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799 results on '"Distler, J"'

1. Apolipoprotein E controls Dectin-1-dependent development of monocyte-derived alveolar macrophages upon pulmonary β-glucan-induced inflammatory adaptation

Author

Theobald, H., Bejarano, D. A., Katzmarski, N., Haub, J., Schulte-Schrepping, J., Yu, J., Bassler, K., Ament, A. L., Osei-Sarpong, C., Piattini, F., Vornholz, L., T’Jonck, W., Györfi, A. H., Hayer, H., Yu, X., Sheoran, S., Al Jawazneh, A., Chakarov, S., Haendler, K., Brown, G. D., Williams, D. L., Bosurgi, L., Distler, J. H. W., Ginhoux, F., Ruland, J., Beyer, M. D., Greter, M., Bain, C. C., Vazquez-Armendariz, A. I., Kopf, M., Schultze, J. L., and Schlitzer, A.

Published
2024
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4. AB1144 THE ROLE OF SYSTEMIC S100A4 LEVELS IN SCLERODERMA AS A PREDICTOR OF TREATMENT RESPONSE AND EARLY PROGRESSION

Author

Štorkánová, H., primary, Andres Cerezo, L., additional, Oreska, S., additional, Jirankova, A., additional, Bobrova, A., additional, Bobrova, K., additional, Heřmánková, B., additional, Klingelhöfer, J., additional, Hussain, R. I., additional, Hallén, J., additional, Distler, J., additional, Pavelka, K., additional, Šenolt, L., additional, Vencovský, J., additional, Bečvář, R., additional, and Tomčík, M., additional

Published
2024
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5. OP0166 SKIN RETICULARIZATION IN SYSTEMIC SCLEROSIS - POTENTIAL MEDIATORS

Author

Chenguiti Fakhouri, S., primary, Zhu, H., additional, Ronicke, M., additional, Dees, C., additional, Konstantinidis, L., additional, Rius Rigau, A., additional, Matei, A. E., additional, LI, Y. N., additional, Eckstein, M., additional, Geppert, C., additional, Ludolph, I., additional, Kreuter, A., additional, Sticherling, M., additional, Berking, C., additional, Schett, G., additional, Horch, R., additional, Distler, J., additional, and Bergmann, C., additional

Published
2024
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10. OP0320 EFFICACY OF CD19-TARGETING CAR T CELL TREATMENT IN PATIENTS WITH DIFFUSE SYSTEMIC SCLEROSIS (dSSc) – AN OPEN LABEL, SINGLE-CENTER PILOT STUDY

Author

Auth, J., primary, Müller, F., additional, Taubmann, J., additional, Völkl, S., additional, Chenguiti Fakhouri, S., additional, Bucci, L., additional, Aigner, M., additional, Atzinger, A., additional, Bayerl, N., additional, Wirsching, A., additional, Dees, C., additional, Raimondo, M. G., additional, Tur, C., additional, Distler, J., additional, Mackensen, A., additional, Schett, G., additional, and Bergmann, C., additional

Published
2024
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15. AB1141 THERAPEUTIC POTENTIAL OF INHIBITION OF JAK1 AND 2 BY RUXOLITINIB, OF ROCK2 BY BELUMOSUDIL, AND OF BTK BY IBRUTINIB IN SYSTEMIC SCLEROSIS

Author

Hong, X., primary, Xiao, Y., additional, Györfi, A. H., additional, Xu, L., additional, Shen, L., additional, Neelagar, R., additional, Devakumar, V., additional, Filla, T., additional, Trinh-Minh, T., additional, Liang, M., additional, Rius Rigau, A., additional, Distler, J., additional, and Matei, A. E., additional

Published
2024
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19. Versorgungsrealität der stationären vasoaktiven Therapie mit Prostazyklinderivaten bei Patienten mit akralen Durchblutungsstörungen bei systemischer Sklerose in Deutschland

Author

Juche, A., Siegert, E., Mueller-Ladner, U., Riemekasten, G., Günther, C., Kötter, I., Henes, J., Blank, N., Voll, R. E., Ehrchen, J., Schmalzing, M., Susok, L., Schmeiser, T., Sunderkoetter, C., Distler, J., Worm, M., Kreuter, A., Horváth, O. N., Schön, M. P., Korsten, P., Zeidler, G., Pfeiffer, C., Krieg, T., Hunzelmann, N., and Moinzadeh, P.

Published
2020
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22. Cohort Enrichment Strategies for Progressive Interstitial Lung Disease in Systemic Sclerosis From European Scleroderma Trials and Research

Author

Hoffmann-Vold A. -M., Brunborg C., Airo P., Ananyeva L. P., Czirjak L., Guiducci S., Hachulla E., Li M., Mihai C., Riemekasten G., Sfikakis P. P., Valentini G., Kowal-Bielecka O., Allanore Y., Distler O., Vacca A., Giollo A., Balbir-Gurman A., Gheorghiu A. M., Marcoccia A., Herrick A., Radic M., Stamenkovic B., Anic B., Granel B., Ribi C., Selmi C. F., Carlos de la Puente M., de Souza Muller C., Denton C., Kayser C., Tanaseanu C. -M., Majewski D., Rimar D., Krasowska D., Veale D., Walker U., Kerzberg E., Rezus E., Zanatta E., Siegert E., De Langhe E., Oksel F., Ingegnoli F., Cantatore F. P., Szucs G., Cuomo G., Seskute G., Litinsky V., Castellvi I., Morovic-Vergles J., Sibilia J., Henes J., Solanki K., Perdan-Pirkmajer K., Herrmann K., Saketkoo L. A., Stamp L., Mouthon L., Salvador M. J., Pozzi M. R., Uprus M., Vanthuyne M., Engelhart M., Kohm M., Iudici M., Inanc M., Fathi N., Pamuk N., Garcia de la Pena Lefebv P., Carreira P. E., Bancel D. F., Moroncini L., Montecucco C., Ancuta C., Sunderkotter C., Muller-Ladner U., Rosato E., Kucharz E. J., Iannone F., Del Galdo F., Poormoghim H., Kotter I., Distler J., Cutolo M., Tikly M., Damjanov N., Hunzelmann N., Vlachoyiannopoulos P., Hasler P., Sarzi Puttini P., Wiland P., Becvar R., Yavuz S., Zdrojewski Z., Pellerito R., Foti R., Ionescu R. M., Adler S., Kahl S., Moiseev S., Stebbings S., Rednic S., Negrini S., Heitmann S., Ullman S., Agachi S., Martin T., Schmeiser T., Riccieri V., Smith V., Bernardino V., Ortiz-Santamaria V., Hsu V. M., Abdel Atty Mohamed W. A., Hoffmann-Vold, A. -M., Brunborg, C., Airo, P., Ananyeva, L. P., Czirjak, L., Guiducci, S., Hachulla, E., Li, M., Mihai, C., Riemekasten, G., Sfikakis, P. P., Valentini, G., Kowal-Bielecka, O., Allanore, Y., Distler, O., Vacca, A., Giollo, A., Balbir-Gurman, A., Gheorghiu, A. M., Marcoccia, A., Herrick, A., Radic, M., Stamenkovic, B., Anic, B., Granel, B., Ribi, C., Selmi, C. F., Carlos de la Puente, M., de Souza Muller, C., Denton, C., Kayser, C., Tanaseanu, C. -M., Majewski, D., Rimar, D., Krasowska, D., Veale, D., Walker, U., Kerzberg, E., Rezus, E., Zanatta, E., Siegert, E., De Langhe, E., Oksel, F., Ingegnoli, F., Cantatore, F. P., Szucs, G., Cuomo, G., Seskute, G., Litinsky, V., Castellvi, I., Morovic-Vergles, J., Sibilia, J., Henes, J., Solanki, K., Perdan-Pirkmajer, K., Herrmann, K., Saketkoo, L. A., Stamp, L., Mouthon, L., Salvador, M. J., Pozzi, M. R., Uprus, M., Vanthuyne, M., Engelhart, M., Kohm, M., Iudici, M., Inanc, M., Fathi, N., Pamuk, N., Garcia de la Pena Lefebv, P., Carreira, P. E., Bancel, D. F., Moroncini, L., Montecucco, C., Ancuta, C., Sunderkotter, C., Muller-Ladner, U., Rosato, E., Kucharz, E. J., Iannone, F., Del Galdo, F., Poormoghim, H., Kotter, I., Distler, J., Cutolo, M., Tikly, M., Damjanov, N., Hunzelmann, N., Vlachoyiannopoulos, P., Hasler, P., Sarzi Puttini, P., Wiland, P., Becvar, R., Yavuz, S., Zdrojewski, Z., Pellerito, R., Foti, R., Ionescu, R. M., Adler, S., Kahl, S., Moiseev, S., Stebbings, S., Rednic, S., Negrini, S., Heitmann, S., Ullman, S., Agachi, S., Martin, T., Schmeiser, T., Riccieri, V., Smith, V., Bernardino, V., Ortiz-Santamaria, V., Hsu, V. M., and Abdel Atty Mohamed, W. A.

Subjects
interstitial lung disease, Pulmonary and Respiratory Medicine, enrichment, systemic sclerosis, clinical trial, Cardiology and Cardiovascular Medicine, Critical Care and Intensive Care Medicine
Abstract

BACKGROUND: Enrichment strategies from clinical trials for progressive systemic sclerosis-associated interstitial lung disease (SSc-ILD) have not been tested in a real-life cohort.RESEARCH QUESTION: Do enrichment strategies for progressive ILD impact efficacy, repre-sentativeness, and feasibility in patients with SSc-ILD from the European Scleroderma Trials and Research (EUSTAR) database?STUDY DESIGN AND METHODS: We applied the inclusion criteria of major recent SSc-ILD trials (Study of the Efficacy and Safety of Tocilizumab in Participants With Systemic Sclerosis [focuSSced], Scleroderma Lung Study II [SLS II], and Safety and Efficacy of Nintedanib in Systemic Sclerosis [SENSCIS]) and assessed progressive ILD, which was defined as absolute change in FVC and as significant progression (FVC decline $10%). Data were compared with all patients and with patients who did not fulfill any inclusion criteria. RESULTS: In total, 2,258 patients with SSc-ILD were included: 31.2% of the patients met SENSCIS criteria; 5.8% of the patients met SLS II criteria; 1.6% of the patients met focuSSced criteria, and 67.7% (1,529) of the patients did not meet any criteria. In the first 12 + 3 months, the absolute FVC decline in all patients and in patients who fulfilled criteria from SENSCIS was -0.1%, in patients who fulfilled criteria from focuSSced was -3.7%, and in patients who fulfilled criteria from SLS II was 2.3%, with accompanying more progressors in focuSSced. The patient populations that fulfilled the different study inclusion criteria significantly differed in various clinical parameters. In the second 12-month period, SENSCIS-enriched patients had a further absolute FVC% decline as described for the total cohort. In contrast, patients who fulfilled the focuSSced and SLS II criteria showed numeric improvement of lung function. There were no significant associations of enrichment criteria and ILD progression.INTERPRETATION: The application of enrichment criteria from previous clinical trials showed enrichment for progression with variable success, which led to selected patient populations reducing feasibility of recruitment. These findings are important for future clinical trial design and interpretation of the results of published trials.CHEST 2023; 163(3):586-598

Published
2023
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24. Non-birational twisted derived equivalences in abelian GLSMs

Author

Caldararu, A., Distler, J., Hellerman, S., Pantev, T., and Sharpe, E.

Subjects
High Energy Physics - Theory
Abstract

In this paper we discuss some examples of abelian gauged linear sigma models realizing twisted derived equivalences between non-birational spaces, and realizing geometries in novel fashions. Examples of gauged linear sigma models with non-birational Kahler phases are a relatively new phenomenon. Most of our examples involve gauged linear sigma models for complete intersections of quadric hypersurfaces, though we also discuss some more general cases and their interpretation. We also propose a more general understanding of the relationship between Kahler phases of gauged linear sigma models, namely that they are related by (and realize) Kuznetsov's `homological projective duality.' Along the way, we shall see how `noncommutative spaces' (in Kontsevich's sense) are realized physically in gauged linear sigma models, providing examples of new types of conformal field theories. Throughout, the physical realization of stacks plays a key role in interpreting physical structures appearing in GLSMs, and we find that stacks are implicitly much more common in GLSMs than previously realized., Comment: 54 pages, LaTeX; v2: typo fixed

Published
2007
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25. Heterotic compactifications with principal bundles for general groups and general levels

Author

Distler, J. and Sharpe, E.

Subjects
High Energy Physics - Theory
Abstract

We examine to what extent heterotic string worldsheets can describe arbitrary E8xE8 gauge fields. The traditional construction of heterotic strings builds each E8 via a Spin(16)/Z2 subgroup, typically realized as a current algebra by left-moving fermions, and as a result, only E8 gauge fields reducible to Spin(16)/Z2 gauge fields are directly realizable in standard constructions. However, there exist perturbatively consistent E8 gauge fields which can not be reduced to Spin(16)/Z2, and so cannot be described within standard heterotic worldsheet constructions. A natural question to then ask is whether there exists any (0,2) SCFT that can describe such E8 gauge fields. To answer this question, we first show how each ten-dimensional E8 partition function can be built up using other subgroups than Spin(16)/Z2, then construct ``fibered WZW models'' which allow us to explicitly couple current algebras for general groups and general levels to heterotic strings. This technology gives us a very general approach to handling heterotic compactifications with arbitrary principal bundles. It also gives us a physical realization of some elliptic genera constructed recently by Ando and Liu., Comment: 48 pages, LaTeX; v2: references added; v3: typos fixed

Published
2007
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26. Resolving Singularities in (0,2) Models

Author

Distler, J., Greene, B., and Morrison, D.

Subjects
High Energy Physics - Theory
Abstract

In contrast to the familiar (2,2) case, the singularities which arise in the (0,2) setting can be associated with degeneration of the base Calabi-Yau manifold {\it and/or}\/ with degenerations of the gauge bundle. We study a variety of such singularities and give a procedure for resolving those which can be cured perturbatively. Among the novel features which emerge are models in which smoothing singularities in the base yields a gauge {\it sheaf}\/ as opposed to a gauge bundle as the structure to which left moving fermions couple. Supersymmetric $\sigma$-models with target data being an appropriate sheaf on a Calabi-Yau space therefore appear to be the natural arena for N=1 string models in four dimensions. We also indicate a variety of singularities which would require a nonperturbative treatment for their resolution and briefly discuss applications to heterotic models on K3., Comment: 33 pages, harvmac

Published
1996
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27. On the Instanton Contributions to the Masses and Couplings of $E_6$ Singlets

Author

Berglund, P., Candelas, P., de la Ossa, X., Derrick, E., Distler, J., and Hubsch, T.

Subjects
High Energy Physics - Theory, Mathematics - Algebraic Geometry
Abstract

We consider the gauge neutral matter in the low--energy effective action for string theory compactification on a \cym\ with $(2,2)$ world--sheet supersymmetry. At the classical level these states (the \sing's of $E_6$) correspond to the cohomology group $H^1(\M,{\rm End}\>T)$. We examine the first order contribution of instantons to the mass matrix of these particles. In principle, these corrections depend on the \K\ parameters $t_i$ through factors of the form $e^{2\p i t_i}$ and also depend on the complex structure parameters. For simplicity we consider in greatest detail the quintic threefold $\cp4[5]$. It follows on general grounds that the total mass is often, and perhaps always, zero. The contribution of individual instantons is however nonzero and the contribution of a given instanton may develop poles associated with instantons coalescing for certain values of the complex structure. This can happen when the underlying \cym\ is smooth. Hence these poles must cancel between the coalescing instantons in order that the superpotential be finite. We examine also the \Y\ couplings involving neutral matter \ysing\ and neutral and charged fields \ymix, which have been little investigated even though they are of phenomenological interest. We study the general conditions under which these couplings vanish classically. We also calculate the first--order world--sheet instanton correction to these couplings and argue that these also vanish., Comment: 40 pages, plain TeX with epsf, one uuencoded figure

Published
1995
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28. (0,2) Landau-Ginzburg Theory

Author

Distler, J. and Kachru, S.

Subjects
High Energy Physics - Theory
Abstract

A large class of (0,2) Calabi-Yau $\sigma$-models and Landau-Ginzburg orbifolds are shown to arise as different ``phases'' of supersymmetric gauge theories. We find a phenomenon in the Landau-Ginzburg phase which may enable one to understand which Calabi-Yau $\sigma$-models evade destabilization by worldsheet instantons. Examples of (0,2) Landau-Ginzburg vacua are analyzed in detail, and several novel features of (0,2) models are discussed. In particular, we find that (0,2) models can have different quantum symmetries from the (2,2) models built on the same Calabi-Yau manifold, and that a new kind of topology change can occur in (0,2) models of string theory., Comment: 34pp, harvmac Some minor revisions to bring this into line with the published version. The main changes are in the discussion of (0,2) models which are alleged to be deformations of (2,2) models

Published
1993
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30. Notes on N=2 $\sigma$-models$

Author

Distler, J.

Subjects
High Energy Physics - Theory
Abstract

These lectures, given at the 1992 Trieste Spring School, are devoted to some selected topics in N=2 \sm s on Calabi-Yau manifolds and the associated N=2 superconformal field theories. The first lecture is devoted to the ``special geometry" of the moduli space of $c=9$ N=2 superconformal field theories. An important role is played by the extended chiral algebra which appears in theories with integer $U(1)$ charges. The second lecture is devoted to the \sm\ approach. The main focus is an explication of a calculation of Aspinwall and Morrison., Comment: 23 pages, 2 figures (macros: harvmac.tex + epsf.tex)

Published
1992

31. A note on the 3D Ising model as a string theory

Author

Distler, J.

Subjects
High Energy Physics - Theory, Condensed Matter, High Energy Physics - Lattice
Abstract

It has long been argued that the continuum limit of the 3D Ising model is equivalent to a string theory. Unfortunately, in the usual starting point for this equivalence -- a certain lattice theory of surfaces -- it is not at all obvious how to take the continuum limit. In this note, I reformulate the lattice theory of surfaces in a fashion such that the continuum limit is straightforward. I go on to discuss how this new formulation may overcome some fundamental objections to the notion that the Ising model is equivalent to a string theory. In an appendix, I also discuss some aspects of fermion doubling, and the lattice fermion formulation of the 2D Ising model., Comment: 21 pages, 11 figures. This revised manuscript is identical to the final published version (Nuclear Physics B, to appear). Aside from correcting some scattered typos, the final version corrects and expands some of the comments in the original preprint on the unoriented NSR string

Published
1992
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32. World-Sheet Supersymmetry Without Contact Terms

Author

Distler, J. and Doyle, M.

Subjects
High Energy Physics - Theory
Abstract

Green and Seiberg showed that, in simple treatments of fermionic string theory, it is necessary to introduce contact interactions when vertex operators collide. Otherwise, certain superconformal Ward identities would be violated. In this note, we show how these contact terms arise naturally when proper account is taken of the superconformal geometry involved when punctures collide. More precisely, we show that there is no contact term at all! Rather, corrections arise to the ``na\"\i ve" formula when the boundary of moduli space is described correctly., Comment: 14pp., 2 figures (included)

Published
1992
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35. OP0238 IMMUNOSUPPRESSION WITH TARGETED DMARDS REDUCES MORBIDITY AND MORTALITY IN PRE-CAPILLARY PULMONARY HYPERTENSION ASSOCIATED WITH SYSTEMIC SCLEROSIS: A EUSTAR ANALYSIS

Author

Bruni, C., primary, Tofani, L., additional, Fretheim, H., additional, Weber, Y., additional, Hachulla, E., additional, Carreira, P., additional, Giuggioli, D., additional, Airò, P., additional, Siegert, E., additional, Müller-Ladner, U., additional, Matucci-Cerinic, M., additional, Riemekasten, G., additional, Simeon Aznar, C. P., additional, De Vries-Bouwstra, J., additional, Saketkoo, L. A., additional, Distler, J., additional, Balbir-Gurman, A., additional, Castellví, I., additional, Zanatta, E., additional, Smith, V., additional, Denton, C. P., additional, Maurer, B., additional, Giollo, A., additional, Iannone, F., additional, Dagna, L., additional, Truchetet, M. E., additional, Kuwana, M., additional, Allanore, Y., additional, Tanaka, Y., additional, Martin, M., additional, Rosato, E., additional, Gheorghiu, A. M., additional, Del Galdo, F., additional, Solanki, K., additional, Vacca, A., additional, Resende, C., additional, Vieira, S., additional, Czirják, L., additional, Baresic, M., additional, Cantatore, F. P., additional, Riccieri, V., additional, Andréasson, K., additional, Chung, L., additional, Souza Muller, C., additional, Opris-Belinski, D., additional, Rednic, S., additional, Sfikakis, P., additional, Levy, Y., additional, Hsu, V., additional, Heitmann, S., additional, Henes, J., additional, Moroncini, G., additional, Iudici, M., additional, De Langhe, E., additional, Herrick, A., additional, Montecucco, C., additional, Hoffmann-Vold, A. M., additional, and Distler, O., additional

Published
2023
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36. OP0106 SYNOVIAL IMPRINTING OF SKIN-DERIVED IMMUNE CELLS INITIATES SPREADING OF INFLAMMATION FROM SKIN TO JOINT IN PSORIATIC ARTHRITIS

Author

Raimondo, M. G., primary, Rauber, S., additional, Mohammadian, H., additional, Vogg, M., additional, Xu, C., additional, Rius Rigau, A., additional, Luber, M., additional, Labinsky, H., additional, Soare, A., additional, Distler, J., additional, Fearon, U., additional, Veale, D., additional, Sticherling, M., additional, Cañete, J. D. D., additional, Schett, G., additional, and Ramming, A., additional

Published
2023
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37. POS0377 ACCURACY OF AN AI-BASED SYMPTOM CHECKER AND AN ONLINE SELF-REFERRAL TOOL IN RHEUMATOLOGY: RESULTS FROM A MULTICENTER RANDOMIZED CONTROLLED TRIAL

Author

Knitza, J., primary, Tascilar, K., additional, Fuchs, F., additional, Mohn, J., additional, Simon, D., additional, Kleyer, A., additional, Bergmann, C., additional, Labinsky, H., additional, Morf, H., additional, Araujo, E., additional, Bohr, D., additional, Muehlensiepen, F., additional, Englbrecht, M., additional, Vorbrüggen, W., additional, Von der Decken, C. B., additional, Kleinert, S., additional, Ramming, A., additional, Distler, J., additional, Bartz-Bazzanella, P., additional, Vuillerme, N., additional, Schett, G., additional, Welcker, M., additional, and Hueber, A., additional

Published
2023
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38. POS1255 CLINICAL SIGNIFICANCE OF THE ANTI-NUCLEOLAR ORGANIZER REGION 90 ANTIBODIES (NOR90) IN SYSTEMIC SCLEROSIS: A EUROPEAN SCLERODERMA TRIALS AND RESEARCH (EUSTAR) ANALYSIS

Author

Dima, A., primary, Garaiman, A., additional, Vonk, M., additional, Kersten, B., additional, Bečvář, R., additional, Tomcik, M., additional, Hoffmann-Vold, A. M., additional, Castellví, I., additional, Tandaipan, J. L., additional, Brzosko, M., additional, Milchert, M., additional, Krasowska, D., additional, Michalska-Jakubus, M., additional, Airò, P., additional, Matucci-Cerinic, M., additional, Bruni, C., additional, Iudici, M., additional, Distler, J., additional, Gheorghiu, A. M., additional, Poormoghim, H., additional, Motta, F., additional, De Santis, M., additional, Parvu, M., additional, Distler, O., additional, and Mihai, C., additional

Published
2023
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39. Clinical spectrum time course in non-Asian patients positive for anti-MDA5 antibodies

Author

Cavagna, L, Meloni, F, Meyer, A, Sambataro, G, Belliato, M, De Langhe, E, Cavazzana, I, Pipitone, N, Triantafyllias, K, Mosca, M, Barsotti, S, Zampogna, G, Biglia, A, Emmi, G, De Visser, M, Van Der Kooi, A, Parronchi, P, Hirschi, S, da Silva, J, Scire, C, Furini, F, Giannini, M, Martinez Gonzalez, O, Damian, L, Piette, Y, Smith, V, Mera-Valera, A, Bachiller-Corral, J, Cabezas Rodriguez, I, Brandy-Garcia, A, Maurier, F, Perrin, J, Gonzalez-Moreno, J, Drott, U, Delbruck, C, Schwarting, A, Arrigoni, E, Sebastiani, G, Iuliano, A, Nannini, C, Quartuccio, L, Rodriguez Cambron, A, Blazquez Canamero, M, Villa Blanco, I, Cagnotto, G, Pesci, A, Luppi, F, Dei, G, Romero Bueno, F, Franceschini, F, Chiapparoli, I, Zanframundo, G, Lettieri, S, De Stefano, L, Cutolo, M, Mathieu, A, Piga, M, Prieto-Gonzalez, S, Moraes-Fontes, M, Fonseca, J, Jovani, V, Riccieri, V, Santaniello, A, Montfort, S, Bilocca, D, Erre, G, Bartoloni, E, Gerli, R, Monti, M, Lorenz, H, Sambataro, D, Bellando Randone, S, Schneider, U, Valenzuela, C, Lopez-Mejias, R, Cifrian, J, Mejia, M, Gonzalez Perez, M, Wendel, S, Fornaro, M, De Luca, G, Orsolini, G, Rossini, M, Dieude, P, Knitza, J, Castaneda, S, Voll, R, Rojas-Serrano, J, Valentini, A, Vancheri, C, Matucci-Cerinic, M, Feist, E, Codullo, V, Iannone, F, Distler, J, Montecucco, C, Gonzalez-Gay, M, Cavagna L., Meloni F., Meyer A., Sambataro G., Belliato M., De Langhe E., Cavazzana I., Pipitone N., Triantafyllias K., Mosca M., Barsotti S., Zampogna G., Biglia A., Emmi G., De Visser M., Van Der Kooi A., Parronchi P., Hirschi S., da Silva J. A. P., Scire C. A., Furini F., Giannini M., Martinez Gonzalez O., Damian L., Piette Y., Smith V., Mera-Valera A., Bachiller-Corral J., Cabezas Rodriguez I., Brandy-Garcia A. M., Maurier F., Perrin J., Gonzalez-Moreno J., Drott U., Delbruck C., Schwarting A., Arrigoni E., Sebastiani G. D., Iuliano A., Nannini C., Quartuccio L., Rodriguez Cambron A. B., Blazquez Canamero M. A., Villa Blanco I., Cagnotto G., Pesci A., Luppi F., Dei G., Romero Bueno F. I., Franceschini F., Chiapparoli I., Zanframundo G., Lettieri S., De Stefano L., Cutolo M., Mathieu A., Piga M., Prieto-Gonzalez S., Moraes-Fontes M. F., Fonseca J. E., Jovani V., Riccieri V., Santaniello A., Montfort S., Bilocca D., Erre G. L., Bartoloni E., Gerli R., Monti M. C., Lorenz H. M., Sambataro D., Bellando Randone S., Schneider U., Valenzuela C., Lopez-Mejias R., Cifrian J., Mejia M., Gonzalez Perez M. -I., Wendel S., Fornaro M., De Luca G., Orsolini G., Rossini M., Dieude P., Knitza J., Castaneda S., Voll R. E., Rojas-Serrano J., Valentini A., Vancheri C., Matucci-Cerinic M., Feist E., Codullo V., Iannone F., Distler J. H., Montecucco C., Gonzalez-Gay M. A., Cavagna, L, Meloni, F, Meyer, A, Sambataro, G, Belliato, M, De Langhe, E, Cavazzana, I, Pipitone, N, Triantafyllias, K, Mosca, M, Barsotti, S, Zampogna, G, Biglia, A, Emmi, G, De Visser, M, Van Der Kooi, A, Parronchi, P, Hirschi, S, da Silva, J, Scire, C, Furini, F, Giannini, M, Martinez Gonzalez, O, Damian, L, Piette, Y, Smith, V, Mera-Valera, A, Bachiller-Corral, J, Cabezas Rodriguez, I, Brandy-Garcia, A, Maurier, F, Perrin, J, Gonzalez-Moreno, J, Drott, U, Delbruck, C, Schwarting, A, Arrigoni, E, Sebastiani, G, Iuliano, A, Nannini, C, Quartuccio, L, Rodriguez Cambron, A, Blazquez Canamero, M, Villa Blanco, I, Cagnotto, G, Pesci, A, Luppi, F, Dei, G, Romero Bueno, F, Franceschini, F, Chiapparoli, I, Zanframundo, G, Lettieri, S, De Stefano, L, Cutolo, M, Mathieu, A, Piga, M, Prieto-Gonzalez, S, Moraes-Fontes, M, Fonseca, J, Jovani, V, Riccieri, V, Santaniello, A, Montfort, S, Bilocca, D, Erre, G, Bartoloni, E, Gerli, R, Monti, M, Lorenz, H, Sambataro, D, Bellando Randone, S, Schneider, U, Valenzuela, C, Lopez-Mejias, R, Cifrian, J, Mejia, M, Gonzalez Perez, M, Wendel, S, Fornaro, M, De Luca, G, Orsolini, G, Rossini, M, Dieude, P, Knitza, J, Castaneda, S, Voll, R, Rojas-Serrano, J, Valentini, A, Vancheri, C, Matucci-Cerinic, M, Feist, E, Codullo, V, Iannone, F, Distler, J, Montecucco, C, Gonzalez-Gay, M, Cavagna L., Meloni F., Meyer A., Sambataro G., Belliato M., De Langhe E., Cavazzana I., Pipitone N., Triantafyllias K., Mosca M., Barsotti S., Zampogna G., Biglia A., Emmi G., De Visser M., Van Der Kooi A., Parronchi P., Hirschi S., da Silva J. A. P., Scire C. A., Furini F., Giannini M., Martinez Gonzalez O., Damian L., Piette Y., Smith V., Mera-Valera A., Bachiller-Corral J., Cabezas Rodriguez I., Brandy-Garcia A. M., Maurier F., Perrin J., Gonzalez-Moreno J., Drott U., Delbruck C., Schwarting A., Arrigoni E., Sebastiani G. D., Iuliano A., Nannini C., Quartuccio L., Rodriguez Cambron A. B., Blazquez Canamero M. A., Villa Blanco I., Cagnotto G., Pesci A., Luppi F., Dei G., Romero Bueno F. I., Franceschini F., Chiapparoli I., Zanframundo G., Lettieri S., De Stefano L., Cutolo M., Mathieu A., Piga M., Prieto-Gonzalez S., Moraes-Fontes M. F., Fonseca J. E., Jovani V., Riccieri V., Santaniello A., Montfort S., Bilocca D., Erre G. L., Bartoloni E., Gerli R., Monti M. C., Lorenz H. M., Sambataro D., Bellando Randone S., Schneider U., Valenzuela C., Lopez-Mejias R., Cifrian J., Mejia M., Gonzalez Perez M. -I., Wendel S., Fornaro M., De Luca G., Orsolini G., Rossini M., Dieude P., Knitza J., Castaneda S., Voll R. E., Rojas-Serrano J., Valentini A., Vancheri C., Matucci-Cerinic M., Feist E., Codullo V., Iannone F., Distler J. H., Montecucco C., and Gonzalez-Gay M. A.

Abstract

Objective To define the clinical spectrum time-course and prognosis of non-Asian patients positive for anti-MDA5 antibodies. Methods We conducted a multicentre, international, retrospective cohort study. Results 149 anti-MDA5 positive patients (median onset age 53 years, median disease duration 18 months), mainly females (100, 67%), were included. Dermatomyositis (64, 43%) and amyopathic dermatomyositis (47, 31%), were the main diagnosis; 15 patients (10%) were classified as interstitial pneumonia with autoimmune features (IPAF) and 7 (5%) as rheumatoid arthritis. The main clinical findings observed were myositis (84, 56%), interstitial lung disease (ILD) (108, 78%), skin lesions (111, 74%), and arthritis (76, 51%). The onset of these manifestations was not concomitant in 74 cases (50%). Of note, 32 (21.5%) patients were admitted to the intensive care unit for rapidly progressive-ILD, which occurred in median 2 months from lung involvement detection, in the majority of cases (28, 19%) despite previous immunosuppressive treatment. One-third of patients (47, 32% each) was ANA and anti-ENA antibodies negative and a similar percentage was anti-Ro52 kDa antibodies positive. Non-specific interstitial pneumonia (65, 60%), organising pneumonia (23, 21%), and usual interstitial pneumonia-like pattern (14, 13%) were the main ILD patterns observed. Twenty-six patients died (17%), 19 (13%) had a rapidly progressive-ILD. Conclusion The clinical spectrum of the anti-MDA5 antibodies-related disease is heterogeneous. Rapidly-progressive ILD deeply impacts the prognosis also in non-Asian patients, occurring early during the disease course. Anti-MDA5 antibody positivity should be considered even when baseline autoimmune screening is negative, anti-Ro52 kDa antibodies are positive, and radiology findings show a NSIP pattern.

Published
2022

42. Disturbed spatial activation of WNT/beta-catenin signaling in Systemic Sclerosis

Author

Bergmann, C, Chenguiti Fakhouri, S, Konstantinidis, L, Zhu, H, Rigaz, A, Györfi, AH, Eckstein, M, Geppert, CI, Dees, C, Kreuter, A, Sticherling, M, Schett, G, Distler, J, Bergmann, C, Chenguiti Fakhouri, S, Konstantinidis, L, Zhu, H, Rigaz, A, Györfi, AH, Eckstein, M, Geppert, CI, Dees, C, Kreuter, A, Sticherling, M, Schett, G, and Distler, J

Published
2023

47. Late Skin Fibrosis in Systemic Sclerosis: A Study from the EUSTAR Cohort

Author

Michael Hughes 1 2, Suiyuan Huang 3 4, Juan Jose Alegre-Sancho 5, Patricia E Carreira 6, Merete Engelhart 7, Eric Hachulla 8, Joerg Henes 9, Eduardo Kerzberg 10, Maria Rosa Pozzi 11, Gabriela Riemekasten 12, Vanessa Smith 13, Gabriella Szücs 14, Marie Vanthuyne 15, Elisabetta Zanatta 16, Oliver Distler 17, Armando G Gabrielli 18, Anna-Maria Hoffmann-Vold 19, Virginia D Steen 20, Dinesh Khanna 3 21, EUSTAR Airò P, Allanore A, Ananieva Lp, Anic B, Balbir-Gurman A, Becvar R, Benvenuti F, Cantatore F P, Chung L S, Cuomo G, Cutolo M, Czirják L, Damjanov N, de Vries-Bouwstra J, Del Galdo F, Distler J, Eyerich K, Farge D, Foti R, Gheorghiu A M, Giollo A, Heitmann S, Herrick A, Hesselstrand R, Hsu I M, Hunzelmann N, Iannone F, Iudici M, Ionescuc M R, Ingegnoli F, Jose J, Joven B E, Kerzberg E, Kucharz E J, Kuwana M, Langhe E D, Launay D, Lefebvre P, Litinsky I, García de la Peña Lefebvre P, González-Martín J J, Li M, Loyo E, Martin T, Matucci-Cerinic M, Maurer B, Moroncini G, Mouthon L, Müller Cs, Müller-Ladner U, Novak S, Pastor P, Pecher A-C, Pellerito R, Pozzi M R, Oksel F, Rednic S, Rezus E, Riccieri V, Rosato E, Saketkoo L A, Salvador M J, Schmeiser T, Selmi C F, Sibilia J, Siegert E, Solanki K, Sommerlatte S, Spertini F, Stamenkovic B, Stamp L, Tanaseanu C-M, Tikly M, Tineo C, Ullman S, Üprus M, Vanthuyne M, Veale D, Walker U, Wiland P, Yargucu F, Yavuz S, University of Zurich, Michael Hughes, 1 2, Suiyuan Huang, 3 4, Juan Jose Alegre-Sancho, 5, Patricia, E Carreira 6, Merete Engelhart, 7, Eric Hachulla, 8, Joerg Henes, 9, Eduardo Kerzberg, 10, Maria Rosa Pozzi, 11, Gabriela Riemekasten, 12, Vanessa Smith, 13, Gabriella Szücs, 14, Marie Vanthuyne, 15, Elisabetta Zanatta, 16, Oliver Distler, 17, Armando, G Gabrielli 18, Anna-Maria Hoffmann-Vold, 19, Virginia, D Steen 20, Dinesh Khanna, 3 21, EUSTAR Airò, P, Allanore, A, Ananieva, Lp, Anic, B, Balbir-Gurman, A, Becvar, R, Benvenuti, F, Cantatore, F P, Chung, L S, Cuomo, G, Cutolo, M, Czirják, L, Damjanov, N, de Vries-Bouwstra, J, Del Galdo, F, Distler, J, Eyerich, K, Farge, D, Foti, R, Gheorghiu, A M, Giollo, A, Heitmann, S, Herrick, A, Hesselstrand, R, Hsu, I M, Hunzelmann, N, Iannone, F, Iudici, M, Ionescuc, M R, Ingegnoli, F, Jose, J, Joven, B E, Kerzberg, E, Kucharz, E J, Kuwana, M, Langhe, E D, Launay, D, Lefebvre, P, Litinsky, I, García de la Peña Lefebvre, P, González-Martín, J J, Li, M, Loyo, E, Martin, T, Matucci-Cerinic, M, Maurer, B, Moroncini, G, Mouthon, L, Müller, C, Müller-Ladner, U, Novak, S, Pastor, P, Pecher, A-C, Pellerito, R, Pozzi, M R, Oksel, F, Rednic, S, Rezus, E, Riccieri, V, Rosato, E, Saketkoo, L A, Salvador, M J, Schmeiser, T, Selmi, C F, Sibilia, J, Siegert, E, Solanki, K, Sommerlatte, S, Spertini, F, Stamenkovic, B, Stamp, L, Tanaseanu, C-M, Tikly, M, Tineo, C, Ullman, S, Üprus, M, Vanthuyne, M, Veale, D, Walker, U, Wiland, P, Yargucu, F, and Yavuz, S

Subjects
Rheumatology, Cohort enrichment, 10051 Rheumatology Clinic and Institute of Physical Medicine, Late disease, Systemic sclerosis, Pharmacology (medical), 610 Medicine & health, Fibrosis, Clinical trial design, Scleroderma, Skin
Abstract

Objectives The early trajectory of skin fibrosis provides insights into the disease course of systemic sclerosis (SSc) including mortality; however, little is known about late skin fibrosis. The aims of our study were to ascertain the prevalence and characteristics of late skin fibrosis in SSc. Methods We developed and tested three conceptual scenarios of late (>5 years after first non-RP feature) skin fibrosis including new worsening of skin disease, and failure to improve after worsening within 5-year window. We defined skin worsening as change in modified Rodnan skin score (mRSS) ≥5 units or ≥25%. Using strict inclusion criteria including complete mRSS, we identified 1,043 (out of 19 115) patients within the EUSTAR database for our analysis. We further restricted analysis within 887 (out of 1043) patients who had lcSSc or dcSSc at baseline. Results One-fifth of patients among the whole cohort (n = 208/1043, 19.9%) experienced mRSS worsening, including in patients with lcSSc or dcSSc at baseline (n = 193/887, 21.8%). This was largely due to new skin worsening after the 5-year window or failure to improve with worsening within the 5-year window. Patients with lower baseline mRSS and lcSSc were more likely to develop late skin fibrosis. Anti-Scl-70 was associated with progression from baseline lcSSc to dcSSc, and anticentromere was protective. Conclusions Late skin fibrosis is not uncommon in SSc. We have identified different patterns relevant to clinical practice and trial design. Late skin fibrosis is a neglected manifestation of SSc and warrants further investigation including to determine clinical outcomes and optimal therapeutic strategy.

Published
2022

48. Persistent inflammation in systemic sclerosis is strongly associated with mortality: a EUSTAR database analysis

Author

Guler, S A, primary, Sarbu, A, additional, Stadler, O, additional, Allanore, Y, additional, Bernardino, V, additional, Distler, J H, additional, Gabrielli, A, additional, Hoffmann-Vold, A, additional, Matucci-Cerinic, M, additional, Müller-Ladner, U, additional, Ortiz-Santamaria, V, additional, Rednic, S, additional, Riccieri, V, additional, Smith, V, additional, Ullman, S, additional, Walker, U, additional, Geiser, T, additional, Distler, O, additional, Maurer, B, additional, and Kollert, F, additional

Published
2022
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50. POS0140 PREDICTING OUTCOMES IN SYSTEMIC SCLEROSIS: STRATIFICATION BY AUTO-ANTIBODIES OUTPERFORMS CUTANEOUS SUBSETTING IN THE EUSTAR COHORT

Author

Elhai, M., primary, Boubaya, M., additional, Sritharan, N., additional, Balbir-Gurman, A., additional, Siegert, E., additional, Hachulla, E., additional, De Vries-Bouwstra, J., additional, Riemekasten, G., additional, Distler, J. H. W., additional, Veale, D., additional, Rosato, E., additional, Del Galdo, F., additional, Mendoza, F. A., additional, Furst, D., additional, De la Puente Bujidos, C., additional, Hoffmann-Vold, A. M., additional, Gabrielli, A., additional, Distler, O., additional, Bloch-Queyrat, C., additional, and Allanore, Y., additional

Published
2022
Full Text
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