1/208. Successful treatment of rapidly progressive lupus nephritis associated with anti-MPO antibodies by intravenous immunoglobulins. We report a case of systemic lupus erythematosus (SLE) associated with crescentic glomerulonephritis and myeloperoxidase-specific anti-neutrophil cytoplasmic antibodies (MPO-ANCA). A 34-year-old Japanese female patient diagnosed with SLE developed rapidly progressive renal failure and nephrotic syndrome. Haemodialysis was required to restore renal function. methylprednisolone pulse therapy followed by plasmapheresis did not suppress the progression of renal failure, so she was treated with high-dose intravenous immunoglobulin (IV-IG) therapy, which was well tolerated and effectively prevented renal failure. A renal biopsy showed diffuse proliferative lupus nephritis (WHO classification IVc) with predominant crescent formation and scant subendothelial immune deposits. These findings indicate that, in addition to lupus nephritis, which usually results from the deposition of circulating or locally formed immune complexes, MPO-ANCA may be involved in the pathogenesis of crescentic glomerulonephritis. Furthermore, we propose that IV-IG is an effective therapy for MPO-ANCA-related renal crisis in lupus nephritis. ( info) |
| A 17-year-old girl with systemic lupus erythematosus presented with painful edematous abdominal striae. She had been treated with systemic steroid for the systemic lupus erythematosus. At the time of presentation, she had abruptly gained 10 kg due to combined lupus nephritis. The histopathologic finding of the edematous striae distensae included dermal edema with separation of collagen fibers and small fragmented elastic fibers. Edematous striae distensae are uncommon but can develop from the combined effects of glucocorticoid and generalized edema. ( info) |
3/208. 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. ( info) |
4/208. Cryosupernatant plasma exchange in the treatment of antiphospholipid antibody syndrome with lupus nephritis. We report a case of a 22-year-old female with antiphospholipid antibody syndrome (APS) associated with systemic lupus erythematosus in whom cryosupernatant plasma exchange was effective and improved both the refractory venous thrombosis in her legs and relapsing thrombocytopenia. A renal biopsy specimen showed not only features of active lupus nephritis but also renal arteriolar thrombosis which is considered to be a type of thrombotic microangiopathy (TMA). Because a pathological role of unusually large von willebrand factor (vWF) multimers has been reported in patients with TMA including thrombotic thrombocytopenic purpura, plasma exchange using replacement with cryosupernatant, which is free of unusually large vWF multimers, is likely to be an option of treatment modality for patients with refractory and chronic relapsing APS manifesting TMA. ( info) |
5/208. Sclerosing peritonitis and systemic lupus erythematosus: a report of two cases. OBJECTIVE: To heighten the awareness of a possible association of sclerosing peritonitis in patients with systemic lupus erythematosus (SLE). methods AND RESULTS: Over the course of 17 years (from January 1981 to December 1997), 371 patients were treated with continuous ambulatory peritoneal dialysis (CAPD) at Rush-Presbyterian-St Lukes Medical Center. The patients were followed on CAPD for an average of 25 /- 21 (SD) months with a median of 19 months (range 0.2-115 months). During this time only 2 (0.5%) patients were diagnosed with sclerosing peritonitis, and both had SLE with ongoing evidence of active disease while on CAPD. With a total of 26 SLE patients being treated with CAPD during the observation period, the prevalence of sclerosing peritonitis can be said to be as high as 8% in this patient population. CONCLUSION: These cases suggest that autoimmune diseases, such as SLE, that are well known to cause immune-mediated serositis may represent an additional factor predisposing to the development of sclerosing peritonitis in patients treated with CAPD. ( info) |
| A 13-year-old girl developed lupus nephritis and Hashimoto thyroiditis in the chronic phase of juvenile myelomonocytic leukemia (JMML). At age 7 months, she was diagnosed as having JMML based on the hepatosplenomegaly, leukocytosis, thrombocytopenia, increased levels of fetal hemoglobin, and spontaneous in vitro growth of granulocyte-macrophage progenitors. At the onset of JMML, she had hypergammaglobulinemia, antinuclear antibodies, rheumatoid factors and anti-smooth muscle antibody. She had been placed on oral 6-mercaptopurine for about 12 years, with clinical improvement. At age 13 years, she was found to have hematuria and proteinuria. She also developed arthritis and Raynaud's phenomenon as well. She had antinuclear antibodies, rheumatoid factors, LE phenomenon, beta-1C (C3) nephritic factor (C3NeF), antithyroid antibodies, and hypocomplementemia. The renal biopsy specimens revealed a diffuse increase in the mesangial cells and matrix by light microscopy, and intense staining of IgG, Clq and C3 by immunofluorescence microscopy. The hormonal study ultimately showed decreased thyroid functions. So she was diagnosed as lupus nephritis and Hashimoto thyroiditis. The patient is the first example to show close relationship between stem cell abnormalities in JMML and development of overt autoimmune disorders. ( info) |
7/208. lupus nephritis in an anti-nuclear antibody-negative young male. The simultaneous presence of class III and class V renal lesions. We report about a 27-year-old white male, a known case of class III lupus nephritis with a very high anti-nuclear antibody (ANA) titer, who after 10 years of complete clinical and serological remission presented with sudden development of malar rash, proteinuria and an increase in the serum creatinine. Repeated serologic studies were all negative for ANA. A repeat kidney biopsy disclosed the presence of focal segmental glomerulosclerosis lupus nephritis (class IIIc) superimposed with a new membranous lupus nephritis (class V). ( info) |
8/208. 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. ( info) |
9/208. Improvement in lupus nephritis following treatment with a Chinese herbal preparation. OBJECTIVE: To study the effect of a Chinese herbal decoction (CM), which contained 21 different herbs, on clinical remission in a patient with lupus nephritis and chronic nephrotic syndrome. DESIGN: Case report describing the clinical and laboratory markers of lupus activity in the patient before and after treatment with CM. We also studied the in vitro effect of CM and its hydrophobic extract on spontaneous IgG production by peripheral blood mononuclear cells (PBMCs) from 12 patients with systemic lupus erythematosus (SLE) compared with 9 healthy control subjects. RESULTS: Spontaneous PBMC IgG production was significantly higher in patients with SLE (mean /- SD, 20.4 /-10.6 x 10(-5) g/L) compared with controls (4.7 /-1.9 x 10(-5) g/L) (P<.001). Addition of CM and its hydrophobic extract to PBMCs from patients with SLE resulted in significant suppression of spontaneous IgG production. CONCLUSIONS: The CM may contain some active pharmacological compound with immunosuppressive properties useful in the treatment of SLE. Further controlled studies are important to evaluate the efficacy of this medicine, potential toxic effects, and the possible immunosuppressive mechanisms of the active component(s). ( info) |
10/208. Crossroads of the effects of cyclophosphamide pulse therapy for lupus nephritis--experience of 11 cases. In this study time for initial assessment of monthly intravenous cyclophosphamide (CP) pulse therapy is discussed for a better outcome with less complications. Eleven patients with lupus nephritis (LN) resistant to conventional therapy (serum creatinine level < or = 2.7 mg/dl) were given 500 mg/m2 of CP 7-9 times with an interval of one month. Urinary protein (Up) decreased in all patients after 3 courses of CP pulse therapy and kept similar levels thereafter. In one group of patients (n = 7), Up decreased to < 2 g/day after 3 courses, while in the other group (n = 4), it did not decrease to < 4 g/day. creatinine clearance increased by 0-100% in the former group, while it decreased by 5-20% in the latter group after 6-9 courses. Renal function of the patients with insufficient response after 3 courses tended to show no further improvement or worsened thereafter, although Up decreased during CP pulse therapy. A relatively small dose of CP (500 mg/m2) pulse therapy was useful in most LN patients regardless of the renal histology and it was thought important to assess its effects after 3 courses for a prediction of the clinical course. Modification of the protocol at that time might be necessary in regard to dose or interval of CP administration especially for patients with insufficient outcome. ( info) |