedly elevated serum ferritin concentration of 26,000 ng/mL. High-dose pulse methylprednisolone therapy resulted in a remarkable clinical improvement. Such a severe case of systemic inflammatory response syndrome, masquerading as septic shock, has not been reported previously. ( info)

3/126. calcinosis cutis and intestinal pseudoobstruction in a patient with adult onset Still's disease associated with recurrent relapses of disordered coagulopathy.

    adult onset Still's disease (AOSD) is a systemic inflammatory disorder of unknown origin, characterized by a typical spiking fever, evanescent salmon-colored rash, polyarthralgia, and myalgia. calcinosis cutis and gastrointestinal involvement have rarely been noted in AOSD. We herein describe a 54-year-old woman who demonstrated repeated disseminated intravascular coagulation (DIC), and adult respiratory distress syndrome (ARDS), associated with AOSD. The patient also revealed a remarkable degree of digital calcinosis cutis and intestinal pseudoobstruction. A connective tissue disease, such as systemic sclerosis, might have been the underlying factor in the latter two symptoms. ( info)

4/126. Severe hepatitis and pure red cell aplasia in adult Still's disease: good response to immunosuppressive therapy.

    adult-onset Still's disease is a systemic inflammatory disorder with a highly variable clinical course. Mild hepatitis and anemia are common manifestations. We describe a patient with adult Still's disease who developed a severe hepatitis and a life-threatening pure red cell aplasia. The hepatitis developed after treatment with NSAIDs was started. The patient was successfully treated with a combination of prednisone, cyclosporin, and methotrexate. physicians should be aware that severe hepatitis and pure red cell aplasia can occur in adult Still's disease. We recommend a careful monitoring of liver functions in patients with adult Still's disease who are being treated with NSAIDs. ( info)

5/126. Bone scintigraphy and magnetic resonance imaging in adult-onset Still's disease.

    adult-onset Still's disease (AOSD) is an acute systemic inflammatory disorder of unknown origin. We report a patient whose AOSD presented with the commonly accepted diagnostic clinical signs and laboratory parameters. The painful joints distinctly demonstrated increased uptake of 99mTc-methylene diphosphonate in scintigraphy and areas of increased gadolinium-enhanced signal in MRI. Biopsies indicated bone marrow edema. AOSD in association with bone marrow edema had not been previously demonstrated. AOSD is often diagnosed after a considerable delay, bone scintigraphy, and magnetic resonance imaging may offer new imaging techniques for early diagnosis and successful therapy in follow-up examinations. ( info)

6/126. adult-onset Still's disease with persistent plaques.

    adult-onset Still's disease (AOSD) is a systemic disorder characterized by intermittent fever, evanescent rash, arthralgias or arthritis and predominantly neutrophilic leucocytosis. We report on a 16-year-old woman with Still's disease who developed, in addition to the typical rash, persistent papular lesions on her face, neck and upper and lower back. Although the presence of fixed skin lesions is not a characteristic feature of AOSD, their appearance at the onset of the disease and their evolution suggest that they represent a specific manifestation of the disease. ( info)

7/126. Localised hyaline vascular type of Castleman's disease mimicking adult-onset Still's disease.

    A previously healthy 18-year-old boy presented with daily spiking fever, polyarthritis, and evanescent skin rashes, as well as hepatomegaly and Raynaud's phenomena for 2 months. He was initially diagnosed with adult-onset Still's disease (AOSD). During the period of follow-up, intermittent fever and migratory polyarthritis persisted and an insidiously growing mass over the right axillary region was noted 1 year after the diagnosis of AOSD. Excisional biopsy of the mass revealed a group of lymph nodes with histological features of the hyaline vascular type of Castleman's disease. The patient's symptoms disappeared soon after excision of the lymph nodes. evanescent rash, lymphadenopathy, hepatosplenomegaly and serositis [5]. A clinical picture compatible with the diagnosis of AOSD has not been described in the localised hyaline vascular type of Castleman's disease. We report such a case in an 18-year-old male patient who presented prolonged fever and polyarthritis with an initial diagnosis of AOSD. The diagnosis of hyaline vascular type of Castleman's disease was made 1 year later, when the patient developed an insidiously growing mass over the right axilla. ( info)

8/126. A family with cases of adult onset Still's disease and psoriatic arthritis.

    adult onset Still's disease is recognized as an adult variant of the systemic form of juvenile rheumatoid arthritis, whose disease-predisposition is still debated. On the other hand, the association between HLA subtypes and several groups of seronegative arthritis including psoriatic arthritis has been well documented. This report describes a family where adult onset Still's disease in a young man and psoriatic arthritis in his father were seen. Both patients were HLA-B39-positive, which was likely playing important pathogenic roles in the latter case. Clinical and immunological aspects of HLA-B39-related inflammatory diseases are also discussed. ( info)

9/126. Acute hepatitis in adult Still's disease apparently resulting from oral iron substitution--a case report.

    The authors report a case of a young woman with adult-onset Still's disease (AOSD) with massive hyperferritinaemia who developed acute florid hepatitis with intraparenchymatous histiocytic infiltration following oral iron substitution for presumed iron deficiency, which settled on withdrawal of the iron. This suggests that the iron exacerbated the macrophage hyperactivity which is presumed to be present in AOSD. Oral iron substitution in the acute phase of this disease may be inadvisable. ( info)

10/126. Prolonged remission state of refractory adult onset Still's disease following CD34-selected autologous peripheral blood stem cell transplantation.

    We report a 38-year-old patient affected by refractory adult onset Still's disease who achieved a prolonged remission following CD34-selected ABMT. The conditioning regimen was based on the use of CY and anti-thymocyte globulin. A 3.0 and 2.0 log reduction of T (CD3 ) and B (CD19 ) lymphocytes, respectively, was obtained using a Ceprate device to select CD34 cells from PBSC. In the pre-transplant period (1994-1998) the patient had a chronic persistent disease course with frequent and recurrent systemic articular flares and loss of some functional abilities, despite daily prednisone, pulses of CY and immunosuppressive therapy (CYA or MTX). At the time of ABMT the patient had become non-ambulatory. Within 3 weeks of ABMT the patient showed a marked decrease in joint swelling, and morning stiffness. Joint pain and systemic symptoms disappeared, the patient was able to walk and run and gained general well being. ESR, c-reactive protein and WBC count were significantly decreased, while Hb level increased. This partial remission persisted for at least 1 year after ABMT, although at 15 months of follow-up a reappearance of moderate synovitis in the knees and wrists was noted. Our data further showed that both patient BM microenvironment and stem-progenitor cell function (as assessed by LTC-IC assay) were damaged even 1 year after CD34-selected ABMT, suggesting that the persistence of these alterations could have facilitated the favorable outcome of the disease following ABMT. bone marrow transplantation (2000) 25, 1307-1310. ( info)
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