Hungarian Immunology

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


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.]



Further articles in this publication

Hungarian Immunology

[In memoriam professor Gyula Petrányi]


Hungarian Immunology

[Neonatal activation of interferon-γ in macrophages]

ERDŐS Melinda, MARÓDI László

[Each individual passes through developmental or transient immunodeficiency due to the immaturity of the immune system in early childhood, expecially in the neonatal period. Therefore, neonates contract infections by intracellular and extracellular microorganisms more easily than older children and adults, and develop more severe disease with a high mortality rate. A number of abnormalities in the neonate’s host defense systems have been described suggesting that the immune system at birth functionally differs from that in adults. Neonatal T and B cells show decreased reactivity to antigens and mitogens and have deficienct IgM-IgG isotype switching. Newborns have decreased functional capacities of the hemolytic complement system. Under the same in vitro and in vivo conditions neonatal granulocytes show functional deficiency earlier than adult cells. Effector mechanisms of the cell-mediated immunity involve activation of macrophages by T helper1 cytokines, particularly interferon- γ (IFN-γ). IFN-γ is the most important macrophage-activating cytokine in vivo. Neonatal T cells express lower levels of IFN-γ and macrophages are hyporesponsive to activation by this cytokine. This deficiency may be explained by decreased phosphorilation of STAT1 despite comparable expression of STAT1 protein in neonatal and adult macrophages.]

Hungarian Immunology

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

ERDŐS Melinda, MARÓDI László

Hungarian Immunology

[2nd C1-esterase inhibitor deficiency workshop, April 2001, Budapest]

FARKAS Henriette, VARGA Lilian, HARMAT György, FÜST György

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.]

All articles in the issue

Related contents

Clinical Neuroscience

Long-term follow-up results of concomitant chemoradiotherapy followed by adjuvant temozolomide therapy for glioblastoma multiforme patients. The importance of MRI information in survival: Single-center experience

LUKÁCS Gábor, TÓTH Zoltán, SIPOS Dávid, CSIMA Melinda, HADJIEV Janaki, BAJZIK Gábor, CSELIK Zsolt, SEMJÉN Dávid, REPA Imre, KOVÁCS Árpád

Introduction - Glioblastoma multiforme (GBM) is the most common malignant primary anomaly of central nervous system. The GBM infiltrates the nearly sturctures from the initial tumor and its metastatic attribution is well known. The aim of our single-centered retrospective study was to introduce the importance of postoperative medical imaging confirmation of total tumor resection for patient with GBM combined concomitant and adjuvant chemoradiotherapy on a 10 year long patient follow up. Methods - From January 2006 to April 2015 we registered 59 patients with newly diagnosed GBM at the University of Kaposvár Health Center Institute of Diagnostic Imaging and Radiation Oncology. The histological diagnosis was confirmed by a proficient neuropathologist (World Health Organisation WHO; grade IV astrocytoma). According to histological status if the ECOG performance status of patients allowed it the mutidisciplinary oncoteam recommended adjuvant chemoradiotherapy all features strictly by Stupp protocol. (60 Gy dose on the gross tumor volume and 2-3 cm margin for the clinical target volume with parallel 75 mg/m2 TMZ. Four weeks after monotherapial phase patients had to recieve 6 cycles of TMZ first cycle with 150 mg/m2 up to 200 mg/m2). The irradiation was carried out by a conformal three dimensional planning system. Results - 59 patients with the median age of 63 (range 17-84) year. Our sample counted 34 male patients and 25 woman patients. 14 patients underwent gross total tumor resection while, 39 patients underwent partial resection and the rest from our sample 6 patients passed through biopsy. Statistical analysis showed a lengthier survival among males than females, with a median survival of 13 months for males and females, the OS of 26.209 for males, meanwhile 15.625 for females. However, the difference is not considerable (log-rank p=0.203). Our study found that the estimated survival of patients at least 50 years old is significantly shorter at a median survival of 12 months (log rank p=0.027) than that of patients below 50 years of age at a median survival of 23 months. The longest estimated median survival was calculated with patients of ECOG '0' condition (16 months). However, no significant difference was found in the estimated survival of patients of different ECOG conditions (log-rank p=0.146). Based on the extent of surgery, complete resection resulted in the longest average survival of 36.4 months, followed by 21.5 months among patients with biopsy, and 15.8 months among patients with partial resection. Different surgical procedures, however, did not result in significant differences in survival (log-rank p=0.059). The overal survival of patients who had complete resection confirmed by MRI compared with the overal survival of patients with residual tumor confirmed by MRI as well we can estimate that there is significant difference between these two groups (p=0,004). Conclusion - Despite complex and intense treatment, recurrence is inevitable and causes relatively rapid death. In our analysis complete resection, as defined from the neurosurgeon’s report and postoperative MRI, resulted in an independently significant improvement in OS. Our results are the evidences that the treatment of patients with glioblastoma multiforme in Hungary is at least on the same level as any other developed European countries.

Clinical Neuroscience


GULÁCSI László, MÁJER István, KÁRPÁTI Krisztián, BRODSZKY Valentin, BONCZ Imre, NAGY Attila, BERECZKI Dániel

[The aim of our research was to assess the incidence and the 12- and 24-month mortality of hospitalized stroke in Hungary. We analyzed the rate of mortality after stroke and compared it to the standard mortality rate of the population. To assess the incidence we extracted the data of “new” stroke patients (ICD- 10 diagnoses: I60-64) hospitalized in May 2003 from the database of the National Health Insurance Fund Administration. We regarded those as “new” patients who had not been treated with these primary or secondary diagnoses in the previous 24 months. Data were collected by sex and age (age groups: 25-44, 45-64, 65 and over). We analyzed the patients' survival on the basis of their April 2004 and April 2005 data. The incidence of the “new” hospitalized stroke patients was higher in men than in women; the incidence in the age group of 65 and over was 2112/100.000 in males and 1582/100.000 in females, the corresponding values in the 45-64 age group were 623 vs. 366 per 100.000, respectively. In 2003 more than 42 thousand “new” stroke patients were hospitalized in Hungary of whom over 10 thousand died in the first year, followed by a further 2 thousand in the second year. Women’s survival is more favourable than men's: in the first year it is 71.47% vs. 69.24% (65+ group), and 88.18% vs. 83.16% (45-64 group); in the second year the corresponding values are 66.95% vs. 61.62% (65+), and 85.45% vs. 80.90% (45-64), respectively. The risk of death in the first year after stroke, compared to the standard population, is 5.17- fold in women and 4.70-fold in men in the total sample, and 10-15-fold in the 45-64 group. There are large differences by gender, particularly in men of the working age groups (25-44, 45-64), whose mortality is twice as high as that of women of the same age.]

Clinical Oncology

[Clinical role of multigenic prognostic tests in breast cancer therapy]


[Current clinical practice for breast cancer originates in “evidence based medicine”. In this, each tumor receives a therapy optimal for a given patient population - which might not be optimal for each individual patient. Multigenic tests determining expression of a set of genes can provide additional support in this decision process. Two such tests (MammaPrint and Prosigna) have already received FDA clearance. A number of additional test are commercially available (IHC4, Oncotype DX, EndoPredict, BCI). A common property of these assays is their utility in estrogen receptor positive early breast cancer. The main clinical problem answered by them is the necessity of adjuvant chemotherapy. To date, no reliable algorithm has been identifi ed capable to pinpoint the most effective chemotherapy combination for a given patient. Furthermore, there is no trustworthy test for triple negative breast cancer. The assays utilize different technologies (immunohistochemistry, gene chips, RT-PCR) and a discrepant list of genes - these result in discordance of the predictions for the individual patient. Despite these shortcomings, multigenic tests quickly gained foothold in breast cancer therapy decision process. Their utility is supported by the cost reduction for the health care providers by lowering the number of patients eligible for chemotherapy.]

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.]

Hungarian Immunology

[Immune complex clearance in systemic lupus erythematosus]


[Impaired clearance of immune complexes is regarded as a central factor in the pathogenesis of systemic lupus erythematosus (SLE). Receptors for IgG (FcγRs) are expressed on phagocytes and madiate binding and endocytosis of IgG immune complexes. At first the binding of the ligand to the receptor of monocytes was determied with reaction kinetic method and microscopically. The results demonstrated that the binding of monomeric IgG is higher but that of immune complexes is lower to receptors of patient's monocytes. This discrepancy could be explained than the molecular heterogeneity of FcγRs on human phagocytes was revealed. The FcγRI binds the monomeric IgG, at the same time the FcγRII and III both bind and ingest the immune complex. After that the expressions of the different FcRs, as antigens, were investigated with monoclonal antibodies in flow cytometer. According to the authors' earlier results the expression of FcγRI on monocytes of patients was elevated but that of FcγRII and III were decreased parallely with the phagocytosis. The explanation for this discrepancy may be the structural and functional difference of the FcγR. The expressions of FcγRII and III decreased also on the granulocytes of patients. Impaired in vivo clearance of particle immune complex was measured in SLE patients correlated with the clinical activity of disease and the renal involvement. The data suggest that the alterations of FcγRI expression on phagocytes in SLE are much better a disease-related process and depend on acquired factors than on inherited one. In the transport of complement containing immune complex to macrophages the erythrocyte complement receptors (CR1) has important activity which are also decreased in SLE. The number of CR1 on erythrocytes was investigated by the binding of labelled ligand and monoclonal antibodies to the receptor in flow cytometer in paralell with the genetically determination of receptor expression. The data revealed a correlation between kidney involement of patient and CD1 deficiency, and their expression can be corrected with epoetin α treatment and with plasmapheresis. These data also suggest the role of acquired factors contributing to CR1 deficiency in SLE.]