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Hungarian Immunology

JANUARY 20, 2003

[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

JANUARY 20, 2003

[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

JUNE 20, 2002

[Anti-synthatase syndrome]

PONYI Andrea, CONSTANTIN Tamás, DANKÓ Katalin

[The idiopathic inflammatory myopathies (IIM) are autoimmune diseases, characterized by symmetric proximal muscle weakness. Over the last several decades, many abnormalities of the cellular and humaral immune systems of IIM patients have been described. Some of these are autoantibodies unique to the IIM (the myositis specific autoantibodies MSA). The MSAs are antigen-driven, arise months prior to the onset of myositis, correlate in titre with disease-activity. Studies in the recent years proved that clinically and immuno-geneticaly different disease entities can be defined using myositis specific auto-antibodies. Their use make the serological classification. Myositis specific autoantibodies make it possible to define more homologies subgroups within polymyositis/dermatomyositis that may support the adequate treatment. The most common MSA is the antihistidyl- transfer RNA synthetase (anti-Jo-1), which can be found in 5-30% of patients with myositis. Patients with anti-synthatase antibodies tend to have characteristic clinical presentation of fever, small joint arthritis, intestinal lung disease, Raynaud's phenomenon, mechanic's hands and severe myositis. Sera of 65 PM/DM were tested for anti-Jo- 1 antibody. 15 patients (23%) had anti-Jo-1 antibody (10 PM, 5 DM). The patients with anti-Jo- 1 antibody has a significantly higher incidence of interstitial lung disease, arthritis, fever and Raynaud's phenomenon. These patients needed not only corticosteoid therapy, but other immunosupressive treatment. All 15 patients presented with the onset of weakness between February and July. The determination of myositis-specific autoantibodies has produced more homogenous grouping within the polymyositis/dermatomyositis patients.]

Lege Artis Medicinae

JANUARY 20, 2007

[PATHOLOGICAL FEATURES OF SYSTEMIC SCLEROSIS]

VARJÚ Cecília, KUMÁNOVICS Gábor, CZIRJÁK László

[Systemic sclerosis is characterized by fibrosis and subsequent atrophy of the skin and several internal organs as well as by generalized obliterative vasculopathy. The ethiology of systemic sclerosis is not quite clear yet, but the role of certain environmental factors, genetic properties and microchimaerism has been proven. Vasculopathy is a key feature that includes both functional changes (Raynaud's phenomenon) and morphological alterations (lesion of the endothel). The triggering event is the activation of endothelial cells. This is followed by an autoimmune inflammatory process causing vascular lesion, which will eventually lead to progressive pathologic fibrosis with increased deposition of collagen and intercellular matrix proteins. Normal tissues of vital internal organs will gradually loose structure, become atrophic and irreversibly damaged. In the treatment of systemic sclerosis the most significant achievements of the past decade have been made in the therapy and prevention of scleroderma renal crisis, pulmonary arterial hypertension and other vascular complications, resulting in higher survival rates and better quality of life. In pulmonary fibrosis the beneficial effect of cyclophosphamide therapy has been proven. Today, research focuses on new therapeutic approaches based on the recently clarified molecular pathological processes, as well as on laboratory and clinical markers that predict the activity of the disease or the efficiency of therapy. The aim of the present paper is to review current knowledge on the pathology of systemic sclerosis and provide help in the diagnosis, therapy and follow-up of the disease.]

Lege Artis Medicinae

SEPTEMBER 21, 2009

[Up-to-date management of systemic sclerosis]

SZŰCS Gabriella

[Systemic sclerosis is a chronic autoimmune disease characterized by three major features: widespread fibrosis in the skin and internal organs, a non-inflammatory small vessel obliterative vasculopathy and immunological activation with disease-specific autoantibodies. It is necessary to take a systematic approach to the diagnosis and evaluation of each case in order to provide appropriate treatment. Disease-modifying approaches can be classified according to the underlying pathogenic process. Thus vascular therapies include agents used for Raynaud’s phenomenon, critical digital ischaemia and organ-based vascular complications such as scleroderma renal crisis and pulmonary hypertension. Immunosuppressive drugs are used in lung involvement or rapid skin progression. The results of different anti-fibrotic therapies are controversial. Finally in managing organ-based manifestations and complications a multidisciplinary approach to the therapy is useful with patient education as an integral component of successful management.]

Hungarian Immunology

FEBRUARY 20, 2005

[Functional measurements of the hand’s circulation in Raynaud’s patients]

CSIKI Zoltán, GALUSKA László, GARAI Ildikó, SZABÓ Nóra, GALAJDA Zoltán, VARGA József, ZEHER Margit

[INTRODUCTION - In patients presenting with isolated tissue perfusion disturbance without large vessel involvement the examination of hand microcirculation is of major importance. In our study we present the results of three examination methods used for hand perfusion monitoring which measure the tissue microcirculation in different depths. PATIENTS AND METHODS - We examined 58 primary Raynaud's patients using capillary microscopy, laser doppler perfusion imaging and hand perfusion scintigraphy with Tc-99m-DTPA. From our patients 38 were smokers, 42 patients frequently suffered from headache. For the validation of our laser Doppler results we involved into the study also 16 non-smoker healthy volunteers. For the standardisation of the results the studies were performed in a climatised room. During the laser examination we performed and analysed also the post-occlusion hyperaemia test. RESULTS - In the majority of our patients no morphological alterations were found with capillary microscopy. In primary Raynaud’s patients the perfusion values measured with laser-Doppler scanner in basic condition were significantly lower both in fingers’ and palm’s regions compared to healthy controls. In the primary Raynaud’s group the smokers had significantly lower hyperaemic response than the non-smokers and we measured also significantly lower FPR (finger-to-palm ratio) values with hand perfusion scintigraphy. Both with laser doppler imaging and hand perfusion scintigraphy there was no difference between the values measured in patients with or without headache. CONCLUSIONS - We consider of benefit the functional examination of hand circulation in all diseases involving the hand microcirculation.]

Hungarian Immunology

JANUARY 20, 2003

[Undifferentiated connective tissue disease]

BODOLAY Edit, SZEGEDI Gyula

[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.]