Hungarian Immunology

[The outcome of the renal involvement in primary Sjögren’s syndrome]


APRIL 20, 2003

Hungarian Immunology - 2003;2(02)

[OBJECTIVE - Kidney function re-evaluation in primary Sjögren’s syndrome (P-SS) patients years after the first signs of renal involvement. PATIENTS - Of 75 primary SS patients followed up for various periods between 1990 and 1999, 11 had overt kidney involvement. The mean age of these 11 at the time of diagnosis of renal manifestations (first examination) was 39.6 years. In nine of the 11, the renal function was re-examined (second examination: NH4CL loading, determination of urinary concentrating ability, proteinuria and technetium99m-mercaptoacetyltriglycine clearance) on average 8.8 years later. RESULTS - At the first examination overt renal tubular acidosis (RTA) was diagnosed in 11 patients (proximal in one and distal in 10), accompanied by hyposthenuria in five, and proteinuria >0.5 g/24 h in four. Tubulointerstitial nephritis (TIN) was diagnosed in all four biopsied patients with proteinuria, and cryoglobulinaemic glomerulonephritis in one of them. Seven of the 11 were treated with moderate or low doses of glucocorticosteroids, and two with repeated methylprednisolone pulse therapy. The acidification capacity of the kidneys and degree of proteinuria mostly improved significantly (p<0.001), but the degree of hyposthenuria did not change essentially between the examinations. CONCLUSIONS - The outcome of the kidney manifesztation in primary Sjögren’s syndrome is usually favourable, but end-stage renal failure can develop rarely.]



Further articles in this publication

Hungarian Immunology

[Molecular biology of 70 kD heat shock protein and its role in certain immunological processes]


[Heat-shock proteins, or stress proteins play important role in cellular survival owning to their protective function. Their highly conserved structure renders them ideal messengers of cellular stress response. One of the best known representative of these proteins is the 70 kDa heat-shock protein (Hsp70), there is increasing amount of data about the intraand extracellular functions of this stress protein. In the present review the regulation of hsp70 gene expression, and hsp70 polimorfisms, the possible impact of polymorphisms to certain diseases, and the multilevel relationship between Hsp70 and the immun response are discussed. The authors review the role of Hsp70 in anti-tumor immunity, and the presence of anti-Hsp70 antibodies and their possible association with certain diseases. Here they present some of their recent observations: they detected the presence of anti-Hsp70 antibodies in all adult sera and found no correlation between these antibody levels and the presence of severe coronary heart disease. Recently we also showed, that human Hsp70 can activate the classical pathway of complement system in vitro, by direct binding of the first complement C1q.]

Hungarian Immunology

[Autoantibodies against α-fodrin in patients with Sjögrens’s syndrome]

SZÁNTÓ Antónia, CSÍPŐ István, ZEHER Margit

[INTRODUCTION, PATIENTS AND METHODS - In this study, the authors examined the presence of the IgA and IgG type autoantibodies against the 120 kDa α-fodrin in the sera of patients affected with primary and secondary Sjögren’s syndrome, rheumatoid arthritis and systemic lupus erythematosus, being treated in the Department of Clinical Immunology of the 3rd Department of Internal Medicine, at the University of Debrecen. As a control population, the sera of healthy blood donors were used. RESULTS - Due to their findings, the presence of autoantibodies against the α-fodrin was significantly higher in patients with primary Sjögren’s syndrome than in the control group. The presence of these autoantibodies occurred significantly more often in patients affected with secondary Sjögren’s syndrome associated to RA and SLE, than in these polysystemic autoimmune diseases without sicca-syndrome. Interestingly, they couldn’t find any connection between the presence of autoantibodies against α-fodrin and the occurrence of SS-A/Ro or SS-B/La autoantibodies. There was no correlation in primary and secondary Sjögren’s-syndrome between the extraglandular symptomes or the swelling of the salivary glands and the presence of the anti-α-fodrin autoantibodies. CONCLUSIONS - The autoantibodies against α- fodrin might be important in the diagnosis of the juvenile Sjögren’s syndrome and other juvenile autoimmune diseases, in the early diagnose of Sjögren’s syndrome, especially in the lack of anti-SSA/ Ro and anti-SS-B/La.]

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.]

Hungarian Immunology

[Immunophenotyping of mature cell non-Hodgkin’s lymphomas with leukemic clinical manifestation - newer approaches]

PÁLÓCZI Katalin, NÉMETH Julianna, BÁNYAI Anikó, GOPCSA László

[Immunophenotyping is commonly used in evaluating malignancies of the lympho-hemopoietic system and its use in various disease states of mature lymphoid leukemias and related non-Hodgkin’s lymphomas is reviewed here. The major goals of immunophenotyping in mature lymphoid neoplasias are the assignment of abnormal cells to the B or T/NK linkage, their maturational analysis, and the characterization of specific phenotypes which might be helpful for the subclassification of disease. There is not known, however, any lymphoma (leukemia) -specific antigen and the individual type of lymphoid leukemias and lymphomas does not follow the antigen expression profile of normal differentiation. Therefore, the approach to analysis of lymphoid neoplasias requires thoughtful utilization of laboratory testing, in order to meet both medical and economic goals of the laboratory and caregivers. The interpreter should expect to see a pattern of both positive and negative immunoreactivities that is appropriate to the final interpretation. The value and type of information provided by immunophenotyping in these malignancies varies and this paper outlines approaches for clinicians and laboratorians to follow when reviewing clinical data. The future for this technology is outstanding because it is the only one available today that can both rapidly and accurately measure multiple correlated cell properties. However, combined clinical-laboratory approach to diagnosis and prognostication seems to be important including traditional and newer (molecular genetic, molecular biology) methodologies.]

Hungarian Immunology

[Biologic therapies in the vasculitides]

SZÁNTÓ Antónia

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[Primary tubulointerstitial nephritis is characterised by an inflammatory infiltrate of tubulointerstitial space. The infiltrate consists of T and B lymphocytes, monocytes, macrophages, neutrophyl and eosinophyl granulocytes in varying degree. It is associated with interstitial oedema and different level of tubular damage. The disease exists in acute and chronic form. The main causes of this condition are: drugs, infection, systemic diseases, malignancy and in some cases the disease is idiopathic. The pathogenesis in most cases is immune-mediated. The secondary form of tubulointerstitial nephritis can occur in primary glomerular and vascular disease and is characterised by tubulointerstitial fibrosis and tubulus atrophy. The morphological alterations are major determinants of the progression of chronic renal disease. In both forms of tubulointerstitial nephritis the development of renal insufficiency is often observed.]

Hungarian Immunology

[MCP-1 (monocyte chemoattractant protein-1) G/A and T-bet (T-helper promoter factor) C/G polymorphisms in primary Sjögren’s syndrome and systemic lupus erythematosus]

KOVÁCS Attila, KONCZ Ágnes, ENDREFFY Emőke, ARANKA László, PETRI Ildikó, ELLER József, SZALAI Csaba

[INTRODUCTION - Monocyte chemoattractant protein- 1 (MCP-1) is a β-chemokine involved in the attraction and accumulation of mononuclear granulocytes towards the site of inflammation. One of the transcriptional factors of T-cells is called T-bet. PATIENTS AND METHODS - The authors investigated the MCP-1-2518 G/A and T-bet 310 C/G (His33Gln) polymorphisms evaluating the distribution of the specific genotypes in 45 patients with primary Sjögren's syndrome (pSS), 51 patients with systemic lupus erythematosus (SLE), and in 320 healthy blood donors as the control group. MCP-1-2518 G/A and T-bet 310 C/G polymorphisms were detected with molecular genetic methods from the purified genomic DNA. RESULTS - The frequency of the MCP-1-2518 AG heterozygous genotype decreased tendentiously only in SLE patients, while the frequency of the MCP-1 AA homozygous genotype increased comparing to the control group (13.7% vs. 5.9%; Pearson’s χ2 test=6.125, ns.). Analyzing the genotype frequency for the MCP-1 wild (GG) and AA homozygous genotypes in pSS group, the MCP-1 AA homozygous genotype proved to be more frequent comparing to the control group (82.8%:17.2% vs. 90.7%:9.3%; Pearson’s χ2 test 1.755, ns). These relations showed only tendentious association in the SLE group (81.6%:18.7% vs. 90.7%:9.3%; Pearson’s χ2 2.811, p=0.094, ns.) There was not any significant correlation between the investigated MCP-1- 2518 G/A and the T-bet 310 C/G polymorphisms and the TNF-α -308 G/A and -238 allele polymorphisms. The frequency of T-bet was equal in relation with heterozygous (CG) to wild CC genotype in the investigated two autoimmune disorders. The GG homozygous genotype for T-bet could not be found in SLE and pSS groups, likely to be a protective factor. CONCLUSIONS - The above mentioned polymorphisms didn’t show any significant correlation with TNF-α -308 and -238 allele polymorphisms. The further research of the MCP-1 G/A and T-bet C/G polymorphisms is important, because of their possible prognostic importance for SLE and pSS.]

Hypertension and nephrology

[Tubulointerstitial nephritis and uveitis syndrome]


[Tubulointerstitial nephritis and uveitis (TINU) syndrome is a rare and underdiagnosed oculorenal disorder that is characterized by the development of acute tubulointerstitial nephritis and uveitis. The median age of onset is 15 years, but it may occur at any age. There is a female predominance. Uveitis might occur before, after, and also concomitantly with tubulointerstitial nephritis. The symptoms are typically non-specific, including fever, loss of appetite, weight loss, nausea and vomiting, weakness, abdominal pain, arthralgias and myalgias. Laboratory findings reveal an acute impairment of renal function, anaemia and elevated inflammatory parameters. Urinary findings are consistent with tubulointerstitial nephritis including subnephrotic proteinuria, sterile leucocyturia, microhaematuria, and tubular dysfunction (e.g. normoglycemic glycosuria). The prognosis appears to be good, especially in children. Persistent renal dysfunction only develops in a small proportion of cases. In this paper, we present the case of a 39-year-old female patient with TINU syndrome, and review the literature.]