CUOMO, Giovanna

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CUOMO, Giovanna

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Dipartimento di Medicina di Precisione

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"To be or not to be," ten years after: evidence for mixed connective tissue disease as a distinct entity.

2012 Cappelli, S; Bellando Randone, S; Martinović, D; Tamas, Mm; Pasalić, K; Allanore, Y; Mosca, M; Talarico, R; Opris, D; Kiss, Cg; Tausche, Ak; Cardarelli, S; Riccieri, V; Koneva, O; Cuomo, Giovanna; Becker, Mo; Sulli, A; Guiducci, S; Radić, M; Bombardieri, S; Aringer, M; Cozzi, F; Valesini, G; Ananyeva, L; Valentini, Gabriele; Riemekasten, G; Cutolo, M; Ionescu, R; Czirják, L; Damjanov, N; Rednic, S; Matucci Cerinic, M.

-238 and +489 TNF-alpha along with TNF-RII gene polymorphisms associate with the diffuse phenotype in patients with Systemic Sclerosis

2005 Tolusso, B; Fabris, M; Caporali, R; Cuomo, Giovanna; Isola, M; Soldano, F; Montecucco, C; Valentini, Gabriele; Ferraccioli, G.

18. Imapired excerise performance in systemic sclerosis. Clinical correlations

2008 Valentini, G.; Santoriello, C.; Cuomo, Giovanna; Polverino, F.; Ruocco, L.; Polverino, M. .

[A comparison between the Simplified Disease Activity Index (SDAI) and the Disease Activity Score (DAS28) as measure of response to treatment in patients undergoing different therapeutic regimens].

2006 Cuomo, Giovanna; Molinaro, G; La Montagna, G; Migliaresi, S; Valentini, Gabriele

[Atherosclerosis and rheumatoid arthritis: relationships between intima-media thickness of the common carotid arteries and disease activity and disability].

2004 Cuomo, Giovanna; Di Micco, P; Niglio, A; La Montagna, G; Valentini, Gabriele

[FRI0311] CORRELATIONS BETWEEN CLINICAL FEATURES OF154 PATIENTS WITH SYSTEMIC SCLEROSIS (SSC) AND SHORT FORM36 (SF36) SCORES G. Cuomo, M. Iudici, G. Abignano, A. Petrillo, G. Valentini. Rheumatology Unit, Second University of Naples, Naples, Italy Background: The quality of life is reduced in patients with systemic sclerosis (SSc) as evaluated by both disease specific (HAQ-DI; SHAQ) or generic questionnaires. The correlations between SF-36 scores and some disease features have not yet been explored. Objectives: To evaluate the relationships between health values as evaluated by SF-36 and clinical features in SSc patients. Methods: From 9/01/07 to 8/31/08 154 consecutive SSc patients (144 females; 10 males); aged from 20–82 years (median age 54), with disease duration from 1-47 years (median 10,5 years); attending the outpatient clinic of the Rheumatology Unit of Second University of Naples, were enrolled in the study. The scores of the eight areas of SF-36 General Health: Physical Function (PF), Role Physical (RP), Bodily Pain (BP), General Health (GH), Vitality (VT), Social function (SF), Role Emotional (RE) and Mental Health (MH), were evaluated in the range of 0–100, the higher values reflecting a better Quality of life (Qol). In addition two global series i.e. the Physical Component Summary Score (PCS) and the Mental Component Summary Score (MCS) were calculated. The patients were also investigated for disease subset (limited or diffuse); skin involvement by the modified Rodnan skin score; disability by HAQ-DI; disease activity by European Activity Index, and disease severity by Medsger severity scale. Results: SF36 score did not differ between the 31 patients with diffuse vs the 123 with limited disease All the items of SF36 were significantly correlated to activity Index (Rho from –0.28 to -0.32: p<0.0001) and HAQ-DI (Rho from -0.38 to -0.82: p<0.0001). GH resulted to correlate with mRss (Rho -0.17; p<0.05) The significant correlations between each item of the Medsger's severity scale and each item of SF36 are reported in table. PF RP BP GH VT SF RE MH PCS/MCS Sev_Gen (Rho) – – – – – – – – –/– Sev_Vasc(Rho) – – – – – – – – –/– Sev_Skin (Rho) – – – – – – – – –/– Sev_Joint (Rho) – – – -0.18* – – – – -0.21/– Sev_muscle (Rho) -0.25 – – -0.16* -0.18* -0.22* – -0.20* -0.20/– Sev_GI (Rho) -0.20 – – -0.22 – – – – –/– Sev_lung (Rho) -0.28* – – -0.23** -0.17* -0.23 -0.22 – -0.19/– Sev_Heart (Rho) – – -0.22 – – – – – -0.21/– Sev_kidney (Rho) – – – – – – – – – p<0.05; p<0.001; *p<0.0001. Conclusion: The present study, confirms that the Health Value, as evaluated by SF36, is diminished in SSc. Our study point out a previously univestigated correlation between SF-36 scores and disease activity. Disclosure of Interest: NONE declared Ann Rheum Dis 2009;68(Suppl3):459 Session: Scleroderma, myositis and related syndromes

2009 Cuomo, Giovanna; Iudici, M.; Abignano, G.; Petrillo, A.; Valentini, G.

[FRI0313] CYCLOPHOSPHAMIDE PULSE THERAPY IN THE TREATMENT OF SYSTEMIC SCLEROSIS-INTERSTITIAL LUNG DISEASE G. Abignano, M. Iudici, A. Petrillo, G. Cuomo, G. Valentini. Rheumatology Unit, Second University of Naples, Napoli, Italy Background: Cyclophosphamide (CYC) is currently used in the treatment of interstitial lung disease (ILD) in patients with systemic sclerosis (SSc). A recent meta-analysis1 (Nannini C et al), pooling data from studies based on different entry criteria, questions its usefulness. Objectives: To investigate the effectiveness of low dose pulse CYC (500 mg/dose) in the treatment of recently deteriorating SSc-ILD. Methods: 51 patients with SSc-ILD, all of them satisfying ACR criteria for the classification of the disease and presenting with a recent (<6 months) decrease (≥10% of the predictive value) of either Forced Vital Capacity (FVC) or Diffusing Lung Capacity for CO (DLCO), were enrolled in the study. All of them underwent a concurrent prednisone therapy (10 mg/daily). CYC was administered i.v. at a dose of 500 mg weekly. Total CYC dose ranged from 4.5 to 11.5 g (median 7.5). An increase of 10% in either FVC or DLCO was considered indicative of improvement; a change between <10% and >10% of stable disease; a decrease ≥10% of worsening. Results: 22 out of the 51 SSc patients (43.14%) resulted to improve at the end of the CYC course; 17 (33.33%) remained stable; 12 (23.53%) worsened. No patients withdrew CYC treatment because of side effects. Conclusion: Our study suggests that the effectiveness of CYC in SSc-ILD may depend on entry criteria. Actually about 76% of patients who had experienced a recent deterioration of lung function, suggesting active alveolitis, underwent improvement or stabilization of their disease in our study. References:[ol][li]Nannini C, West CP, Erwin PJ, Matteson EL. Effects of cyclophosphamide on pulmonary function in patients with scleroderma and interstitial lung disease: a systematic review and meta-analysis of randomized controlled trials and observational prospective cohort studies. Arthritis Res Ther 2008, 10:R124.[/li][/ol]Disclosure of Interest: None declared Ann Rheum Dis 2009;68(Suppl3):460 Session: Scleroderma, myositis and related syndromes

2009 Abignano, G.; Iudici, M.; Petrillo, A.; Cuomo, Giovanna; Valentini, Gabriele

[HAQ-DI Italian version in systemic sclerosis].

2006 La Montagna, G; Cuomo, Giovanna; Chiarolanza, I; Ruocco, L; Valentini, Gabriele

[Hypocomplementemia in systemic sclerosis].

2008 Cuomo, Giovanna; Abignano, G; Ruocco, L; Vettori, Serena; Valentini, Gabriele

[OP0219] EUROPEAN SCLERODERMA STUDY GROUP (ESCSG) ACTIVITY INDEX IS CORRELATED TO QUALITY OF LIFE MEASURES BOTH AT ADMISSION AND OVERTIME M. Iudici, G. Cuomo, G. Abignano, G. Valentini. Rheumatology Unit, Second University of Naples, Naples, Italy Objectives: EScSG activity index has been validated for construct validity [1]. Its discriminant validity awaits to be investigated. In order to address this aspect, we investigated the relationship between the activity index and Health Assessment Questionnaire-Disability Index, physical and mental component scores (PCS and MCS) of Short Form-36 (SF36). Methods: 140 SSc patients consecutively admitted at tertiary center were investigated for EScSG activity index, HAQ-DI and PCS and MCS of SF36 at enrolment and after 1 year. The change (Δ) for each of the all measures was calculated. Results: EScSG activity index was found to be correlated to HAQ-DI, PCS and MCS both at admission (Rho=0.49, p=0.0003; Rho= -0.40, p<0.0001; Rho= -0.29, p=0.001, respectively) and after 1 year (Rho=0,64, p<0,0001; Rho= -0.29, p=0.001; Rho=-0.18, p=0.046 respectively). Moreover, the change between the value of the activity index at 1 year and that at admission (Δ activity index) was significantly correlated to ΔHAQ-DI, ΔPCS, ΔMCS (Rho=0,43, p=0.002; Rho=0,56, p<0.0001; Rho=0.48, p=0.002, respectively). Conclusion: HAQ-DI and SF36 (PCS and MCS) are considered to be validated outcome measure in SSc. Our results further support the construct validity of EScSG activity index and are for the first time underline its discriminant validity. References: • Valentini G. et al. European Scleroderma Study Group to define disease activity criteria for systemic sclerosis. III. Assessment of the construct validity of the preliminary activity criteria. Ann Rheum Dis 2003. Disclosure of Interest: None declared Citation: Ann Rheum Dis 2010;69(Suppl3):128 Session: Abstract Session: Modern prespective in systemic sclerosis

2010 Iudici, M.; Cuomo, Giovanna; Abignano, G.; Valentini, G.

[SAT0013] CANDIDATE GENE STUDY IN SYSTEMIC SCLEROSIS IDENTIFIES A RARE AND FUNCTIONAL VARIANT OF TNFAIP3 LOCUS AS A RISK FACTOR FOR INDIVIDUAL POLYAUTOIMMUNITY E. Koumakis1,2, M. Giraud2, P. Dieudé3, G. Cuomo4, P. Airo5, G. Chiocchia2, Y. Allanore1,2, and GENESYS Consortium. 1Paris Descartes University, Rheumatology A Department, Cochin Hospital; 2INSERM U1016, Institut Cochin, Sorbonne Paris Cité; 3Paris Diderot university, Rheumatology Department, Hôpital Bichat-Claude-Bernard, Paris, France; 4Rheumatology Section, Department of Clinical and Experimental Medicine, Second University of Naples, Naples; 5Rheumatology and Clinical Immunology, Spedali Civili, Brescia, Italy Background: Systemic lupus erythematosus (SLE) and systemic sclerosis (SSc) share some physiopathologic bases as supported by individual and familial polyautoimmunity and common susceptibility genetic factors. For the latter, there is a recent shift from the "common variant" to the "rare variant" paradigm, inasmuch as rare variants of TNFAIP3 and TREX1 with large effect size have recently been uncovered in SLE. Objectives: To investigate whether rare variants of TREX1 and TNFAIP3 are associated with SSc. Methods: TREX1 lupus-associated single-nucleotide polymorphisms (SNPs) rs3135946, rs7626978, rs3135943, rs11797 and TNFAIP3 rs9494883, rs72063345, rs5029939, rs2230926, rs117480515, rs7749323, together with the functional TT>-A dinucleotide variant located 41.5kb downstream of the TNFAIP3 promoter (1), were genotyped in a French "discovery" set consisting of 985 SSc and 1011 controls. The most relevant results were replicated in a second set consisting of Italian individuals (622 SSc and 493 controls). Expression of TNFAIP3 mRNA by peripheral blood mononuclear cells was assessed by quantitative real-time PCR using Taqman methodology in 38 SSc patients and 33 unaffected French donors genotyped for TNFAIP3 variants. Results: No association between any TREX1 variant and SSc or sub-phenotypes was observed. For TNFAIP3, we first observed that a low-frequency variant, rs117480515, tagged the TT>-A SLE dinucleotide polymorphism recently identified and that is highly suspected to be the causal variant (1). In the discovery sample, we observed that all tested TNFAIP3 variants were in linkage disequilibrium and were associated with SSc and various subsets including the polyautoimmune phenotype (i.e. SSc patients having at least one co-existing autoimmune disease, N=150, Padj=5.32×10-5, OR=3.94, [95%CI 2.25-6.90]). The rs117480515 SNP was subsequently genotyped in the replication sample. The allelic association with SSc was replicated solely for patients with the polyautoimmune phenotype, providing the following results in the combined French and Italian populations: Padj=8.58×10-9, OR=3.51, [95%CI 2.28-5.41]. Genotype-mRNA expression correlations revealed that the TNFAIP3 rs117480515 risk allele was associated with decreased TNFAIP3 mRNA expression (p=0.02). Conclusions: Our results establish the TNFAIP3 locus as susceptibility factor for the subset of SSc patients with a polyautoimmune phenotype and highlight the critical role of NFkB antagonist A20 in shared autoimmunity. It also supports the implication of rare/low-frequency functional variants in the genetic susceptibility to complex autoimmune diseases. The lack of association with the TREX1 locus in this study strengthens the knowledge that while some genetic loci may confer susceptibility to several autoimmune phenotypes, other genes may be restricted to specific diseases. References: Adrianto I et al. Association of a functional variant downstream of TNFAIP3 with systemic lupus erythematosus. Nat Genet 2011;43:253-8. Disclosure of Interest: None Declared Citation: Ann Rheum Dis 2012;71(Suppl3):475 Session: Genomics, genetics and epigenetics of rheumatic diseases

2012 Koumakis, E.; Giraud, M.; Dieudé, P.; Cuomo, Giovanna; Airo, P.; Chiocchia, G.; Allanore, Y.

[SAT0516] MEGACAPILLARIES AS DETECTED BY NAILFOLD VIDEOCAPILLAROSCOPY IN A COHORT OF PATIENTS WITH ACROCYANOSIS R. Irace, G. Cuomo, L. Pirro, G. Valentini. Department of Internal Medicine “F. Magrassi- A. Lanzara”, Rheumatology Section, Naples, Italy Background: Patients with acrocyanosis are known to display a capillaroscopic pattern characterised by normal/mild reduction of capillary density, microhemorrhages, asymmetrical capillary ectasias with greater width of venular loop and capillary thrombosis (1). At the best of our knowledge, one small series study only as so far reported the occurrence of megacapillaries (i.e. giant capillary: homogeneously enlarged loops with a diameter >50µm) in patients with acrocyanosis (2). Objectives: To investigate the presence of megacapillaries in a cohort of patients with acrocyanosis Methods: We enrolled 71 consecutive patients attending the Videocapillaroscopy Outpatient clinicof the Second University of Naples from 1st January 2011 to 1st June 2012 (median age 45 years, range 18-70) diagnosed to have acrocyanosis (i.e. persistent, symmetrical and painful cyanosis of extremities, triggered by cold, often associated to hyperhidrosis) and 35 control patients affected by osteoarthritis. Nailfold videocapillaroscopy was carried out with optical probes of 200X (VideoCap 2.5). Results: Megacapillaries (maximal loop width 80 µm) were detected in 14 out of 71 patients (19%). In 12 and 2 patients a mean score of 1 (less than 4 megacapillaries / mm) and of 2 (≥4 megacapillaries ≤ 6 / mm) was registered, respectively. In all patients the capillaroscopic alterations already described in patients with acrocyanosis were found : mild reduction of capillary density (mean capillary number 7±1/mm), asymmetrical capillary ectasias with greater width of venular loop, microhemorrhages, capillary thrombosis. In controls rare ectasias were the only capillaroscopic alterations detected. Conclusions: Our study confirms the possible occurrence of megacapillaries in a larger cohort of patients with acrocyanosis. It suggests the need of a careful clinical approach in order to make differential diagnosis between acrocyanosis and Raynaud's Phenomenon. The patients enrolled will be prospectically followed-up to assess the changes of capillaroscopic scores overtime. References: Kurklinsky et al. Vasc Med 2011 16(4):288-301 Monticone et a J Am Acad Dermatol 2000; 42:787-90 Davis E. Adv Microcirc 1982 ;10:101-119 Disclosure of Interest: None Declared Citation: Ann Rheum Dis 2013;72(Suppl3):756 Session: Diagnostics and imaging procedures

2013 Irace, R.; Cuomo, Giovanna; Pirro, L.; Valentini, G. .

[Survival and death causes in 251 systemic sclerosis patients from a single Italian center].

2010 Vettori, Serena; Cuomo, Giovanna; Abignano, G; Iudici, M; Valentini, Gabriele

[THU0309] CLINICAL MANIFESTATIONS IN LATE VS EARLY –ONSET SYSTEMIC SCLEROSIS (SSC) G. Cuomo, M. Iudici, G. Abignano, G. Valentini. Second Universty of Naples, Rheumatology Unit, Naples, Italy Background: Differences have been so far pointed out in the clinical manifestations and disease course in patients with a number of autoimmune rheumatic diseases and different age at onset (1-3). Objectives: To investigate the occurrence of differences between early and late onset SSc. Methods: 260 SSc patients (233 females) with a median age at the onset of the disease of 40.5 years (range 8-74) were consecutively admitted to a tertiary centre from November 1st 2000 to October 31st 2008. They were divided into 2 groups: early onset (<40.5 years) and late onset (≥40.5 years). Differences in each of the epidemiological, clinical and serological aspects collected in all the patients at admission were analysed. Results: Table 1 lists the significant differences detected between the 2 groups. Early onset patients were found to have a longer disease duration at presentation, a higher prevalence of anti-Scl70 positivity and a higher prevalence of scleroderma renal crisis; a lower prevalence of antinucleolar antibody positivity; a lower percentage of pulmonary hypertension; a lower HAQ-DI and a lower prevalence of heart involvement. No difference was detected in clinical subset distribution. Late onset (138) Early onset (122) p Disease duration mean (sd) 6 (6) 10 (10) 0.003 Range (median) 0.5-26 (5) 0.5-45 (6) Serological subset Scl70 43 54 0.04 Nucleolar 9 23 0.004 PAPs ≥40mmHG (echocardiography) 14/124 3/119 0.01 HAQ-DI mean (sd) 0.65 (0.7) 0.5 (0.7) 0.037 Range (median) 0-0.275 (0.375) 0-0.25 (0.125) Severity scale Late onset (138) Early onset(122) p 0-1 2-3-4 0-1 2-3-4 Heart 117 21 121 1 <0.0001 Kidney 138 0 117 5 0.02 Conclusion: Our study demonstrate that similarly to systemic lupus erithematosus and rheumatoid arthritis, distinct differences related to age at onset also occur in SSc patients. References: • Calvo-Alen J. et al, Clin Rheumatol 2005 • Lalani S. et al, J Rheumatol 2010 • Ho CT et al, Ann Rheum 1998 Disclosure of Interest: None declared Citation: Ann Rheum Dis 2010;69(Suppl3):247 Session: Scleroderma, Myositis and related syndromes

2010 Cuomo, Giovanna; Iudici, M.; Abignano, G.; Valentini, G.

A candidate gene study identifies a haplotype of CD2 as novel susceptibility factor for systemic sclerosis

2016 Koumakis, Eugenie; Bouaziz, Matthieu; Dieudé, Philippe; Cauvet, Anne; Ruiz, Barbara; Airò, Paolo; Cusi, Daniele; Matucci Cerinic, Marco; Salvi, Erika; Cuomo, Giovanna; Hachulla, Eric; Diot, Elisabeth; Caramaschi, Paola; Riccieri, Valeria; Avouac, Jerome; Allanore, Yannick

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

2015 Ingegnoli, F; Boracchi, P; Gualtierotti, R; Smith, V; Cutolo, M; Foeldvari, I; Airò, P; Alegre-Sancho, Jj; Allanore, Y; Ananieva, Lp; Ancuta, C; Andrade, Le; Aringer, M; Becvar, R; Bojinca, M; Butrimiene, I; Cantatore, Fp; Caporali, R; Caramaschi, P; Carreira, Pe; Chirieac, R; Corrado, A; Cosentino, V; Cuomo, G; Czirjak, L; Da Silva, Ja; la Peña Lefebvre, Pg; De Keyser, F; de Souza Müller, C; Damgaard, K; Damjanov, N; Denisov, Ln; Distler, O; Doube, A; Dumitrascu, A; Engelhart, M; Exposito, Mv; Eyerich, K; Farge-Bancel, D; Azevedo, Vf; Foti, R; Frerix, M; Gabrielli, A; Garen, T; Gottschalk, P; Groseanu, L; Guiducci, S; Günther, C; Hachulla, ; Hein, R; Heitmann, S; Henes, J; Hesselstrand, R; Highton, J; Iannone, F; Ionescu, Rm; Kayser, C; Karpec, D; Kerzberg, E; Kotulska, A; Kopec-Medrek, M; Kucharz, E; Krummel-Lorenz, B; Lauffer, F; Launay, D; Li, M; Litinsky, I; Loyo, E; Cerinic, Mm; Meroni, P; Midtvedt, Ø; Mihai, Cm; Montecucco, C; Montoya, F; Morgiel, E; Mouthon, L; Müller-Ladner, U; Nielsen, H; Opris, D; Ortiz-Santamaria, V; Otsa, K; Pileckyte, M; Pisarri, S; Popescu, M; Pozzi, Mr; Puppo, F; Radominski, Sc; Riccieri, V; Rosato, E; Rozman, B; Rubio, Sr; Rugiene, R; Saar, P; Salvador, Mj; Seidel, M; Sokolik, R; Solanki, K; Stamenkovic, B; Stankovic, A; Stebbings, S; Strauss, G; Sulli, A; Szamosi, S; Szechinski, J; Szmyrka-Kaczmarek, M; Szücs, G; Tanaseanu, Cm; Tiglea, I; Tyndall, A; Ughi, N; Uprus, M; Vacca, A; Varju, C; Venalis, A; Venalis, P; Walker, Ua; Widuchowska, M; Wiland, P; Wuttge, Dm; Zeni, S; Zenone, T.

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

2016 Elhai, M; Avouac, J; Walker, Ua; Matucci-Cerinic, M; Riemekasten, G; Airo, P; Hachulla, E; Valentini, G; Carreira, Pe; Cozzi, F; Gurman, Ab; Braun-Moscovici, Y; Damjanov, N; Ananieva, Lp; Scorza, R; Jimenez, S; Busquets, J; Mt, Li; Muller-Ladner, U; Kahan, A; Distler, O; Allanore, ; Y EUSTAR co-authors: Serena Guiducci, ; Alan, Tyndall; Giovanni, Lapadula; Florenzo, Iannone; Radim, Becvar; Stanislaw, Sierakowsky; Otylia Kowal Bielecka, ; Maurizio, Cutolo; Alberto, Sulli; Cuomo, Giovanna; Vettori, Serena; Simona, Rednic; Ileana, Nicoara; Vlachoyiannopoulos, P; Montecucco, C; Roberto, Caporali; Srdan, Novak; László, Czirják; Cecilia, Varju; Carlo, Chizzolini; Eugene, J Kucharz; Anna, Kotulska; Magdalena, Kopec-Medrek; Malgorzata, Widuchowska; Blaz, Rozman; Carmel, Mallia; Bernard, Coleiro; Armando, Gabrielli; Dominique, Farge; Adrian, Hij; Roger, Hesselstrand; Agneta, Scheja; Frank, Wollheim; Duska, Martinovic; Govoni, M; Andrea Lo Monaco, ; Nicolas, Hunzelmann; Raffaele, Pellerito; Lisa Maria Bambara, ; Paola, Caramaschi; Carol, Black; Christopher, Denton; Jörg, Henes; Vera Ortiz Santamaria, ; Stefan, Heitmann; Dorota, Krasowska; Matthias, Seidel; Mara, Oleszowsky; Harald, Burkhardt; Andrea, Himsel; Maria, J Salvador; Bojana, Stamenkovic; Aleksandra, Stankovic; Mohammed, Tikly; Maya, N Starovoytova; Merete, Engelhart; Gitte, Strauss; Henrik, Nielsen; Kirsten, Damgaard; Gabriella, Szücs; Antonio Zea Mendoza, ; Carlos de la Puente Buijdos, ; Walter, A Sifuentes Giraldo; Øyvind, Midtvedt; Torhild, Garen; David, Launay; Guido, Valesini; Valeria, Riccieri; Ruxandra Maria Ionescu, ; Daniela, Opris; Laura, Groseanu; Fredrick, M Wigley; Carmen, M Mihai; Roxana Sfrent Cornateanu, ; Razvan, Ionitescu; Ana Maria Gherghe, ; Marilena, Gorga; Rucsandra, Dobrota; Mihai, Bojinca; Georg, Schett; Jörg Hw Distler, ; Pierluigi, Meroni; Silvana, Zeni; Luc, Mouthon; Filip De Keyser, ; Vanessa, Smith; Francesco, P Cantatore; Ada, Corrado; Susanne, Ullman; Line, Iversen; Maria, R Pozzi; Kilian, Eyerich; Rüdiger, Hein; Elisabeth, Knott; Jacek, Szechinski; Piotr, Wiland; Magdalena, Szmyrka-Kaczmarek; Renata, Sokolik; Ewa, Morgiel; Brigitte, Krummel-Lorenz; Petra, Saar; Martin, Aringer; Claudia, Günther; Branimir, Anic; Marko, Baresic; Miroslav, Mayer; Sebastião, C Radominski; Carolina de Souza Müller, ; Valderílio, F Azevedo; Svetlana, Agachi; Liliana, Groppa; Lealea, Chiaburu; Eugen, Russu; Thierry, Zenone; Simon, Stebbings; John, Highton; Lisa, Stamp; Peter, Chapman; Murray, Baron; John, O'Donnell; Kamal, Solanki; Alan, Doube; Douglas, Veale; Marie, O'Rourke; Esthela, Loyo; Edoardo, Rosato; Simonetta, Pisarri; Cristina-Mihaela, Tanaseanu; Monica, Popescu; Alina, Dumitrascu; Isabela, Tiglea; Rodica, Chirieac; Codrina, Ancuta; Daniel, E Furst; Suzanne, Kafaja; Paloma García de la Peña Lefebvre, ; Silvia Rodriguez Rubio, ; Marta Valero Exposito, ; Jean, Sibilia; Emmanuel, Chatelus; Jacques Eric Gottenberg, ; Hélène, Chifflot; Ira, Litinsky; Algirdas, Venalis; Irena, Butrimiene; Paulius, Venalis; Rita, Rugiene; Diana, Karpec; Eduardo, Kerzberg; Fabiana, Montoya; Vanesa, Cosentino

A genetic variation located in the promoter region of the UPAR (CD87) gene is associated with the vascular complications of systemic sclerosis

2011 Manetti, M; Allanore, Y; Revillod, L; Fatini, C; Guiducci, S; Cuomo, Giovanna; Bonino, C; Riccieri, V; Bazzichi, L; Liakouli, V; Cipriani, P; Giacomelli, R; Abbate, R; Bombardieri, S; Valesini, G; Montecucco, C; Valentini, Gabriele; IBBA MANNESCHI, L; MATUCCI CERINIC, M.

A PILOT STUDY ON LOW-DOSE INTRAVENOUS CICLOPHOSPHAMIDE IN SYSTEMIC SCLEROSIS: EFFICACY, SAFETY AND EFFECTS ON CELLULAR ACTIVATION MARKERS

2002 D'Angelo, S.; Cuomo, Giovanna; Naclerio, C.; Abbadessa, Salvatore; Criscuolo, T.; Valentini, Gabriele

A regulatory variant in CCR6 is associated with susceptibility to antitopoisomerase-positive systemic sclerosis.

2013 Koumakis, E; Bouaziz, M; Dieudé, P; Ruiz, B; Riemekasten, G; Airo, P; Müller Nurasyid, M; Cusi, D; Matucci Cerinic, M; Melchers, I; Salvi, E; Strauch, K; Peters, A; Cuomo, Giovanna; Hachulla, E; Diot, E; Hunzelmann, N; Caramaschi, P; Riccieri, V; Distler, Jh; Tarner, I; Avouac, J; Letenneur, L; Amouyel, P; Lambert, Jc; Chiocchia, G; Boileau, C; Allanore, Y.

Titolo	Data di pubblicazione	Autore(i)
"To be or not to be," ten years after: evidence for mixed connective tissue disease as a distinct entity.	1-gen-2012	Cappelli, S; Bellando Randone, S; Martinović, D; Tamas, Mm; Pasalić, K; Allanore, Y; Mosca, M; Talarico, R; Opris, D; Kiss, Cg; Tausche, Ak; Cardarelli, S; Riccieri, V; Koneva, O; Cuomo, Giovanna; Becker, Mo; Sulli, A; Guiducci, S; Radić, M; Bombardieri, S; Aringer, M; Cozzi, F; Valesini, G; Ananyeva, L; Valentini, Gabriele; Riemekasten, G; Cutolo, M; Ionescu, R; Czirják, L; Damjanov, N; Rednic, S; Matucci Cerinic, M.
-238 and +489 TNF-alpha along with TNF-RII gene polymorphisms associate with the diffuse phenotype in patients with Systemic Sclerosis	1-gen-2005	Tolusso, B; Fabris, M; Caporali, R; Cuomo, Giovanna; Isola, M; Soldano, F; Montecucco, C; Valentini, Gabriele; Ferraccioli, G.
18. Imapired excerise performance in systemic sclerosis. Clinical correlations	1-gen-2008	Valentini, G.; Santoriello, C.; Cuomo, Giovanna; Polverino, F.; Ruocco, L.; Polverino, M. .
[A comparison between the Simplified Disease Activity Index (SDAI) and the Disease Activity Score (DAS28) as measure of response to treatment in patients undergoing different therapeutic regimens].	1-gen-2006	Cuomo, Giovanna; Molinaro, G; La Montagna, G; Migliaresi, S; Valentini, Gabriele
[Atherosclerosis and rheumatoid arthritis: relationships between intima-media thickness of the common carotid arteries and disease activity and disability].	1-gen-2004	Cuomo, Giovanna; Di Micco, P; Niglio, A; La Montagna, G; Valentini, Gabriele
[FRI0311] CORRELATIONS BETWEEN CLINICAL FEATURES OF154 PATIENTS WITH SYSTEMIC SCLEROSIS (SSC) AND SHORT FORM36 (SF36) SCORES G. Cuomo, M. Iudici, G. Abignano, A. Petrillo, G. Valentini. Rheumatology Unit, Second University of Naples, Naples, Italy Background: The quality of life is reduced in patients with systemic sclerosis (SSc) as evaluated by both disease specific (HAQ-DI; SHAQ) or generic questionnaires. The correlations between SF-36 scores and some disease features have not yet been explored. Objectives: To evaluate the relationships between health values as evaluated by SF-36 and clinical features in SSc patients. Methods: From 9/01/07 to 8/31/08 154 consecutive SSc patients (144 females; 10 males); aged from 20–82 years (median age 54), with disease duration from 1-47 years (median 10,5 years); attending the outpatient clinic of the Rheumatology Unit of Second University of Naples, were enrolled in the study. The scores of the eight areas of SF-36 General Health: Physical Function (PF), Role Physical (RP), Bodily Pain (BP), General Health (GH), Vitality (VT), Social function (SF), Role Emotional (RE) and Mental Health (MH), were evaluated in the range of 0–100, the higher values reflecting a better Quality of life (Qol). In addition two global series i.e. the Physical Component Summary Score (PCS) and the Mental Component Summary Score (MCS) were calculated. The patients were also investigated for disease subset (limited or diffuse); skin involvement by the modified Rodnan skin score; disability by HAQ-DI; disease activity by European Activity Index, and disease severity by Medsger severity scale. Results: SF36 score did not differ between the 31 patients with diffuse vs the 123 with limited disease All the items of SF36 were significantly correlated to activity Index (Rho from –0.28 to -0.32: p<0.0001) and HAQ-DI (Rho from -0.38 to -0.82: p<0.0001). GH resulted to correlate with mRss (Rho -0.17; p<0.05) The significant correlations between each item of the Medsger's severity scale and each item of SF36 are reported in table. PF RP BP GH VT SF RE MH PCS/MCS Sev_Gen (Rho) – – – – – – – – –/– Sev_Vasc(Rho) – – – – – – – – –/– Sev_Skin (Rho) – – – – – – – – –/– Sev_Joint (Rho) – – – -0.18* – – – – -0.21/– Sev_muscle (Rho) -0.25 – – -0.16* -0.18* -0.22* – -0.20* -0.20/– Sev_GI (Rho) -0.20 – – -0.22 – – – – –/– Sev_lung (Rho) -0.28* – – -0.23** -0.17* -0.23 -0.22 – -0.19/– Sev_Heart (Rho) – – -0.22 – – – – – -0.21/– Sev_kidney (Rho) – – – – – – – – – p<0.05; p<0.001; *p<0.0001. Conclusion: The present study, confirms that the Health Value, as evaluated by SF36, is diminished in SSc. Our study point out a previously univestigated correlation between SF-36 scores and disease activity. Disclosure of Interest: NONE declared Ann Rheum Dis 2009;68(Suppl3):459 Session: Scleroderma, myositis and related syndromes	1-gen-2009	Cuomo, Giovanna; Iudici, M.; Abignano, G.; Petrillo, A.; Valentini, G.
[FRI0313] CYCLOPHOSPHAMIDE PULSE THERAPY IN THE TREATMENT OF SYSTEMIC SCLEROSIS-INTERSTITIAL LUNG DISEASE G. Abignano, M. Iudici, A. Petrillo, G. Cuomo, G. Valentini. Rheumatology Unit, Second University of Naples, Napoli, Italy Background: Cyclophosphamide (CYC) is currently used in the treatment of interstitial lung disease (ILD) in patients with systemic sclerosis (SSc). A recent meta-analysis1 (Nannini C et al), pooling data from studies based on different entry criteria, questions its usefulness. Objectives: To investigate the effectiveness of low dose pulse CYC (500 mg/dose) in the treatment of recently deteriorating SSc-ILD. Methods: 51 patients with SSc-ILD, all of them satisfying ACR criteria for the classification of the disease and presenting with a recent (<6 months) decrease (≥10% of the predictive value) of either Forced Vital Capacity (FVC) or Diffusing Lung Capacity for CO (DLCO), were enrolled in the study. All of them underwent a concurrent prednisone therapy (10 mg/daily). CYC was administered i.v. at a dose of 500 mg weekly. Total CYC dose ranged from 4.5 to 11.5 g (median 7.5). An increase of 10% in either FVC or DLCO was considered indicative of improvement; a change between <10% and >10% of stable disease; a decrease ≥10% of worsening. Results: 22 out of the 51 SSc patients (43.14%) resulted to improve at the end of the CYC course; 17 (33.33%) remained stable; 12 (23.53%) worsened. No patients withdrew CYC treatment because of side effects. Conclusion: Our study suggests that the effectiveness of CYC in SSc-ILD may depend on entry criteria. Actually about 76% of patients who had experienced a recent deterioration of lung function, suggesting active alveolitis, underwent improvement or stabilization of their disease in our study. References:[ol][li]Nannini C, West CP, Erwin PJ, Matteson EL. Effects of cyclophosphamide on pulmonary function in patients with scleroderma and interstitial lung disease: a systematic review and meta-analysis of randomized controlled trials and observational prospective cohort studies. Arthritis Res Ther 2008, 10:R124.[/li][/ol]Disclosure of Interest: None declared Ann Rheum Dis 2009;68(Suppl3):460 Session: Scleroderma, myositis and related syndromes	1-gen-2009	Abignano, G.; Iudici, M.; Petrillo, A.; Cuomo, Giovanna; Valentini, Gabriele
[HAQ-DI Italian version in systemic sclerosis].	1-gen-2006	La Montagna, G; Cuomo, Giovanna; Chiarolanza, I; Ruocco, L; Valentini, Gabriele
[Hypocomplementemia in systemic sclerosis].	1-gen-2008	Cuomo, Giovanna; Abignano, G; Ruocco, L; Vettori, Serena; Valentini, Gabriele
[OP0219] EUROPEAN SCLERODERMA STUDY GROUP (ESCSG) ACTIVITY INDEX IS CORRELATED TO QUALITY OF LIFE MEASURES BOTH AT ADMISSION AND OVERTIME M. Iudici, G. Cuomo, G. Abignano, G. Valentini. Rheumatology Unit, Second University of Naples, Naples, Italy Objectives: EScSG activity index has been validated for construct validity [1]. Its discriminant validity awaits to be investigated. In order to address this aspect, we investigated the relationship between the activity index and Health Assessment Questionnaire-Disability Index, physical and mental component scores (PCS and MCS) of Short Form-36 (SF36). Methods: 140 SSc patients consecutively admitted at tertiary center were investigated for EScSG activity index, HAQ-DI and PCS and MCS of SF36 at enrolment and after 1 year. The change (Δ) for each of the all measures was calculated. Results: EScSG activity index was found to be correlated to HAQ-DI, PCS and MCS both at admission (Rho=0.49, p=0.0003; Rho= -0.40, p<0.0001; Rho= -0.29, p=0.001, respectively) and after 1 year (Rho=0,64, p<0,0001; Rho= -0.29, p=0.001; Rho=-0.18, p=0.046 respectively). Moreover, the change between the value of the activity index at 1 year and that at admission (Δ activity index) was significantly correlated to ΔHAQ-DI, ΔPCS, ΔMCS (Rho=0,43, p=0.002; Rho=0,56, p<0.0001; Rho=0.48, p=0.002, respectively). Conclusion: HAQ-DI and SF36 (PCS and MCS) are considered to be validated outcome measure in SSc. Our results further support the construct validity of EScSG activity index and are for the first time underline its discriminant validity. References: • Valentini G. et al. European Scleroderma Study Group to define disease activity criteria for systemic sclerosis. III. Assessment of the construct validity of the preliminary activity criteria. Ann Rheum Dis 2003. Disclosure of Interest: None declared Citation: Ann Rheum Dis 2010;69(Suppl3):128 Session: Abstract Session: Modern prespective in systemic sclerosis	1-gen-2010	Iudici, M.; Cuomo, Giovanna; Abignano, G.; Valentini, G.
[SAT0013] CANDIDATE GENE STUDY IN SYSTEMIC SCLEROSIS IDENTIFIES A RARE AND FUNCTIONAL VARIANT OF TNFAIP3 LOCUS AS A RISK FACTOR FOR INDIVIDUAL POLYAUTOIMMUNITY E. Koumakis1,2, M. Giraud2, P. Dieudé3, G. Cuomo4, P. Airo5, G. Chiocchia2, Y. Allanore1,2, and GENESYS Consortium. 1Paris Descartes University, Rheumatology A Department, Cochin Hospital; 2INSERM U1016, Institut Cochin, Sorbonne Paris Cité; 3Paris Diderot university, Rheumatology Department, Hôpital Bichat-Claude-Bernard, Paris, France; 4Rheumatology Section, Department of Clinical and Experimental Medicine, Second University of Naples, Naples; 5Rheumatology and Clinical Immunology, Spedali Civili, Brescia, Italy Background: Systemic lupus erythematosus (SLE) and systemic sclerosis (SSc) share some physiopathologic bases as supported by individual and familial polyautoimmunity and common susceptibility genetic factors. For the latter, there is a recent shift from the "common variant" to the "rare variant" paradigm, inasmuch as rare variants of TNFAIP3 and TREX1 with large effect size have recently been uncovered in SLE. Objectives: To investigate whether rare variants of TREX1 and TNFAIP3 are associated with SSc. Methods: TREX1 lupus-associated single-nucleotide polymorphisms (SNPs) rs3135946, rs7626978, rs3135943, rs11797 and TNFAIP3 rs9494883, rs72063345, rs5029939, rs2230926, rs117480515, rs7749323, together with the functional TT>-A dinucleotide variant located 41.5kb downstream of the TNFAIP3 promoter (1), were genotyped in a French "discovery" set consisting of 985 SSc and 1011 controls. The most relevant results were replicated in a second set consisting of Italian individuals (622 SSc and 493 controls). Expression of TNFAIP3 mRNA by peripheral blood mononuclear cells was assessed by quantitative real-time PCR using Taqman methodology in 38 SSc patients and 33 unaffected French donors genotyped for TNFAIP3 variants. Results: No association between any TREX1 variant and SSc or sub-phenotypes was observed. For TNFAIP3, we first observed that a low-frequency variant, rs117480515, tagged the TT>-A SLE dinucleotide polymorphism recently identified and that is highly suspected to be the causal variant (1). In the discovery sample, we observed that all tested TNFAIP3 variants were in linkage disequilibrium and were associated with SSc and various subsets including the polyautoimmune phenotype (i.e. SSc patients having at least one co-existing autoimmune disease, N=150, Padj=5.32×10-5, OR=3.94, [95%CI 2.25-6.90]). The rs117480515 SNP was subsequently genotyped in the replication sample. The allelic association with SSc was replicated solely for patients with the polyautoimmune phenotype, providing the following results in the combined French and Italian populations: Padj=8.58×10-9, OR=3.51, [95%CI 2.28-5.41]. Genotype-mRNA expression correlations revealed that the TNFAIP3 rs117480515 risk allele was associated with decreased TNFAIP3 mRNA expression (p=0.02). Conclusions: Our results establish the TNFAIP3 locus as susceptibility factor for the subset of SSc patients with a polyautoimmune phenotype and highlight the critical role of NFkB antagonist A20 in shared autoimmunity. It also supports the implication of rare/low-frequency functional variants in the genetic susceptibility to complex autoimmune diseases. The lack of association with the TREX1 locus in this study strengthens the knowledge that while some genetic loci may confer susceptibility to several autoimmune phenotypes, other genes may be restricted to specific diseases. References: Adrianto I et al. Association of a functional variant downstream of TNFAIP3 with systemic lupus erythematosus. Nat Genet 2011;43:253-8. Disclosure of Interest: None Declared Citation: Ann Rheum Dis 2012;71(Suppl3):475 Session: Genomics, genetics and epigenetics of rheumatic diseases	1-gen-2012	Koumakis, E.; Giraud, M.; Dieudé, P.; Cuomo, Giovanna; Airo, P.; Chiocchia, G.; Allanore, Y.
[SAT0516] MEGACAPILLARIES AS DETECTED BY NAILFOLD VIDEOCAPILLAROSCOPY IN A COHORT OF PATIENTS WITH ACROCYANOSIS R. Irace, G. Cuomo, L. Pirro, G. Valentini. Department of Internal Medicine “F. Magrassi- A. Lanzara”, Rheumatology Section, Naples, Italy Background: Patients with acrocyanosis are known to display a capillaroscopic pattern characterised by normal/mild reduction of capillary density, microhemorrhages, asymmetrical capillary ectasias with greater width of venular loop and capillary thrombosis (1). At the best of our knowledge, one small series study only as so far reported the occurrence of megacapillaries (i.e. giant capillary: homogeneously enlarged loops with a diameter >50µm) in patients with acrocyanosis (2). Objectives: To investigate the presence of megacapillaries in a cohort of patients with acrocyanosis Methods: We enrolled 71 consecutive patients attending the Videocapillaroscopy Outpatient clinicof the Second University of Naples from 1st January 2011 to 1st June 2012 (median age 45 years, range 18-70) diagnosed to have acrocyanosis (i.e. persistent, symmetrical and painful cyanosis of extremities, triggered by cold, often associated to hyperhidrosis) and 35 control patients affected by osteoarthritis. Nailfold videocapillaroscopy was carried out with optical probes of 200X (VideoCap 2.5). Results: Megacapillaries (maximal loop width 80 µm) were detected in 14 out of 71 patients (19%). In 12 and 2 patients a mean score of 1 (less than 4 megacapillaries / mm) and of 2 (≥4 megacapillaries ≤ 6 / mm) was registered, respectively. In all patients the capillaroscopic alterations already described in patients with acrocyanosis were found : mild reduction of capillary density (mean capillary number 7±1/mm), asymmetrical capillary ectasias with greater width of venular loop, microhemorrhages, capillary thrombosis. In controls rare ectasias were the only capillaroscopic alterations detected. Conclusions: Our study confirms the possible occurrence of megacapillaries in a larger cohort of patients with acrocyanosis. It suggests the need of a careful clinical approach in order to make differential diagnosis between acrocyanosis and Raynaud's Phenomenon. The patients enrolled will be prospectically followed-up to assess the changes of capillaroscopic scores overtime. References: Kurklinsky et al. Vasc Med 2011 16(4):288-301 Monticone et a J Am Acad Dermatol 2000; 42:787-90 Davis E. Adv Microcirc 1982 ;10:101-119 Disclosure of Interest: None Declared Citation: Ann Rheum Dis 2013;72(Suppl3):756 Session: Diagnostics and imaging procedures	1-gen-2013	Irace, R.; Cuomo, Giovanna; Pirro, L.; Valentini, G. .
[Survival and death causes in 251 systemic sclerosis patients from a single Italian center].	1-gen-2010	Vettori, Serena; Cuomo, Giovanna; Abignano, G; Iudici, M; Valentini, Gabriele
[THU0309] CLINICAL MANIFESTATIONS IN LATE VS EARLY –ONSET SYSTEMIC SCLEROSIS (SSC) G. Cuomo, M. Iudici, G. Abignano, G. Valentini. Second Universty of Naples, Rheumatology Unit, Naples, Italy Background: Differences have been so far pointed out in the clinical manifestations and disease course in patients with a number of autoimmune rheumatic diseases and different age at onset (1-3). Objectives: To investigate the occurrence of differences between early and late onset SSc. Methods: 260 SSc patients (233 females) with a median age at the onset of the disease of 40.5 years (range 8-74) were consecutively admitted to a tertiary centre from November 1st 2000 to October 31st 2008. They were divided into 2 groups: early onset (<40.5 years) and late onset (≥40.5 years). Differences in each of the epidemiological, clinical and serological aspects collected in all the patients at admission were analysed. Results: Table 1 lists the significant differences detected between the 2 groups. Early onset patients were found to have a longer disease duration at presentation, a higher prevalence of anti-Scl70 positivity and a higher prevalence of scleroderma renal crisis; a lower prevalence of antinucleolar antibody positivity; a lower percentage of pulmonary hypertension; a lower HAQ-DI and a lower prevalence of heart involvement. No difference was detected in clinical subset distribution. Late onset (138) Early onset (122) p Disease duration mean (sd) 6 (6) 10 (10) 0.003 Range (median) 0.5-26 (5) 0.5-45 (6) Serological subset Scl70 43 54 0.04 Nucleolar 9 23 0.004 PAPs ≥40mmHG (echocardiography) 14/124 3/119 0.01 HAQ-DI mean (sd) 0.65 (0.7) 0.5 (0.7) 0.037 Range (median) 0-0.275 (0.375) 0-0.25 (0.125) Severity scale Late onset (138) Early onset(122) p 0-1 2-3-4 0-1 2-3-4 Heart 117 21 121 1 <0.0001 Kidney 138 0 117 5 0.02 Conclusion: Our study demonstrate that similarly to systemic lupus erithematosus and rheumatoid arthritis, distinct differences related to age at onset also occur in SSc patients. References: • Calvo-Alen J. et al, Clin Rheumatol 2005 • Lalani S. et al, J Rheumatol 2010 • Ho CT et al, Ann Rheum 1998 Disclosure of Interest: None declared Citation: Ann Rheum Dis 2010;69(Suppl3):247 Session: Scleroderma, Myositis and related syndromes	1-gen-2010	Cuomo, Giovanna; Iudici, M.; Abignano, G.; Valentini, G.
A candidate gene study identifies a haplotype of CD2 as novel susceptibility factor for systemic sclerosis	1-gen-2016	Koumakis, Eugenie; Bouaziz, Matthieu; Dieudé, Philippe; Cauvet, Anne; Ruiz, Barbara; Airò, Paolo; Cusi, Daniele; Matucci Cerinic, Marco; Salvi, Erika; Cuomo, Giovanna; Hachulla, Eric; Diot, Elisabeth; Caramaschi, Paola; Riccieri, Valeria; Avouac, Jerome; Allanore, Yannick
A comparison between nailfold capillaroscopy patterns in adulthood in juvenile and adult-onset systemic sclerosis: A EUSTAR exploratory study	1-gen-2015	Ingegnoli, F; Boracchi, P; Gualtierotti, R; Smith, V; Cutolo, M; Foeldvari, I; Airò, P; Alegre-Sancho, Jj; Allanore, Y; Ananieva, Lp; Ancuta, C; Andrade, Le; Aringer, M; Becvar, R; Bojinca, M; Butrimiene, I; Cantatore, Fp; Caporali, R; Caramaschi, P; Carreira, Pe; Chirieac, R; Corrado, A; Cosentino, V; Cuomo, G; Czirjak, L; Da Silva, Ja; la Peña Lefebvre, Pg; De Keyser, F; de Souza Müller, C; Damgaard, K; Damjanov, N; Denisov, Ln; Distler, O; Doube, A; Dumitrascu, A; Engelhart, M; Exposito, Mv; Eyerich, K; Farge-Bancel, D; Azevedo, Vf; Foti, R; Frerix, M; Gabrielli, A; Garen, T; Gottschalk, P; Groseanu, L; Guiducci, S; Günther, C; Hachulla, ; Hein, R; Heitmann, S; Henes, J; Hesselstrand, R; Highton, J; Iannone, F; Ionescu, Rm; Kayser, C; Karpec, D; Kerzberg, E; Kotulska, A; Kopec-Medrek, M; Kucharz, E; Krummel-Lorenz, B; Lauffer, F; Launay, D; Li, M; Litinsky, I; Loyo, E; Cerinic, Mm; Meroni, P; Midtvedt, Ø; Mihai, Cm; Montecucco, C; Montoya, F; Morgiel, E; Mouthon, L; Müller-Ladner, U; Nielsen, H; Opris, D; Ortiz-Santamaria, V; Otsa, K; Pileckyte, M; Pisarri, S; Popescu, M; Pozzi, Mr; Puppo, F; Radominski, Sc; Riccieri, V; Rosato, E; Rozman, B; Rubio, Sr; Rugiene, R; Saar, P; Salvador, Mj; Seidel, M; Sokolik, R; Solanki, K; Stamenkovic, B; Stankovic, A; Stebbings, S; Strauss, G; Sulli, A; Szamosi, S; Szechinski, J; Szmyrka-Kaczmarek, M; Szücs, G; Tanaseanu, Cm; Tiglea, I; Tyndall, A; Ughi, N; Uprus, M; Vacca, A; Varju, C; Venalis, A; Venalis, P; Walker, Ua; Widuchowska, M; Wiland, P; Wuttge, Dm; Zeni, S; Zenone, T.
A gender gap in primary and secondary heart dysfunctions in systemic sclerosis: a EUSTAR prospective study	1-gen-2016	Elhai, M; Avouac, J; Walker, Ua; Matucci-Cerinic, M; Riemekasten, G; Airo, P; Hachulla, E; Valentini, G; Carreira, Pe; Cozzi, F; Gurman, Ab; Braun-Moscovici, Y; Damjanov, N; Ananieva, Lp; Scorza, R; Jimenez, S; Busquets, J; Mt, Li; Muller-Ladner, U; Kahan, A; Distler, O; Allanore, ; Y EUSTAR co-authors: Serena Guiducci, ; Alan, Tyndall; Giovanni, Lapadula; Florenzo, Iannone; Radim, Becvar; Stanislaw, Sierakowsky; Otylia Kowal Bielecka, ; Maurizio, Cutolo; Alberto, Sulli; Cuomo, Giovanna; Vettori, Serena; Simona, Rednic; Ileana, Nicoara; Vlachoyiannopoulos, P; Montecucco, C; Roberto, Caporali; Srdan, Novak; László, Czirják; Cecilia, Varju; Carlo, Chizzolini; Eugene, J Kucharz; Anna, Kotulska; Magdalena, Kopec-Medrek; Malgorzata, Widuchowska; Blaz, Rozman; Carmel, Mallia; Bernard, Coleiro; Armando, Gabrielli; Dominique, Farge; Adrian, Hij; Roger, Hesselstrand; Agneta, Scheja; Frank, Wollheim; Duska, Martinovic; Govoni, M; Andrea Lo Monaco, ; Nicolas, Hunzelmann; Raffaele, Pellerito; Lisa Maria Bambara, ; Paola, Caramaschi; Carol, Black; Christopher, Denton; Jörg, Henes; Vera Ortiz Santamaria, ; Stefan, Heitmann; Dorota, Krasowska; Matthias, Seidel; Mara, Oleszowsky; Harald, Burkhardt; Andrea, Himsel; Maria, J Salvador; Bojana, Stamenkovic; Aleksandra, Stankovic; Mohammed, Tikly; Maya, N Starovoytova; Merete, Engelhart; Gitte, Strauss; Henrik, Nielsen; Kirsten, Damgaard; Gabriella, Szücs; Antonio Zea Mendoza, ; Carlos de la Puente Buijdos, ; Walter, A Sifuentes Giraldo; Øyvind, Midtvedt; Torhild, Garen; David, Launay; Guido, Valesini; Valeria, Riccieri; Ruxandra Maria Ionescu, ; Daniela, Opris; Laura, Groseanu; Fredrick, M Wigley; Carmen, M Mihai; Roxana Sfrent Cornateanu, ; Razvan, Ionitescu; Ana Maria Gherghe, ; Marilena, Gorga; Rucsandra, Dobrota; Mihai, Bojinca; Georg, Schett; Jörg Hw Distler, ; Pierluigi, Meroni; Silvana, Zeni; Luc, Mouthon; Filip De Keyser, ; Vanessa, Smith; Francesco, P Cantatore; Ada, Corrado; Susanne, Ullman; Line, Iversen; Maria, R Pozzi; Kilian, Eyerich; Rüdiger, Hein; Elisabeth, Knott; Jacek, Szechinski; Piotr, Wiland; Magdalena, Szmyrka-Kaczmarek; Renata, Sokolik; Ewa, Morgiel; Brigitte, Krummel-Lorenz; Petra, Saar; Martin, Aringer; Claudia, Günther; Branimir, Anic; Marko, Baresic; Miroslav, Mayer; Sebastião, C Radominski; Carolina de Souza Müller, ; Valderílio, F Azevedo; Svetlana, Agachi; Liliana, Groppa; Lealea, Chiaburu; Eugen, Russu; Thierry, Zenone; Simon, Stebbings; John, Highton; Lisa, Stamp; Peter, Chapman; Murray, Baron; John, O'Donnell; Kamal, Solanki; Alan, Doube; Douglas, Veale; Marie, O'Rourke; Esthela, Loyo; Edoardo, Rosato; Simonetta, Pisarri; Cristina-Mihaela, Tanaseanu; Monica, Popescu; Alina, Dumitrascu; Isabela, Tiglea; Rodica, Chirieac; Codrina, Ancuta; Daniel, E Furst; Suzanne, Kafaja; Paloma García de la Peña Lefebvre, ; Silvia Rodriguez Rubio, ; Marta Valero Exposito, ; Jean, Sibilia; Emmanuel, Chatelus; Jacques Eric Gottenberg, ; Hélène, Chifflot; Ira, Litinsky; Algirdas, Venalis; Irena, Butrimiene; Paulius, Venalis; Rita, Rugiene; Diana, Karpec; Eduardo, Kerzberg; Fabiana, Montoya; Vanesa, Cosentino
A genetic variation located in the promoter region of the UPAR (CD87) gene is associated with the vascular complications of systemic sclerosis	1-gen-2011	Manetti, M; Allanore, Y; Revillod, L; Fatini, C; Guiducci, S; Cuomo, Giovanna; Bonino, C; Riccieri, V; Bazzichi, L; Liakouli, V; Cipriani, P; Giacomelli, R; Abbate, R; Bombardieri, S; Valesini, G; Montecucco, C; Valentini, Gabriele; IBBA MANNESCHI, L; MATUCCI CERINIC, M.
A PILOT STUDY ON LOW-DOSE INTRAVENOUS CICLOPHOSPHAMIDE IN SYSTEMIC SCLEROSIS: EFFICACY, SAFETY AND EFFECTS ON CELLULAR ACTIVATION MARKERS	1-gen-2002	D'Angelo, S.; Cuomo, Giovanna; Naclerio, C.; Abbadessa, Salvatore; Criscuolo, T.; Valentini, Gabriele
A regulatory variant in CCR6 is associated with susceptibility to antitopoisomerase-positive systemic sclerosis.	1-gen-2013	Koumakis, E; Bouaziz, M; Dieudé, P; Ruiz, B; Riemekasten, G; Airo, P; Müller Nurasyid, M; Cusi, D; Matucci Cerinic, M; Melchers, I; Salvi, E; Strauch, K; Peters, A; Cuomo, Giovanna; Hachulla, E; Diot, E; Hunzelmann, N; Caramaschi, P; Riccieri, V; Distler, Jh; Tarner, I; Avouac, J; Letenneur, L; Amouyel, P; Lambert, Jc; Chiocchia, G; Boileau, C; Allanore, Y.