Β. ΤΖΑΒΑΡΑ, παθολόγος ανοσολόγος

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1 ΚΛΙΝΙΚΗ ΠΕΡΙΠΤΩΣΗ Β. ΤΖΑΒΑΡΑ, παθολόγος ανοσολόγος ΝΕΕΣ Κοργιαλένειο Μπενάκειο

2 60 υπονατριαιμία, σύγχυση Ιστορικό: 1997 DVT κάτω άκρου ( αντιπηκτική αγωγή 6 μήνες) 1998 (Φεβρουάριος) β ά παροδικό ΑΕΕ, CT εγκεφάλου: ισχαιμική βλάβη

3 1998 (Μαιος) περικαρδίτιδα, ρ βραδυψυχισμός (;) () υποθυρεοειδισμός ευρειαγγείες στις παρειές ΑΝΑ + sp, αντικεντρομεριδιακά Abs, ΑΘΑ +, acl IgG +. medrol 16mg, T4, sintrom 1999 ημικρανίες, αλλαγή θυμικού,υπέρταση παυσίπονα, αγχολυτικά, αντικαταθλιπτικά, Τ4, κορτικοειδή : + αρθραλγίες- αρθρίτιδα (;) ()

4 ΠΑΡΟΥΣΑ ΝΟΣΟΣ/ ΕΡΓΑΣΤΗΡΙΑΚΑ ΕΥΡΗΜΑΤΑ Να ούρων (αντιυπερτασικά) MRI εγκεφάλου: χρόνιες μικροαγγειακού τύπου βλάβες, λεπτομηνιγγική ενίσχυση ΟΝΠ: 14 λεμφοκύτταρα, λευκωμα/γλυκόζη=κφ Βασικός εργαστηριακός έλεγχος = κφ ΑΝΑ + 1/1280 sp,κεντρομερίδιο, C3/C4=κφ, acl IgG

5 ΙΑΓΝΩΣΗ Σκληρόδερμα (ssc,crest) Ε ΟΜΕΝΑ Τηλαγγειεκτασίες, ACA ΣΕΛ-προσβολή ΚΝΣ,, Acl abs, ψύχωση, δευτεροπαθές (ασηπτη μηνιγγίτιδα, αντιφωσφωλιπιδικό μικροισχαιμικές σύνδρ αλλοιώσεις), ΑΝΑ + ΠΡΟΒΛΗΜΑΤΙΣΜΟΙ-ΕΡΩΤΗΣΕΙΣ

7 J Rheumatol Jun;27(6): Clinical i l significance ifi of anticentromere t antibodies in patients t with systemic lupus erythematosus. Nakano M 1, Ohuchi Y, Hasegawa H, Kuroda T, Ito S, Gejyo F. Author information Abstract OBJECTIVE: To clarify the clinical significance of anticentromere antibodies (ACA) in patients with systemic lupus erythematosus (SLE). METHODS: Two hundred sixteen patients with SLE who were treated in our department were surveyed cross sectionally for the presence of ACA using indirect immunofluorescence on HEp-2 cell lines. ACA were identified by their discrete speckled pattern. Antibodies to the major centromere protein, CENP-B, were also studied with ELISA. Serial determinations of anti-cenp-b were carried out using stored serum samples, if available. RESULTS: ACA were recognized in 12 (5.6%) patients with SLE. All patients were receiving steroid therapy, with a mean dose of prednisolone of 14.4 mg/day. These patients also tested positive for anti-cenp-b with high titers despite the low serological disease activity in most. Three or more CREST features were observed in 2 patients and 2 others had no such features. Both patients without CREST features had a relatively short disease duration. The age at onset of SLE was significantly higher and Raynaud's phenomenon was more frequent in patients with ACA than in patients without ACA. In 8 of 10 patients tested, retrospective analysis using stored sera revealed no consistent change in anti- CENP-B titers over time. CONCLUSION: The presence of ACA in patients with SLE is apparently more frequent than previously believed. Patients with SLE with ACA may be a distinct subgroup. A longterm followup is warranted to fully determine the clinical significance of ACA in patients with SLE.

8 Scand J Rheumatol Jul-Aug;35(4): Anti-centromere antibodies in patients with systemic lupus erythematosus. Respaldiza N1, Wichmann I, Ocaña C, Garcia-Hernandez FJ, Castillo MJ, Magariño MI, Magariño R, Torres A, Sanchez-Roman J, Nuñez-Roldan A. Abstract Anti-centromere autoantibodies (ACA) are frequently detected in systemic sclerosis (SScl), especially in the calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia (CREST) syndrome, in which a prevalence of 55% has been reported. The presence of ACA in systemic lupus erythematosus (SLE) is so rare that its detection can raise serious doubts about the validity of the diagnosis. OBJECTIVE: To determine the frequency of ACA positive subjects from a wide monocentric cohort of SLE patients and analyse the clinical and biological characteristics of this group. METHODS: Five hundred and sixty consecutive SLE patients were systematically analysed for the presence of ACA and other autoantibodies using indirect immunofluorescence, counterimmunoelectrophoresis, double immunodiffusion, enzyme-linked immunosorbent assay (ELISA), and Western-blot. RESULTS: ACA were detected in 11 SLE patients (1.9%); all of them were women. The CENP- B-specific ELISA was positive in all patients. The main clinical features of scleroderma (cutaneous sclerosis, sclerodactylia, digital ulcers, or pulmonary fibrosis) were not present in these patients, who did not differ clinically from the whole SLE group. CONCLUSIONS: ACA can be detected in patients with genuine SLE without concurrent scleroderma. Therefore, the presence of this antibody does not preclude the possibility of the diagnosis of SLE. In addition, SLE patients with ACA do not represent a different clinical subgroup.

9 ΣΥΜΠΕΡΑΣΜΑ Ο τύπος φθορισμού των ΑΝΑ δεν καθορίζει την διάγνωση εφόσον η κλινική εικόνα είναι συμβατή.

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