Hungarian Immunology

[Undifferentiated connective tissue disease]

BODOLAY Edit, SZEGEDI Gyula

JANUARY 20, 2003

Hungarian Immunology - 2003;2(01)

[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.]

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Hungarian Immunology

[Hand perfusion scintigraphy in primary and secondary Raynaud's syndrome]

GARAI Ildikó, GALUSKA László, VARGA József, SZŰCS Gabriella, CSIKI Zoltán

[INTRODUCTION - The aim of the study was to investigate the circulatory characteristics of primary and secondary Raynaud’s syndrome patients using the hand perfusion scintigraphic method developed by the Nuclear Medicine Department of the DUMHC. PATIENTS AND METHODS - The authors examined 84 patients presenting classical symptoms of the disease: episodic ischemia with three-phase color change of the fingers. After visual evaluation theyanalyzed the obtained images quantitatively, using of the finger/palm ratio. Statistical analysis comparisons were made between the clinically separated primary and the secondary Raynaud's group. RESULTS - With the visual evaluation regional perfusion disturbances were frequently found in the secondary group - 37 from 51 patients - while only 2 from 33 patients in the primery group; the difference was statistically significant (p<0,001). The results of the quantitative analysis showed that the finger/palm ratio (FPR) values were significantly lower (p<0.05) in primary Raynaud’s group. In the FPR values there were not any differences between genders or the fingers of the right and left hands. CONCLUSIONS - The hand perfusion scintigraphy applying Tc-99m-DTPA is a noninvasive, cost effective diagnostic tool, which reflects objectively the global and regional microcirculatory abnormalities of the hands, and offers quantitative data for the follow-up.]

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.]

Hungarian Immunology

[Early arthritis]

SZEKANECZ Zoltán

[Definitive autoimmune-inflammatory rheumatic diseases are often preceded by undifferentiated clinical syndromes including undifferentiated polyarthritis (UDP), undifferentiated connective tissue disease (UCTD) and early rheumatoid arthritis (RA). These are considered as distinct clinical entities. However, due to extensive overlapping patterns in clinical picture and treatment, these diseases may be commonly termed as ”early arthritis”. The optimal follow-up of such patients may only be carried out at specialized early arthritis clinics. Effective patient care should include the early use of prognostic indicators, as well as flexible pharmacological therapy.]

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[INTRODUCTION - Significant stenosis of the internal carotid artery is frequently treated with stent placement. With growing clinical experience and usage of finer instrumentation, the incidence of periprocedural complications have reduced in larger centers. Two-thirds of the complications are postprocedural, due to the embolisation through the stent structure. Covered stents seem to be a good option against such embolisation. Our study demonstrates the efficiency, safety and feasibility of covered stent grafts, and the long term outcome of patients who underwent endovascular treatment of extracranial internal carotid artery stenoses, caused by highly embologenic plaques. MATERIALS AND METHODS - Between 2002 and 2003, 30 patients (22 male, 8 female, aged 50-89yrs, mean: 66 yrs) with 30 internal carotid artery stenoses having ipsilateral symptoms and/or stenotic lesions caused by irregular or ulcerated soft plaques or restenosis were treated with self-expanding covered stents (Symbiot, Boston Scientific). Predilatation and protecting devices were not used. Postdilatation was applied in every patient. Mean followup was 60 months (range 57-66 months), by Doppler ultrasonography as well as clinical examination. RESULTS - The degree of stenosis was found to range from 70% to subtotal occlusion. The plaque surfaces were irregular or ulcerated in 70%. The stenotic lesions were up to 30 mm in length. The narrowing of the internal carotid artery never extended to the common carotid artery. The technical success rate of stenting was 100%. The stents could be positioned with an accuracy of 2-3 mm. Periprocedurally, there were no neurological complications or deaths. During follow-up no strokes or stroke-related deaths occurred. Restenosis was found in two patients (6,6%) who underwent successful balloon dilatation. CONCLUSION - Our experience indicates that the covered stent is an efficient periprocedural and postprocedural “protecting device” to prevent neurological complications due to embolizations caused by high-risk plaques in stenotic lesions of extracranial internal carotid artery.]

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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.]

Hungarian Immunology

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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.]