Cases reported "Lupus Nephritis"

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1/12. Systemic amyloidosis and sacroiliitis in a patient with systemic lupus erythematosus.

    We report a case of a 25-year-old female with juvenile onset systemic lupus erythematosus who developed systemic secondary amyloidosis with renal and gastrointestinal involvement. She has also had radiological signs of bilateral asymptomatic sacroiliitis without lower back pain or hla-b27 antigen.
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2/12. Immunoadsorption as a tool for the immunomodulation of the humoral and cellular immune system in autoimmune disease.

    Immunoadsorption onto staphylococcal protein a is a newly developed semiselective extracorporeal adsorption technique for immunoglobulins applied in patients suffering from severe autoimmune disease. Its effect on the humoral and cellular immune system was investigated using standard immunological assays. The elimination capacity for total IgG and IgG subclasses 1, 2, and 4 was more than 90% but for subclass IgG3 varied between 30 and 90%. autoantibodies, e.g., anti-dsDNA, anti-glomerular basement membrane (anti-GBM), anti-cardiolipin, and anti-human leukocyte antigen (anti-HLA) antibodies, were eliminated in comparable amounts. The affinity of protein A for circulating immune complexes (CIC) was 300 times greater than for soluble IgG. HLA-II expression on monocytes and T lymphocytes was reduced over time during repeated IAs (IA). The number of activated T lymphocytes declined while the percentage of naive T cells increased. A diminished CD4/CD8 ratio normalized during IA treatment. These results indicate that IA actively modulates the humoral as well as the cellular immune system in addition to its immunoglobulin reducing effect.
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3/12. Hypocomplementemic urticarial vasculitis or systemic lupus erythematosus?

    The 2 patients presented here showed the typical signs of hypocomplementemic urticarial vasculitis syndrome (HUVS). During follow-up, there was an inverse correlation between anti-C1q autoantibody titer and C1q antigen concentration in serum in both patients over a period of 2 years. The first patient had nephritis characterized by immune deposits in glomeruli and around the tubules. The histological findings, C1q deposits, and presence of tubuloreticular inclusions in capillary endothelial cells suggested a disease process identical to systemic lupus erythematosus (SLE). The second patient, after a lag phase of 2 years, fulfilled a fourth American College of rheumatology criteria for SLE when she developed anti-double-stranded dna antibodies. HUVS and SLE overlap, and the criteria for identifying HUVS as an entity distinct from SLE are lacking.
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4/12. Systemic lupus erythematosus complicated by cytomegalovirus-induced hemophagocytic syndrome and colitis.

    Here, we report a case of systemic lupus erythematosus (SLE) complicated by cytomegalovirus (CMV)-induced hemophagocytic syndrome (HPS) and colitis. A 44-year-old woman with SLE was treated with corticosteroid and cyclophosphamide for lupus nephritis. Although her lupus nephritis improved, fever, progressive pancytopenia and intestinal bleeding were observed. A bone marrow aspiration showed an increase in mature histiocytes with hemophagocytosis. In addition, a colonoscopy showed hemorrhagic colitis with ulcer and the biopsy specimen from the colon revealed typical CMV cells with CMV inclusions confirmed by immunohistochemistry. Furthermore, a large number of CMV antigen-positive leukocytes was detected, suggesting an active CMV infection. CMV infection is serious in compromised hosts. Therefore clinicians should be aware of the clinical settings in which this infection can arise and the target organs potentially affected in order to initiate the appropriate intervention.
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5/12. cytomegalovirus colitis following immunosuppressive therapy for lupus peritonitis and lupus nephritis.

    We report a woman with lupus nephritis complicated with lupus peritonitis and cytomegalovirus (CMV) colitis. diagnosis of lupus peritonitis was made by abdominal computed tomography scan, colonoscopy, and ascitic fluid analysis. Steroid and cyclophosphamide therapy resulted in the improvement of severe lupus nephritis and peritonitis. Thereafter, she developed multiple colonic ulcers as diagnosed by colonoscopy and positive CMV antigenemia assay. Treatment with ganciclovir resulted in the disappearance of colonic lesions. The low cluster of differentiation (CD)4 lymphocyte count (41/mm3) suggested that the cell-mediated immunity of this patient was comparable to that seen in patients with acquired immunodeficiency syndrome (AIDS).
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6/12. ANA negative (Ro) lupus erythematosus with multiple major organ involvement: a case report.

    Anti-nuclear antibody (ANA) negative systemic lupus erythematosus (SLE) occurs in about 4-13% of SLE cases. A small group of ANA negative SLE patients with positive anti-Ro antibodies usually present with typical vasculitic skin lesions which can be associated with photosensitivity, renal disease, congenital heart block or neonatal lupus. We present a case of a persistently ANA negative patient who presented with joint pain, rashes, mouth ulcer and alopecia. Clinical diagnosis of systemic lupus erythematosus was made even though ANA was negative. She was started on steroids and went into remission. Later, she developed several episodes of convulsions associated with fever and prominent vasculitic lesions. The patient was also found to have microscopic hematuria, proteinuria, anemia and thrombocytopenia. Renal biopsy showed lupus nephritis class 1B. Due to the prominent skin lesions, we performed anti-extractable nuclear antigens (ENA) antibodies test and anti-Ro turned out to be positive. The final diagnosis was ANA negative SLE (Ro lupus) with cutaneous, renal, musculoskeletal, hematological and cerebral Involvement.
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7/12. EBER-1 positive diffuse large cell lymphoma presenting as lupus nephritis.

    Approximately one-third of membranous glomerulonephritis (MGN) cases in adults are associated with systemic diseases, including systemic lupus erythematosus (SLE) or malignancies. Malignancy-associated glomerulonephritis is rarely found in non-Hodgkin's lymphoma (NHL). Epstein-Barr virus (EBV) has been postulated to contribute to the pathogenesis of both SLE and NHL. We described a 37-year-old woman with nephrotic syndrome who presented with clinical features of SLE and renal-biopsy revealed lupus MGN. The patient also suffered from concomitant progressive lymphadenopathy and NHL (diffuse large B-cell type) was demonstrated by neck lymph node biopsy. Serologic studies demonstrated EBV infection and specific EBV antigens were present on lymph node and metastatic sites. We offer a discussion regarding the complex relationships between SLE, NHL, MGN and EBV.
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8/12. A case of cytophagic histiocytic panniculitis with sicca symptoms and lupus nephritis.

    A 48-year-old Japanese woman presented with many subcutaneous nodules. The skin was purplish in color and tender; the nodules were scattered over the entire surface. Histological findings of biopsy specimens from the nodules indicated septal panniculitis comprised of histiocyte and/or macrophage infiltrates, often with erythro- and/or leukophagocytosis. Phagocytic cells were OKM1 (CD11b), MT1 (CD43), LeuM3 (CD14), and histiocyte antigen positive, indicating the presence of histiocytes and/or macrophages. The patient had sicca symptoms, positive homogenous, speckled pattern ANA (x320), and diffuse proliferative lupus nephritis.
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9/12. Isaacs' syndrome (autoimmune neuromyotonia) in a patient with systemic lupus erythematosus.

    patients with systemic lupus erythematosus (SLE) often produce autoantibodies against a large number of antigens. A case of SLE is presented in which muscle twitching and muscle cramps were associated with an autoantibody directed against the voltage-gated potassium channel of peripheral nerves (Isaacs' syndrome).
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10/12. The immunochemical characterisation of circulating immune complex constituents in candida albicans osteomyelitis by isoelectric focusing, immunoblot, and immunoprint.

    Circulating immune complexes present in the serum of a patient with systemic lupus erythematosus, end-stage renal failure, and thoracic vertebral candida albicans osteomyelitis were sequentially analysed by isoelectric focusing, immunoblotting, and immunoprinting. Candida antigens (including mannoproteins), Clq and C3 complement components, and specific anti-Candida antibody were detected within polyethylene glycol precipitated complexes. An antigen of 47K molecular weight was amongst those demonstrated by polyacrylamide gel electrophoresis to be present within the complexes. It has been proposed elsewhere that a serologic response to a 47K protein is predictive of recovery from candida albicans infection. Free antibody had specificity for Candida antigens ranging in molecular weight from 18K to more than 100K including a 47K component. The analytical techniques employed allowed rapid and precise identification of the components of one particular immune complex system and will be widely applicable to the dissection of other diverse systems.
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