الفهرس 
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Abstract
Systemic lupus erythematosus (SLE) is a chronic, heterogeneous autoimmune inflammatory disease of unknown cause that can affect virtually every organ. Immunologic abnormalities, particularly the production of autoantibodies are prominent feature of the disease. It is characterized by relapsing and remitting course. Women are affected more frequently than men.
It is nine times more common in females, although the specific cause of SLE is unknown, multiple factors are associated with the development of the disease, including genetic, epigenetic, ethnic, immunoregulatory, hormonal, and environmental factors. Many immune disturbances, both innate and acquired, occur in SLE.
Lupus nephritis carries bad prognosis on both morbidity and mortality. An abnormal urinalysis with or without an elevated plasma creatinine concentration is present in a large proportion of patients at the time of diagnosis of lupus nephritis, and may eventually develop in up to 75 % of patients with a diagnosis of SLE. The most frequently observed abnormality in patients with lupus nephritis is proteinuria.
The complement cascade is a part of the innate immune system. Genetic deficiency of the initiator of the classical pathway, C1q, predisposes strongly to SLE. It was initially suggested that SLE development in C1q-deficient patients was due to a reduced ability to clear apoptotic cells since C1q is an important opsonin of such cells. However, new exciting roles for C1q and other complement proteins in SLE have since emerged. Genetic deficiencies of many classical pathway components are strongly associated with development of SLE.
The ability to quantify and grade disease activity whether in clinical practice or in research settings, is very important. Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) is based on the presence of 24 descriptors in 9 organ systems. It has been used in both research and clinical settings and as a predictive variable and outcome measure in prognostic studies of lupus.
SLEDAI-2K was modified to allow the documentation of ongoing disease activity in the descriptors: skin rash, alopecia, mucosal ulcers and proteinuria. Thus SLEDAI-2K includes the presence of any inflammatory rash, alopecia, or mucosal ulcers and new, recurrent, or persistent proteinuria >0.5 g/24 hours.
Hemodiafiltration with on-line endogenous reinfusion (HFR) is a dialytic method which combines the processes of diffusion, convection and adsorption. The performance of this system is linked to the optimal combination of the membrane permeability and cartridge resin bed. It is highly biocompatible, able to adsorb proinflammatory cytokines, and could improve LN prognosis may be through the counterbalance of the immune-modulatory response.
The aim of this study is to evaluate the role of HFR in the improvement of signs and symptoms of SLE activity in addition to laboratory parameters in patients not responding to traditional immune suppressive therapy. This study is a controlled clinical study which was conducted in Alexandria University Hospital patients after the approval of the ethical committee. It included two groups, group A, Sixty SLE patients in activity. Subdivided into, 47 patients (cases1) receiving the conventional immune suppressive therapy (Corticosteroids, Mycophenolate mofetil, Cyclophosphamide). 13 patients (cases 2) underwent hemodiafiltration with endogenous reinfusion (HFR) in addition to medical treatment. group B, subdivided into controls 1, 20 age and sex matched healthy volunteers. They have no history of any disease. And controls 2, 10 cases with different glomerular diseases other than SLE, all patients were subjected to through history taking and clinical examination plus routine laboratory investigations in addition to C3, C4, Anti-ds DNA, C1q done using ELISA before and after treatment and HFR sessions. Disease activity as well as quality of life were assessed using both SLEDAI-2K and SLEQOL.
HFR (for non- responder cases) is a two-chamber single use filter. That utilizes separated convection, diffusion and adsorption.
The results of our study showed that:
- C1q can significantly discriminate between SLE patients and both healthy controls and other glomerular non-lupus patients, p<0.001. so both the specific and sensitive to lupus patients.
- C1q was more deficient in proliferative lupus in comparison to other classes.
- C1q increased significantly after than before treatment in cases 1, p 0.048 while in cases 2 it increased significantly after the second HFR session with p 0.028, So C1q may help as a marker of evaluation of activity and response to treatment.
- Both SLEQOL and SLEDAI-2K improved significantly in both groups of cases after treatment.
- HFR might ameliorate inflammation and thus assist in the restoration of C1q levels.
- In both cases 1 and cases 2, C1q values were lower when ACR exceeds 500 mg/gm denoting possible correlation between C1q and Lupus nephritis.