Hungarian Immunology

[Long-term follow-up of a large Hungarian lupus patient population]

KISS Emese, SONKOLY Ildikó, SZEGEDI Gyula

MARCH 20, 2002

Hungarian Immunology - 2002;1(01)

[INTRODUCTION - The authors give a report about their experiences obtained by long-term follow-up of a large Hungarian lupus patient population. They also compare their results with literature data. PATIENTS AND METHODS - Clinical and laboratory characteristics of 800 SLE patients followed at this institution were analysed. Issues of diagnosis, therapy and outcome measures were also discussed. RESULTS AND DISCUSSION - Results indicate that the incidence and prevalence of SLE are increasing. Recently, milder cases are also recognised. Early diagnosis makes possible an early adequate therapy. Partly due to these the survival improved, and the mortality reduced. The compliance of patients also improved. The authors suggest the importance of regular medical follow-up. Despite of that, the clinical presentations of SLE have not been changed the disease became milder. Considering the longer survival in lupus, chronic complications, such as vascular diseases, osteoporosis and cancer are suspected to appear more frequently. On the other hand, there is a small group of patients still being in risk, as they fail to respond conventional immunosuppressive therapy. These patients die within a short period due to progression of SLE. This indicates the importance of research work on the pathogenesis of SLE. Based on the results of basic research novell immune modulator modalities should be developed.]

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Hungarian Immunology

[Relevances of anti-CD44 immunotherapy for cellular biology]

GÁL István, GLANT T. Tibor, MIKECZ Katalin

[INTRODUCTION - The CD44 molecule - the physiologic hialuronic acid receptor - is one of key mediators that direct the traffic of leukocytes into inflamed tissues. When applied in animal models of autoimmune arthritis, parenteral anti-CD44 antibody treatment exerts a dramatic antiinflammatory effect, but at high doses also a leukopenic effect. The goal of the present work is to elucidate the cellular basis of these phenomena. METHODS - In this study the authors used Western blot, immunoprecipitation, cell adhesion studies, flow chamber system studies (leukocyte rolling) and fluorescence microscopy following fluorescent labeling of actin cytoskeleton. RESULTS - Adhesion of CD44-expressing leukocytes to immobilized hialuronic acid does not result marked changes in cellular morphology. When incubated on immobilized anti-CD44 antibody, however, these cells spread, reorganize the actin cytoskeleton, and they adhere strongly to the surface. Studying the mechanisms of signal transduction, the authors found that engagement of CD44 with anti- CD44 antibody results in its enhanced association with numerous cytoskeletal regulator proteins, including ezrin, ankyrin, spectrin and focal adhesion kinase, thereby increasing the interaction between the cytoskeleton and the plasma membrane. Strong adhesion of the cells to immobilized anti-CD44 also prevents the rolling movement of these cells, mediated by CD44-hialuronic acid interactions, which precedes the extravasation of leukocytes to sites of inflammation. CONCLUSION - These results may provide insight into the antiinflammatory mechanisms of anti- CD44 antibody treatment. Based on these results and results published by other investigators, anti- CD44 antibodies may be uselful in the immunotherapy of rheumatic diseases.]

Hungarian Immunology

[PAPER REVIEW]

SZABÓ Zoltán

Hungarian Immunology

[In memoriam professor Gyula Petrányi]

SZEGEDI Gyula

Hungarian Immunology

[Anti-inflammatory effects of high-dose intravenous immunoglobulin therapy in immunothrombocytopenic purpura]

ERDŐS Melinda, MARÓDI László

Hungarian Immunology

[Patomechanism of hereditary angioneurotic oedema and provoking factors of oedematous attacks]

FARKAS Henriette

[The author describes the genetic background of hereditary angioneurotic edema, an autosomal dominant disorder. The pathomechanism of edemaformation and the significance of major mediator substances are explained along with clinical manifestations and their management. A special emphasis is placed on prophylaxis, the mainstay of which is the elimination of precipitating factors. The latter include mechanical trauma, diagnostic and therapeutic interventions performed in the cephalic-cervical region, mental stress, and sex hormones. The effect of endocrine therapies, ACE inhibitors, and infections - Helicobacter pylori in particular - on the natural course of the disease is also discussed.]

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[Recent development of surgery resulted in fundamental changes in assessment of resectability of liver tumors. Surgical interventions became more radical and more effective. Colorectal liver metastasis (CLM) represents the most frequent hepatic tumor, where therapeutic options require close collaboration between surgeons and oncologists, and up-to-date approach from both. As the fact is, that CLM is a metastasis of a primary colorectal carcinoma, it seems to be obvious to apply perioperative chemotherapy. Results justify serious precaution. Neoadjuvant chemotherapy did not improve overall survival. Several data testify, that even perioperative chemotherapy is not indicated in these cases. Adjuvant chemotherapy can be applied after extended liver resections and two stages hepatectomies. About 20% of patients with initially inoperable CLM may be rendered resecable after systemic chemotherapy. Prognosis of synchron CLM is bad, 5 year survival is less than 20%. Disappearing CLM needs special respect, high level of perfection in liver surgery is essential. After chemotherapy postoperative morbidity is rising, technical diffi culties may occur. Further studies are required to examine possible effect of new targeted molecular therapy-based regimens on resectability. Individualized multidisciplinary treatment planning is mandatory.]

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[CRITICAL SITUATIONS IN SYSTEMIC AUTOIMMUNE DISEASES]

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[Systemic autoimmune diseases characteristically show multiple organ involvements. Hence, Clinical manifestations and the outcome are quite variable. The survival has continuously been improving due to more sophisticated diagnostic tools and therapeutic possibilities. Despite the technical development, complications and organ manifestations may lead to emergency or often provoke permanent functional or morphologic deterioration of the affected organs. Critical situations can be directly attributed to the manifestations of the underlying disease, deterioration of the affected organs, side effects of immune suppressive therapy complications of Intercurrent or co-incidental diseases also may induce the occurrence of critical complications. The Authors describe all conditions that may lead to the development of critical situations at systemic autoimmune disease regarding different organ and organ systems. The symptoms and diagnostic possibilities are also analysed. A checklist is given about those systemic autoimmune disorders where particular critical events can be present. Medication in general and other possible immune modulator managements with beneficial effects are discussed.]

Hypertension and nephrology

[Hungarian Vasculitis Registry – results of the first five years]

HARIS Ágnes, TISLÉR András, ONDRIK Zoltán, FILE Ibolya, MÁTYUS János, ZSARGÓ Eszter, DEÁK György, AMBRUS Csaba

[Launching the Hungarian Vasculitis Registry aimed to collect information about prevalence and outcome of our patients with ANCA-associated vasculitis, and treatment protocols of the disease. The on-line data collection has been developing dynamically since its initiation five years ago, presently 278 patients’ files are available. Patients’ mean age is 58.2±14.5 years, 62% are women; their disease is associated with c-ANCA positivity in 51% and p-ANCA in 49%. At diagnosis GFR was 24.6±21.6 ml/min/1,73 m2, that time 29%, during the total follow up 39% of the registered subjects needed dialysis. Renal replacement therapy could be discontinued in 23% of them. In cases with focal histological changes, also with upper respiratory tract and skin involvement dialysis was significantly less frequently necessary, which underlines the importance of early diagnosis. In induction therapy steroid was administered for 94% of the patients, 85% of them got cyclophosphamide, 59% was treated by plasmapheresis, 11% got rituximab. Maintenance treat ment contained steroid in 80%, per os cyclophosphamide in 23%, parenteral cyclophosphamide in 22%, furthermore 40% of the patients got azathioprin, 8 subjects got mycophenolate and 6 got methotrexate. Median follow up was 30 months (IQR 6-78), during which period 20% of the patients died, 5% got kidney transplantation, and 5% were lost to follow up. Median survival was 14.8 years, five years survival was 85%, and ten years survival was 70%. Long term survival in patients with c-ANCA vasculitis seemed better comparing to p-ANCA vasculitis, but when correcting by age this difference disappeared. Predictors of death were age and dialysis dependent renal failure. Relapses developed in 27% of patients, 28% of them presented in the first year, 21% suffered it after five years of care. Collected data by the Hungarian Vasculitis Registry shows our society’s successful professional activity. Our results are comparable to the published data in the literature, yet there are several areas in our care where further improvements are warranted in order to increase our patient’s survival and quality of life.]

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[Treatment of hepatocellular carcinoma by liver transplantation: results in Hungary]

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[INTRODUCTION - Hepatocellular carcinoma is an internationally accepted indication for liver transplantation in selected cases. The aim of this study was to present treatment results of patients diagnosed with hepatocellular carcinoma and put on the waiting list between 1995 and 2010. PATIENTS AND METHODS - Sixty patients were put on the waiting list, 23 of who died or became unfit for operation while waiting for transplantation. Liver transplantation was performed in 37 patients. Pathological examination revealed no tumour in 3 of the explanted livers, whereas incidentalomas were detected in 4 livers that were explanted because of cirrhosis. Thus, in total 38 patients were examined. RESULTS - Four patients died within 3 months of surgery because of arterial circulatory problems or graft dysfunction and 14 patients died thereafter, including seven whose tumour recurred. There was no recurrence of tumours in the early state among the 25 patients meeting the Milan criteria, and the overall 1-, 3-, and 5-year survival rates with an average follow-up period of 39 months were similar to those after transplantation because of viral cirrhosis: 72%, 72% and 67% vs. 78%, 71% and 67%, respectively. Survival rates after tumours beyond the Milan criteria were significantly lower (69%, 38%, 23%). CONCLUSION - Early-state (T1,T2) hepatocellular carcinoma that has developed following cirrhosis and is unsuitable for resection can be efficiently treated with liver transplantation, but our results do not support the use of transplantation for the treatment of tumours that do not fit the Milan criteria.]

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[OUR EXPERIENCE WITH BORTEZOMIB-BASED THERAPY OF MULTIPLE MYELOMA BASED ON THE FIRST 60 PATIENTS]

MIKALA Gábor, BÁTAI Árpád, CEGLÉDI Andrea, CSUKLY Zoltán, DOLGOS János, HALM Gabriella, JÁNOSI Judit, KAPÁS Balázs, LOVAS Nóra, LUEFF Sándor, PETŐ Mónika, REMÉNYI Péter, SIPOS Andrea, TÓTH Zsuzsanna

[INTRODUCTION - Bortezomib, a first-in-itsclass proteasome-inhibitor drug was registered in 2004 for the salvage treatment of relapsed and/or refractory multiple myeloma. We have been using this drug in our department for the treatment of myeloma patients since 2005. PATIENTS AND METHODS - In this retrospective study, treatment results (response rate, response duration, survival) as well as the complications and side effects were analysed based on 60 myeloma patients treated over a period of 18 months. The patients received at least one full cycle of non-first-line bortezomib-based (predominantly combinational) therapy. RESULTS - At least minimal laboratory and/or clinical response was observed in 47 of the 56 patients who could be analysed. Clinically meaningful (at least partial remission) response was seen in 41 of 56 patients. Immune-fixation negative complete remission was achieved in six patients. Median progression-free survival of our patient population was 13 months (10.8-14.8 months, n=49, adjusted for patients lost in the first 6 weeks and for those with less than 6 weeks of follow-up). As for overall survival, the median has not been reached, while treated patients had an 80.3% probability of survival at one year. CONCLUSIONS - Based on the treatment results of 60 myeloma patients, bortezomibbased therapy is clearly effective in relapsed and/or refractory myeloma.]