Cases reported "Rheumatic Diseases"

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1/17. mycobacterium tuberculosis infection in patients with systemic rheumatic diseases. A case-series.

    OBJECTIVE: To describe the clinical characteristics of patients with systemic rheumatic diseases and tuberculosis. A retrospective case series from 1987 to 1994, drawn from a tertiary-care hospital in mexico City, was studied. RESULTS: Thirty patients were included (20 women, 10 men), with mean age of 39.8 years (range 14-66), and a mean duration of the systemic rheumatic disease of 44 months (1-372). The rheumatic diseases included systemic lupus erythematosus (SLE) (n = 13), rheumatoid arthritis (7), polymyositis or dermatomyositis (5), and other diseases (5). During the six months previous to the diagnosis of tuberculosis, 22 patients had received corticosteroids, and 13 others immunosuppressants. mycobacterium tuberculosis was isolated from 18 patients. Pulmonary tuberculosis was found in 10 patients, and extrapulmonary tuberculosis in 20, seven of these with miliary disease. SLE was seen in 6 of the patients with miliary tuberculosis. The clinical manifestations were: fever (67%), weight loss (67%), diaphoresis (60%), cough and sputum (53%), lymph node enlargement (43%), and dyspnea (33%). Sixteen patients had an abnormal chest film. Of 18 patients tested by PPD RT-2, 8 had an induration > 10 mm. patients were initially treated with 3 or 4 anti-tuberculosis drugs for 15 days to 6 months, followed by 6 to 10 months of isoniazid plus rifampicin. Three relapsed, and 2 died of respiratory failure. CONCLUSIONS: This case series showed a particular pattern of tuberculosis in patients with systemic rheumatic diseases.
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ranking = 1
keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus
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2/17. Infectious CNS disease as a differential diagnosis in systemic rheumatic diseases: three case reports and a review of the literature.

    BACKGROUND: Immunosuppressive treatment of rheumatic diseases may be associated with several opportunistic infections of the brain. The differentiation between primary central nervous system (CNS) involvement and CNS infection may be difficult, leading to delayed diagnosis. OBJECTIVE: To differentiate between CNS involvement and CNS infection in systemic rheumatic diseases. methods and results: Three patients with either longstanding or suspected systemic rheumatic diseases (systemic lupus erythematodes, Wegener's granulomatosis, and cerebral vasculitis) who presented with various neuropsychiatric symptoms are described. All three patients were pretreated with different immunosuppressive drugs (leflunomide, methotrexate, cyclophosphamide) in combination with corticosteroids. magnetic resonance imaging of the brain was suggestive of infectious disease, which was confirmed by cerebrospinal fluid analysis or stereotactic brain biopsy (progressive multifocal leucoencephalopathy (PML) in two and nocardiosis in one patient). DISCUSSION: More than 20 cases of PML or cerebral nocardiosis in patients receiving corticosteroids and cytotoxic drugs for rheumatic disease have been reported. The clinical aspects of opportunistic CNS infections and the role of brain imaging, cerebrospinal fluid analysis and stereotactic brain biopsy in the differential diagnosis are reviewed.
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ranking = 0.087409682493242
keywords = systemic lupus, lupus
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3/17. fibrosis regression induced by intravenous gammaglobulin treatment.

    OBJECTIVES: To review case histories of patients in whom fibrosis played a significant role in the pathogenesis of their disease, and to determine whether intravenous gammaglobulin (IVIg) contributed to the regression of their fibrotic condition. methods: Eight patients with excess fibrotic reaction in the course of diverse diseases were analysed; a tendency that reverted with different IVIg treatment options. Myelofibrosis was predominant in three patients (a patient with a myeloproliferative syndrome, one with systemic lupus erythematosus, and one with sjogren's syndrome). Three patients had scleroderma as their main feature, one patient had hepatitis c cirrhosis, and one had idiopathic thrombocytopenic purpura. RESULTS: Fibrotic excess was reduced in all the patients by IVIg treatment. In five patients the disease as a whole benefited from the infusion of immunoglobulins. CONCLUSION: IVIg may enhance resorption of fibrosis and promote healing in patients with fibrotic associated disorders.
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ranking = 1
keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus
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4/17. Kaposi's sarcoma in rheumatic diseases.

    OBJECTIVE: To review the clinical features and outcome of all reported cases of Kaposi's sarcoma in patients with rheumatic diseases. methods: In addition to our patient, we identified cases from a medline search between the years 1966 and 2002. Cases associated with human immunodeficiency virus infection were excluded. RESULTS: Including our patient, there were a total of 25 cases reported (11 men and 14 women). Rheumatoid arthritis was present in 8 cases, polymyositis/dermatomyositis in 5, vasculitis syndromes in 5, systemic lupus erythematosus in 3, polymyalgia rheumatica in 2, and 1 each of undifferentiated connective tissue disease and Behcet disease. All but 1 patient had been given systemic corticosteroids for a duration that ranged from 6 weeks to 22 years, and immunosuppressive drugs from 25 days to 3.5 years. The Kaposi's lesions usually involved the skin on the extremities; internal organ involvement occurred in 7 cases. Most lesions responded to a decreasing dosage of corticosteroids and immunosuppressive drugs, or to the administration of radiation or cytotoxic therapy. Six patients died, 4 of which were related to the progression of Kaposi's sarcoma. CONCLUSION: Kaposi's sarcoma in patients with rheumatologic conditions is rare. The clinical features are similar to those with classical Kaposi's sarcoma. Tumor regression usually occurs with decreasing corticosteroids and/or immunosuppressive drugs, local irradiation, or cytotoxic therapy.
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ranking = 1
keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus
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5/17. calcinosis in rheumatic diseases.

    BACKGROUND: calcinosis, or dystrophic soft-tissue calcification, occurs in damaged or devitalized tissues in the presence of normal calcium/phosphorus metabolism. It is often noted in the subcutaneous tissues of connective tissues diseases--primarily systemic lupus erythematosus, scleroderma, or dermatomyositis--and may involve a relatively localized area or be widespread. The calcinotic accumulations may lead secondarily to muscle atrophy, joint contractures, and skin ulceration complicated by recurrent episodes of local inflammation and infection. OBJECTIVES: To review the classification, pathogenesis, clinical features, and treatment of calcinosis in rheumatic diseases. METHOD: A medline search of articles from 1972 to 2004 was conducted utilizing the index word "calcinosis" with the coindexing terms "scleroderma," "lupus," "dermatomyositis," and "dystrophic calcification." RESULTS: calcinosis may be the source of both pain and disability in connective tissue disease patients. Illustrative cases of patients with severe calcinosis are described. The literature available was critically reviewed. While warfarin, colchicine, probenecid, bisphosphonates, diltiazem, minocycline, aluminum hydroxide, salicylate, surgical extirpation, and carbon dioxide laser therapies have been used, no treatment has convincingly prevented or reduced calcinosis. CONCLUSIONS: calcinosis is common in the conditions reviewed and a number of agents have been used for treatment. However, the approach to calcinosis management is disorganized, beginning with the lack of a generally accepted classification and continuing with a lack of systematic study and clinical therapeutic trials.
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ranking = 1.037736014014
keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus
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6/17. Desensitization to hydroxychloroquine--experience of 4 patients.

    hydroxychloroquine (HCQ) is an antimalarial agent with immunomodulatory effects. It is widely used in rheumatologic diseases, and has a very high efficacy/toxicity ratio. It is particularly important in the treatment of systemic lupus erythematosus (SLE) since it reduces new organ involvement and disease flares, and relieves skin and joint symptoms. Some patients develop hypersensitivity rash in response to HCQ. In such patients the drug is withdrawn and replaced by another medication. All the alternative medications for rheumatological patients are significantly more toxic than HCQ. We describe our initial experience of HCQ slow oral desensitization. All 4 patients who were recruited completed the procedure successfully without significant difficulty. Our results suggest that HCQ slow oral desensitization is safe, effective, and easy to perform.
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ranking = 1
keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus
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7/17. Nodular regenerative hyperplasia of the liver in rheumatic diseases: report of seven cases and review of the literature.

    Nodular regenerative hyperplasia (NRH) of the liver is an uncommon pathologic finding associated, in most cases, with rheumatic and hematologic diseases. Although its pathogenesis remains unclear, NRH probably results from liver regeneration to maintain its functional capacity after ischemia-induced injury. An intrahepatic microvascular occlusive mechanism has been considered most likely pathogenetically. NRH may lead to portal hypertension. Thus, the diagnosis of Felty's syndrome must be considered with caution in patients with rheumatoid arthritis (RA) and NRH of the liver. We report seven additional cases of NRH in patients with rheumatic disorders and review the literature to determine the patterns of clinical presentation and natural history of this condition. We also report four patients (three systemic lupus erythematosus [SLE] and one primary antiphospholipid syndrome [PAPS]) in whom antiphospholipid antibodies may have played a role in the genesis of NRH.
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ranking = 1
keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus
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8/17. Malignant lymphoma in systemic rheumatic diseases. A report of five cases.

    We describe five patients suffering from systemic rheumatic diseases who developed a malignant lymphoma. Two patients, one with systemic lupus erythematosus and another with systemic necrotizing vasculitis, suffered from Hodgkin's disease and three other patients, one with lupus and two with sjogren's syndrome, developed non-Hodgkin's lymphoma. Only one of these patients had been treated with immunosuppressive drugs before the onset of the lymphoproliferative malignancy. Four patients were given combination chemotherapy and complete remission was achieved in the three patients for whom follow-up data were available. We discuss the relationships between systemic rheumatic diseases and lymphoma.
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ranking = 1.037736014014
keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus
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9/17. Improving compliance with prednisone therapy in pediatric patients with rheumatic disease.

    compliance with regimens for pediatric rheumatic diseases is often poor, and few studies have evaluated strategies for improving compliance. This study utilized relatively simple behavioral and educational strategies to improve compliance with prednisone for three patients with pediatric rheumatic diseases (systemic lupus erthematosus and dermatomyositis). These strategies were implemented in a pediatric rheumatology setting and resulted in improved compliance that was maintained at 6- and 12-month follow-up. During baseline, patients were found to be overmedicating as well as undermedicating. This study is a systematic replication of an earlier study that demonstrated that behavioral and educational strategies can improve compliance with medications for juvenile rheumatoid arthritis. It also raises the possibility of overmedicating as a compliance problem to be managed.
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ranking = 0.087409682493242
keywords = systemic lupus, lupus
(Clic here for more details about this article)

10/17. factor viii related antigen and childhood rheumatic diseases.

    factor viii related antigen (FVIIIRAg) levels were measured in the plasma of 63 children with rheumatic diseases and 20 controls. High levels were found in patients with systemic juvenile arthritis, systemic lupus erythematosus, dermatomyositis and systemic forms of vasculitis. The amount of circulating FVIIIRAg seemed to be independent of values for erythrocyte sedimentation rate, c-reactive protein and fibrinogen, implying that it was not just another acute phase reactant. Rather, a high level of circulating FVIIIRAg most likely reflects the presence of vascular endothelial injury, and this test may be useful in monitoring disease activity in children with rheumatic diseases in which vasculitis is present.
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ranking = 1
keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus
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