Zapalenia naczyń w etiopatogenezie udaru mózgu

W przebiegu pierwotnych układowych zapaleń naczyń może dojść do wtórnego zajęcia naczyń w ośrodkowym układzie nerwowym. Współistnienie objawów neurologicznych z objawami ogólnymi (gorączka, brak apetytu z utratą masy ciała, męczliwość), a także z objawami zapalenia stawów, zmianami skórnymi lub objawami narządowymi wskazuje na konieczność przeprowadzenia diagnostyki różnicowej pod kątem zapalenia naczyń. Jest to heterogenna grupa chorób, która charakteryzuje się występowaniem zmian zapalnych i martwiczych w ścianie naczyniowej. Istnieje kilka klasyfikacji zapaleń naczyń, obecnie najczęściej stosuje się klasyfikację ustaloną na konferencji w Chapel Hill oraz klasyfikację American College of Rheumatology (ACR). Według kryteriów Chapel Hill Consensus Conference (CHCC ) pierwotne układowe zapalenia naczyń można podzielić na trzy grupy: z zajęciem dużych, średnich i drobnych naczyń. Inny podział wyróżnia zapalenia ziarniniakowe i nieziarniniakowe. Poza pierwotnymi układowymi zapaleniami naczyń można wyróżnić także pierwotne zapalenie naczyń ośrodkowego układu nerwowego (PACNS, primary angitis of the central nervous system), które jest rzadkim schorzeniem dotyczącym średnich i drobnych naczyń mózgu, bez objawów zajęcia innych narządów. Najgroźniejszym następstwem zapaleń naczyń jest udar mózgu — niedokrwienny lub krwotoczny, do innych objawów należą między innymi bóle głowy oraz encefalopatia i mielopatia. Polski Przegląd Neurologiczny 2011; 7 (2): 97–10

Bóle kręgosłupa w schorzeniach reumatycznych

W praktyce lekarza reumatologa bóle kręgosłupa należą do najczęstszych dolegliwości zgłaszanych przez chorych. Większość przypadków bólu ma charakter mechaniczny i trudną do ustalenia przyczynę; ustępują samoistnie w czasie 2–6 tygodni. Około 5% bólów kręgosłupa wiąże się z chorobą układową, przewlekłym zapaleniem czy poważnym uszkodzeniem struktur kręgosłupa. Jest wiele objawów alarmujących, nasuwających podejrzenie tych przyczyn. W niniejszej pracy przedstawiono przegląd najczęstszych układowych przyczyn bólów kręgosłupa, do których należą spondyloartropatie seronegatywne, reumatoidalne zapalenie stawów, zapalenie krążka międzykręgowego w przebiegu zakażenia, osteoporozę i inne. Polski Przegląd Neurologiczny 2010; 6 (2): 75–8

Progressive skin fibrosis is associated with a decline in lung function and worse survival in patients with diffuse cutaneous systemic sclerosis in the European Scleroderma Trials and Research (EUSTAR) cohort.

Objectives To determine whether progressive skin fibrosis is associated with visceral organ progression and mortality during follow-up in patients with diffuse cutaneous systemic sclerosis (dcSSc). Methods We evaluated patients from the European Scleroderma Trials and Research database with dcSSc, baseline modified Rodnan skin score (mRSS) ≥7, valid mRSS at 12±3 months after baseline and ≥1 annual follow-up visit. Progressive skin fibrosis was defined as an increase in mRSS >5 and ≥25% from baseline to 12±3 months. Outcomes were pulmonary, cardiovascular and renal progression, and all-cause death. Associations between skin progression and outcomes were evaluated by Kaplan-Meier survival analysis and multivariable Cox regression. Results Of 1021 included patients, 78 (7.6%) had progressive skin fibrosis (skin progressors). Median follow-up was 3.4 years. Survival analyses indicated that skin progressors had a significantly higher probability of FVC decline ≥10% (53.6% vs 34.4%; p<0.001) and all-cause death (15.4% vs 7.3%; p=0.003) than non-progressors. These significant associations were also found in subgroup analyses of patients with either low baseline mRSS (≤22/51) or short disease duration (≤15 months). In multivariable analyses, skin progression within 1 year was independently associated with FVC decline ≥10% (HR 1.79, 95% CI 1.20 to 2.65) and all-cause death (HR 2.58, 95% CI 1.31 to 5.09). Conclusions Progressive skin fibrosis within 1 year is associated with decline in lung function and worse survival in dcSSc during follow-up. These results confirm mRSS as a surrogate marker in dcSSc, which will be helpful for cohort enrichment in future trials and risk stratification in clinical practice

Racial differences in systemic sclerosis disease presentation: a European Scleroderma Trials and Research group study

Objectives. Racial factors play a significant role in SSc. We evaluated differences in SSc presentations between white patients (WP), Asian patients (AP) and black patients (BP) and analysed the effects of geographical locations.Methods. SSc characteristics of patients from the EUSTAR cohort were cross-sectionally compared across racial groups using survival and multiple logistic regression analyses.Results. The study included 9162 WP, 341 AP and 181 BP. AP developed the first non-RP feature faster than WP but slower than BP. AP were less frequently anti-centromere (ACA; odds ratio (OR) = 0.4, P < 0.001) and more frequently anti-topoisomerase-I autoantibodies (ATA) positive (OR = 1.2, P = 0.068), while BP were less likely to be ACA and ATA positive than were WP [OR(ACA) = 0.3, P < 0.001; OR(ATA) = 0.5, P = 0.020]. AP had less often (OR = 0.7, P = 0.06) and BP more often (OR = 2.7, P < 0.001) diffuse skin involvement than had WP.AP and BP were more likely to have pulmonary hypertension [OR(AP) = 2.6, P < 0.001; OR(BP) = 2.7, P = 0.03 vs WP] and a reduced forced vital capacity [OR(AP) = 2.5, P < 0.001; OR(BP) = 2.4, P < 0.004] than were WP. AP more often had an impaired diffusing capacity of the lung than had BP and WP [OR(AP vs BP) = 1.9, P = 0.038; OR(AP vs WP) = 2.4, P < 0.001]. After RP onset, AP and BP had a higher hazard to die than had WP [hazard ratio (HR) (AP) = 1.6, P = 0.011; HR(BP) = 2.1, P < 0.001].Conclusion. Compared with WP, and mostly independent of geographical location, AP have a faster and earlier disease onset with high prevalences of ATA, pulmonary hypertension and forced vital capacity impairment and higher mortality. BP had the fastest disease onset, a high prevalence of diffuse skin involvement and nominally the highest mortality

Przeciwciała anty-HSP70 w surowicy chorych na toczeń rumieniowaty układowy

Cel pracy: Celem pracy była ocena związku pomiędzy obecnościąprzeciwciał anty-HSP70 w surowicy chorych na toczeń rumieniowatyukładowy (systemic lupus erythematosus – SLE) a występowaniemwczesnych zmian miażdżycowych. Materiał i metody: Do badania zakwalifikowano 16 chorych naSLE w wieku 27–60 lat (średnia wieku 44,4 roku). Grupę kontrolnąstanowiło 13 zdrowych ochotników w wieku 24–57 lat(średnia wieku 43,1 roku). U chorych na SLE oraz w grupie kontrolnejwykonano badanie ultrasonograficzne tętnic szyjnychw celu oceny grubości kompleksu błony środkowej (intima-mediathicknes – IMT) oraz oceniono stężenie przeciwciał anty-HSP70w surowicy żylnej. Wyniki: W grupie chorych na SLE stwierdzono obustronnie istotniewiększą wartość IMT w tętnicy szyjnej wspólnej, tętnicy szyjnejwewnętrznej oraz opuszce tętnicy szyjnej wewnętrznej, coświadczy o istnieniu bardziej zaawansowanych zmianmiażdżycowych w tej grupie. W grupie chorych na SLE IMT korelowałaistotnie statystycznie z wiekiem. Ponadto, w grupiepacjentów z SLE stwierdzono ujemną korelację pomiędzystężeniem cholesterolu frakcji HDL a średnią IMT prawej tętnicy szyjnej wspólnej (RP = –0,54, p 0,05).Nie wykazano także związku pomiędzy IMT a stężeniem przeciwciałanty-HSP70 w badanych grupach. Wnioski: W przedstawionej pracy nie stwierdzono opisywanegow piśmiennictwie związku pomiędzy obecnością przeciwciałprzeciwko HSP70 a rozwojem miażdżycy u chorych na SLE

Incidence and predictors of cutaneous manifestations during the early course of systemic sclerosis: a 10-year longitudinal study from the EUSTAR database

Objectives: To longitudinally map the onset and identify risk factors for skin sclerosis and digital ulcers (DUs) in patients with systemic sclerosis (SSc) from an early time point after the onset of Raynaud's phenomenon (RP) in the European Scleroderma Trials and Research (EUSTAR) cohort. Methods: 695 patients with SSc with a baseline visit within 1 year after RP onset were followed in the prospective multinational EUSTAR database. During the 10-year observation period, cumulative probabilities of cutaneous lesions were assessed with the Kaplan–Meier method. Cox proportional hazards regression analysis was used to evaluate risk factors. Results: The median modified Rodnan skin score (mRSS) peaked 1 year after RP onset, and was 15 points. The 1-year probability to develop an mRSS ≥2 in at least one area of the arms and legs was 69% and 25%, respectively. Twenty-five per cent of patients developed diffuse cutaneous involvement in the first year after RP onset. This probability increased to 36% during the subsequent 2 years. Only 6% of patients developed diffuse cutaneous SSc thereafter. The probability to develop DUs increased to a maximum of 70% at the end of the 10-year observation. The main factors associated with diffuse cutaneous SSc were the presence of anti-RNA polymerase III autoantibodies, followed by antitopoisomerase autoantibodies and male sex. The main factor associated with incident DUs was the presence of antitopoisomerase autoantibodies. Conclusion: Early after RP onset, cutaneous manifestations exhibit rapid kinetics in SSc. This should be accounted for in clinical trials aiming to prevent skin worsening

A comparison between nailfold capillaroscopy patterns in adulthood in juvenile and adult-onset systemic sclerosis: a EUSTAR exploratory study

Objective: Qualitative capillaroscopy patterns in juvenile- and adult-onset systemic sclerosis (SSc) were studied in adulthood using data from the EULAR Scleroderma Trials and Research (EUSTAR) database. Methods: Data collected between June 2004 and April 2013 were examined with focus on capillaroscopy. In this retrospective exploratory study, series of patients with juvenile-onset SSc were matched with series of adult-onset SSc having the same gender and autoantibody profile. Results: 30 of 123 patientswith juvenile-onset and 2108 of 7133with adult-onset SSc had data on capillaroscopy. Juvenile-onset SSc showed scleroderma pattern more frequently than adult-onset SSc (93.3% and 88%). The OR was 2.44 and 95% CI 0.57–10.41. An active scleroderma pattern was present in 58% of juvenile- and 61% of adult-onset SSc. The OR was 0.91 and 95% CI 0.28–2.93. The late scleroderma pattern was present in 61% of juvenile- and 55.5% of adult-onset SSc. The OR was 1.06 and 95% CI 0.34–3.56. Conclusion: This is the first exploratory study on the comparison of capillaroscopy between juvenile- and adult-onset SSc in adulthood. Juvenile-onset SSc had an increase prevalence of scleroderma pattern, but a similar distribution of the three patterns was suggested. Further studies are needed to define this issue. © 2015 Elsevier Inc. All rights reserved

A gender gap in primary and secondary heart dysfunctions in systemic sclerosis: A EUSTAR prospective study

Objectives. In agreement with other autoimmune diseases, systemic sclerosis (SSc) is associated with a strong sex bias. However, unlike lupus, the effects of sex on disease phenotype and prognosis are poorly known. Therefore, we aimed to determine sex effects on outcomes. Method. We performed a prospective observational study using the latest 2013 data extract from the EULAR scleroderma trials and research (EUSTAR) cohort. We looked at (i) sex influence on disease characteristics at baseline and (ii) then focused on patients with at least 2 years of follow-up to estimate the effects of sex on disease progression and survival. Results. 9182 patients with SSc were available (1321 men) for the baseline analyses. In multivariate analysis, male sex was independently associated with a higher risk of diffuse cutaneous subtype (OR: 1.68, (1.45 to 1.94); p<0.001), a higher frequency of digital ulcers (OR: 1.28 (1.11 to 1.47); p<0.001) and pulmonary hypertension (OR: 3.01 (1.47 to 6.20); p<0.003). In the longitudinal analysis (n=4499), after a mean follow-up of 4.9 (±2.7) years, male sex was predictive of new onset of pulmonary hypertension (HR: 2.66 (1.32 to 5.36); p=0.006) and heart failure (HR: 2.22 (1.06 to 4.63); p=0.035). 908 deaths were recorded, male sex predicted deaths of all origins (HR: 1.48 (1.19 to 1.84); p<0.001), but did not significantly account for SSc-related deaths. Conclusions. Although more common in women, SSc appears as strikingly more severe in men. Our results obtained through the largest worldwide database demonstrate a higher risk of severe cardiovascular involvement in men. These results raise the point of including sex in the management and the decisionmaking process

Prediction of improvement in skin fibrosis in diffuse cutaneous systemic sclerosis: A EUSTAR analysis

none194Dobrota, Rucsandra; Maurer, Britta; Graf, Nicole; Jordan, Suzana; Mihai, Carina; Kowal-Bielecka, Otylia; Allanore, Yannick; Distler, Oliver; Cerinic, Marco Matucci; Guiducci, Serena; Walker, Ulrich; Lapadula, Giovanni; Iannone, Florenzo; Becvar, Radim; Sierakowsky, Stanislaw; Cutolo, Maurizio; Sulli, Alberto; Valentini, Gabriele; Cuomo, Giovanna; Vettori, Serena; Riemekasten, Gabriela; Siegert, Elise; Rednic, Simona; Nicoara, Ileana; Kahan, André; Vlachoyiannopoulos, P.; Montecucco, C.; Caporali, Roberto; Carreira, Patricia E.; Novak, Srdan; Czirják, László; Varju, Cecilia; Chizzolini, Carlo; Kucharz, Eugene J.; Kotulska, Anna; Kopec-Medrek, Magdalena; Widuchowska, Malgorzata; Cozzi, Franco; Rozman, Blaz; Mallia, Carmel; Coleiro, Bernard; Gabrielli, Armando; Farge, Dominique; Wu, Chen; Marjanovic, Zora; Faivre, Helene; Hij, Darin; Dhamadi, Roza; Airò, Paolo; Hesselstrand, Roger; Wollheim, Frank; Wuttge, Dirk M.; Andréasson, Kristofer; Martinovic, Duska; Balbir-Gurman, Alexandra; Braun-Moscovici, Yolanda; Trotta, F.; Monaco, Andrea Lo; Hunzelmann, Nicolas; Pellerito, Raffaele; Mauriziano, Ospedale; Bambara, Lisa Maria; Caramaschi, Paola; Black, Carol; Denton, Christopher; Damjanov, Nemanja; Henes, Jörg; Santamaria, Vera Ortiz; Heitmann, Stefan; Krasowska, Dorota; Seidel, Matthias; Burkhardt, Harald; Himsel, Andrea; Salvador, Maria J.; Da Silva, José Antonio Pereira; Stamenkovic, Bojana; Stankovic, Aleksandra; Tikly, Mohammed; Ananieva, Lidia P.; Denisov, Lev N.; Müller-Ladner, Ulf; Frerix, Marc; Tarner, Ingo; Scorza, Raffaella; Engelhart, Merete; Strauss, Gitte; Nielsen, Henrik; Damgaard, Kirsten; Mendoza, Antonio Zea; de la Puente, Carlos; Giraldo, Walter A. Sifuentes; Midtvedt, Øyvind; Reiseter, Silje; Hachulla, Eric; Launay, David; Valesini, Guido; Riccieri, Valeria; Ionescu, Ruxandra Maria; Opris, Daniela; Groseanu, Laura; Cornateanu, Roxana Sfrent; Ionitescu, Razvan; Gherghe, Ana Maria; Soare, Alina; Gorga, Marilena; Bojinca, Mihai; Schett, Georg; Distler, Jörg H.W.; Beyer, Christian; Meroni, Pierluigi; Ingegnoli, Francesca; Mouthon, Luc; Keyser, Filip De; Smith, Vanessa; Cantatore, Francesco P.; Corrado, Ada; Pozzi, Maria R.; Eyerich, Kilian; Hein, Rüdiger; Knott, Elisabeth; Wiland, Piotr; Szmyrka-Kaczmarek, Magdalena; Sokolik, Renata; Morgiel, Ewa; Madej, Marta; Krummel-Lorenz, Brigitte; Saar, Petra; Aringer, Martin; Günther, Claudia; Westhovens, Rene; de Langhe, Ellen; Lenaerts, Jan; Anic, Branimir; Baresic, Marko; Mayer, Miroslav; Radominski, Sebastião C.; de Souza Müller, Carolina; Azevedo, Valderílio F.; Agachi, Svetlana; Groppa, Liliana; Chiaburu, Lealea; Russu, Eugen; Popa, Sergei; Zenone, Thierry; Stebbings, Simon; Highton, John; Stamp, Lisa; Chapman, Peter; O'Donnell, John; Solanki, Kamal; Doube, Alan; Veale, Douglas; O'Rourke, Marie; Loyo, Esthela; Li, Mengtao; Rosato, Edoardo; Amoroso, Antonio; Gigante, Antonietta; Tanaseanu, Cristina-Mihaela; Popescu, Monica; Dumitrascu, Alina; Tiglea, Isabela; Foti, Rosario; Chirieac, Rodica; Ancuta, Codrina; Villiger, Peter; Adler, Sabine; de la Peña Lefebvre, Paloma García; Rubio, Silvia Rodriguez; Exposito, Marta Valero; Sibilia, Jean; Chatelus, Emmanuel; Gottenberg, Jacques Eric; Chifflot, Hélène; Litinsky, Ira; Venalis, Algirdas; Butrimiene, Irena; Venalis, Paulius; Rugiene, Rita; Karpec, Diana; Saketkoo, Lesley Ann; Lasky, Joseph A.; Kerzberg, Eduardo; Montoya, Fabiana; Cosentino, Vanesa; Limonta, Massimiliano; Brucato, Antonio Luca; Lupi, Elide; Spertini, François; Ribi, Camillo; Buss, Guillaume; Pasquali, Jean Louis; Martin, Thierry; Gorse, AudreyDobrota, Rucsandra; Maurer, Britta; Graf, Nicole; Jordan, Suzana; Mihai, Carina; Kowal Bielecka, Otylia; Allanore, Yannick; Distler, Oliver; Cerinic, Marco Matucci; Guiducci, Serena; Walker, Ulrich; Lapadula, Giovanni; Iannone, Florenzo; Becvar, Radim; Sierakowsky, Stanislaw; Cutolo, Maurizio; Sulli, Alberto; Valentini, Gabriele; Cuomo, Giovanna; Vettori, Serena; Riemekasten, Gabriela; Siegert, Elise; Rednic, Simona; Nicoara, Ileana; Kahan, André; Vlachoyiannopoulos, P.; Montecucco, Carlomaurizio; Caporali, Roberto; Carreira, Patricia E.; Novak, Srdan; Czirják, László; Varju, Cecilia; Chizzolini, Carlo; Kucharz, Eugene J.; Kotulska, Anna; Kopec Medrek, Magdalena; Widuchowska, Malgorzata; Cozzi, Franco; Rozman, Blaz; Mallia, Carmel; Coleiro, Bernard; Gabrielli, Armando; Farge, Dominique; Wu, Chen; Marjanovic, Zora; Faivre, Helene; Hij, Darin; Dhamadi, Roza; Airò, Paolo; Hesselstrand, Roger; Wollheim, Frank; Wuttge, Dirk M.; Andréasson, Kristofer; Martinovic, Duska; Balbir Gurman, Alexandra; Braun Moscovici, Yolanda; Trotta, F.; Monaco, Andrea Lo; Hunzelmann, Nicolas; Pellerito, Raffaele; Mauriziano, Ospedale; Bambara, Lisa Maria; Caramaschi, Paola; Black, Carol; Denton, Christopher; Damjanov, Nemanja; Henes, Jörg; Santamaria, Vera Ortiz; Heitmann, Stefan; Krasowska, Dorota; Seidel, Matthias; Burkhardt, Harald; Himsel, Andrea; Salvador, Maria J.; Da Silva, José Antonio Pereira; Stamenkovic, Bojana; Stankovic, Aleksandra; Tikly, Mohammed; Ananieva, Lidia P.; Denisov, Lev N.; Müller Ladner, Ulf; Frerix, Marc; Tarner, Ingo; Scorza, Raffaella; Engelhart, Merete; Strauss, Gitte; Nielsen, Henrik; Damgaard, Kirsten; Mendoza, Antonio Zea; de la Puente, Carlos; Giraldo, Walter A. Sifuentes; Midtvedt, Øyvind; Reiseter, Silje; Hachulla, Eric; Launay, David; Valesini, Guido; Riccieri, Valeria; Ionescu, Ruxandra Maria; Opris, Daniela; Groseanu, Laura; Cornateanu, Roxana Sfrent; Ionitescu, Razvan; Gherghe, Ana Maria; Soare, Alina; Gorga, Marilena; Bojinca, Mihai; Schett, Georg; Distler, Jörg H. W.; Beyer, Christian; Meroni, Pierluigi; Ingegnoli, Francesca; Mouthon, Luc; Keyser, Filip De; Smith, Vanessa; Cantatore, Francesco P.; Corrado, Ada; Pozzi, Maria R.; Eyerich, Kilian; Hein, Rüdiger; Knott, Elisabeth; Wiland, Piotr; Szmyrka Kaczmarek, Magdalena; Sokolik, Renata; Morgiel, Ewa; Madej, Marta; Krummel Lorenz, Brigitte; Saar, Petra; Aringer, Martin; Günther, Claudia; Westhovens, Rene; de Langhe, Ellen; Lenaerts, Jan; Anic, Branimir; Baresic, Marko; Mayer, Miroslav; Radominski, Sebastião C.; de Souza Müller, Carolina; Azevedo, Valderílio F.; Agachi, Svetlana; Groppa, Liliana; Chiaburu, Lealea; Russu, Eugen; Popa, Sergei; Zenone, Thierry; Stebbings, Simon; Highton, John; Stamp, Lisa; Chapman, Peter; O'Donnell, John; Solanki, Kamal; Doube, Alan; Veale, Douglas; O'Rourke, Marie; Loyo, Esthela; Li, Mengtao; Rosato, Edoardo; Amoroso, Antonio; Gigante, Antonietta; Tanaseanu, Cristina Mihaela; Popescu, Monica; Dumitrascu, Alina; Tiglea, Isabela; Foti, Rosario; Chirieac, Rodica; Ancuta, Codrina; Villiger, Peter; Adler, Sabine; de la Peña Lefebvre, Paloma García; Rubio, Silvia Rodriguez; Exposito, Marta Valero; Sibilia, Jean; Chatelus, Emmanuel; Gottenberg, Jacques Eric; Chifflot, Hélène; Litinsky, Ira; Venalis, Algirdas; Butrimiene, Irena; Venalis, Paulius; Rugiene, Rita; Karpec, Diana; Saketkoo, Lesley Ann; Lasky, Joseph A.; Kerzberg, Eduardo; Montoya, Fabiana; Cosentino, Vanesa; Limonta, Massimiliano; Brucato, Antonio Luca; Lupi, Elide; Spertini, François; Ribi, Camillo; Buss, Guillaume; Pasquali, Jean Louis; Martin, Thierry; Gorse, Audre