Hungarian Immunology

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

SZÁNTÓ Antónia, CSÍPŐ István, ZEHER Margit

APRIL 20, 2003

Hungarian Immunology - 2003;2(02)

[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.]



Further articles in this publication

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

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

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


[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.]

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Clinical Neuroscience

Acute motor and sensory axonal neuropathy associated with Sjögren’s syndrome


Sjögren’s syndrome (SS) is an autoimmune disease with mononuclear cell infiltration and destruction of the lacrimal gland and salivary glands, which cause dryness of the eyes and mouth. The most common neurological condition seen in SS is peripheral neuropathy. Initial manifestation of SS as an acute fulminant peripheral neuropathy is extremely rare. We report a 42-year-old patient presenting with acute motor sensory-axonal neuropathy in the presence of SS. She showed partial response to intravenous immunoglobulin but favourable clinical improvement was seen after initiation of corticosteroid treatment.

Hungarian Immunology

[Undifferentiated connective tissue disease]


[Undifferentiated connective tissue disease (UCTD) is a term used by many authors to define a group a diffuse connective tissue disorders that lack definitive characteristics of any particular well-defined disorder. UCTD was diagnosed if the patients had at least two clinical symptoms and their sera contained one type of the anti nuclear antibody. Six hundred and sixty five patients with UCTD were followed between 1994 and 1999. The presence of the fever and anti-DNS antibodies correlated with SLE, arthritis/arthralgia and anti-RNP antibodies with MCTD, Raynaud phenomenon and ANA positivity with scleroderma, xerostomia/xerophtalmia and anti-SSA/SSB antibodies with Sjögren' syndromes, rheumatoid factor positivity and polyarthritis with rheumatoid arthritis. In conclusion, the UCTD represents a dynamic phase, one part of the patients show progression to definite connective tissue diseases, one part show regression, and on part of the patients stay in UCTD phase.]

Hungarian Immunology

[SS-A(Ro) and SS-B(La) autoantibodies in systemic lupus erythematosus]

SALLAI Krisztina, NAGY Eszter, GERGELY Péter

[OBJECTIVE - To assess the relation between clinical features and the presence of SS-A(Ro) and SS-B(La) autoantibodies in systemic lupus erythematosus. PATIENTS - The data of 200 patients with definite systemic lupus erythematosus were analysed. SSA( Ro) and SS-B(La) antibodies were assessed by enzyme immunoassay. RESULTS - 40.5% of systemic lupus erythematosus' patients were SS-A(Ro) and/or SS-B(La) antibody positive (’positive group’); the majority of such patients displayed both antibodies, 16.5% had SSA( Ro) antibodies alone, while only 2% has SS-B(La) antibodies alone. There were no differences in the occurrence of arthritis, secondary antiphospholipid syndrome and hematologic manifestations between the positive and negative groups; serositis was more common in the positive group. Skin manifestations, in particular subacute cutaneous lupus erythematosus and urticaria vasculitis were more frequent in the positive group, while kidney and central nervous system involvation, in particular severe forms were less frequent. Secondary Sjögren's syndrome occurred exclusively in antibody positive patients. Sm, RNP and Scl-70 antibodies were more frequently found in the positive group. CONCLUSIONS - The presence of SS-A(Ro) and/or SS-B(La) antibodies in systemic lupus erythematosus has some prognostic significance; in antibody-positive patients there is an increased risk for skin lesions (in particular subacute cutaneous lupus erythematosus and urticaria vasculitis) and secondary Sjögren’s syndrome and a decreased risk for severe nephritis or central nervous system involvement.]

Hungarian Immunology

[Importance of Raynaud’s phenomenon in the connective tissue diseases]

CZIRJÁK László, NAGY Zoltán

[In the presence of Raynaud’s phenomenon compression syndromes, vibration, disorders causing hyperviscosity or arterial lesion and drog induced vasospasm should be excluded. In the background of unilateral Raynaud’s phenomenon organic, morphological abnormalities are present. Raynaud's phenomenon may also be the first symptom of connective tissue diseases, therefore the follow up of these cases is required. In case of patients with primary Raynaud’s phenomenon, the probability of developing a connective tissue diseases is very low. In the presence of antinuclear antibody positivity and/or scleroderma capillary pattern by capillarmicroscopy, the follow up is important, because these cases may develop a connective tissue disease, which predominantly belong to the scleroderma family. Simultaneous presence of Raynaud’s phenomenon and inflammation also strongly indicates that a connective tissue disease may later develop.]