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1. Geographical heterogeneity of clinical and serological phenotypes of systemic sclerosis observed at tertiary referral centres. The experience of the Italian SIR-SPRING registry and review of the world literature

Author

Ferri, Clodoveo, De Angelis, Rossella, Giuggioli, Dilia, Bajocchi, Gianluigi, Dagna, Lorenzo, Zanframundo, Giovanni, Foti, Rosario, Cacciapaglia, Fabio, Cuomo, Giovanna, Ariani, Alarico, Rosato, Edoardo, Guiducci, Serena, Girelli, Francesco, Riccieri, Valeria, Zanatta, Elisabetta, Bosello, Silvia Laura, Cavazzana, Ilaria, Ingegnoli, Francesca, De Santis, Maria, Murdaca, Giuseppe, Abignano, Giuseppina, Romeo, Nicoletta, Della Rossa, Alessandra, Caminiti, Maurizio, Iuliano, Annamaria, Ciano, Giovanni, Beretta, Lorenzo, Bagnato, Gianluca, Lubrano, Ennio, De Andres, Ilenia, Giollo, Alessandro, Saracco, Marta, Agnes, Cecilia, Lumetti, Federica, Spinella, Amelia, Magnani, Luca, Campochiaro, Corrado, De Luca, Giacomo, Codullo, Veronica, Visalli, Elisa, Masini, Francesco, Gigante, Antonietta, Bellando-Randone, Silvia, Pellegrino, Greta, Pigatto, Erika, Lazzaroni, Maria Grazia, Franceschini, Franco, Generali, Elena, Mennillo, Gianna, Barsotti, Simone, Mariano, Giuseppa Pagano, Calabrese, Francesca, Furini, Federica, Vultaggio, Licia, Parisi, Simone, Peroni, Clara Lisa, Rozza, Davide, Zanetti, Anna, Carrara, Greta, Landolfi, Giampiero, Scirè, Carlo Alberto, Bianchi, Gerolamo, Fusaro, Enrico, Sebastiani, Gian Domenico, Govoni, Marcello, D'Angelo, Salvatore, Cozzi, Franco, Doria, Andrea, Iannone, Florenzo, Salvarani, Carlo, Matucci-Cerinic, Marco, Bosello, Silvia (ORCID:0000-0002-4837-447X), Ferri, Clodoveo, De Angelis, Rossella, Giuggioli, Dilia, Bajocchi, Gianluigi, Dagna, Lorenzo, Zanframundo, Giovanni, Foti, Rosario, Cacciapaglia, Fabio, Cuomo, Giovanna, Ariani, Alarico, Rosato, Edoardo, Guiducci, Serena, Girelli, Francesco, Riccieri, Valeria, Zanatta, Elisabetta, Bosello, Silvia Laura, Cavazzana, Ilaria, Ingegnoli, Francesca, De Santis, Maria, Murdaca, Giuseppe, Abignano, Giuseppina, Romeo, Nicoletta, Della Rossa, Alessandra, Caminiti, Maurizio, Iuliano, Annamaria, Ciano, Giovanni, Beretta, Lorenzo, Bagnato, Gianluca, Lubrano, Ennio, De Andres, Ilenia, Giollo, Alessandro, Saracco, Marta, Agnes, Cecilia, Lumetti, Federica, Spinella, Amelia, Magnani, Luca, Campochiaro, Corrado, De Luca, Giacomo, Codullo, Veronica, Visalli, Elisa, Masini, Francesco, Gigante, Antonietta, Bellando-Randone, Silvia, Pellegrino, Greta, Pigatto, Erika, Lazzaroni, Maria Grazia, Franceschini, Franco, Generali, Elena, Mennillo, Gianna, Barsotti, Simone, Mariano, Giuseppa Pagano, Calabrese, Francesca, Furini, Federica, Vultaggio, Licia, Parisi, Simone, Peroni, Clara Lisa, Rozza, Davide, Zanetti, Anna, Carrara, Greta, Landolfi, Giampiero, Scirè, Carlo Alberto, Bianchi, Gerolamo, Fusaro, Enrico, Sebastiani, Gian Domenico, Govoni, Marcello, D'Angelo, Salvatore, Cozzi, Franco, Doria, Andrea, Iannone, Florenzo, Salvarani, Carlo, Matucci-Cerinic, Marco, and Bosello, Silvia (ORCID:0000-0002-4837-447X)

Abstract

Introduction: Systemic sclerosis (SSc) is characterized by a complex etiopathogenesis encompassing both host genetic and environmental -infectious/toxic- factors responsible for altered fibrogenesis and diffuse microangiopathy. A wide spectrum of clinical phenotypes may be observed in patients' populations from different geographical areas. We investigated the prevalence of specific clinical and serological phenotypes in patients with definite SSc enrolled at tertiary referral centres in different Italian geographical macro-areas. The observed findings were compared with those reported in the world literature.Materials and methods: The clinical features of 1538 patients (161 M, 10.5%; mean age 59.8 +/- 26.9 yrs.; mean disease duration 8.9 +/- 7.7 yrs) with definite SSc recruited in 38 tertiary referral centres of the SPRING (Systemic sclerosis Progression INvestiGation Group) registry promoted by Italian Society of Rheumatology (SIR) were obtained and clustered according to Italian geographical macroareas.Results: Patients living in Southern Italy were characterized by more severe clinical and/or serological SSc phenotypes compared to those in Northern and Central Italy; namely, they show increased percentages of diffuse cutaneous SSc, digital ulcers, sicca syndrome, muscle involvement, arthritis, cardiopulmonary symptoms, interstitial lung involvement at HRCT, as well increased prevalence of serum anti-Scl70 autoantibodies. In the same SSc population immunusppressive drugs were frequently employed. The review of the literature underlined the geographical heterogeneity of SSc phenotypes, even if the observed findings are scarcely comparable due to the variability of methodological approaches.Conclusion: The phenotypical differences among SSc patients' subgroups from Italian macro-areas might be correlated to genetic/environmental co-factors, and possibly to a not equally distributed national network of information and healthcare facilities.

Published
2022

2. Contributors

Author

Abdellatif, Engy, primary, Abeles, Aryeh M., additional, Abelson, Abby G., additional, Abhishek, Abhishek, additional, Abramson, Steven B., additional, Adachi, Jonathan D., additional, Adams, Michael A., additional, Aigner, Thomas, additional, Akira, Shizuo, additional, Aletaha, Daniel, additional, Aliprantis, Antonios O., additional, Almeida de Jesus, Adriana, additional, Altman, Roy D., additional, Amigo, Mary-Carmen, additional, Aringer, Martin, additional, Ascherman, Dana P., additional, Assassi, Shervin, additional, Atamas, Sergei P., additional, Azevedo, Pedro Ming, additional, Baer, Alan N., additional, Baeten, Dominique, additional, Baines, Colin, additional, Baker, Nancy A., additional, Balogh, Emese, additional, Balsa, Alejandro, additional, Baraliakos, Xenofon, additional, Bardin, Thomas, additional, Barnsley, Les, additional, Bathon, Joan M., additional, Bauch, Angela, additional, Belch, Jill J.F., additional, Bellamy, Nicholas, additional, Bellido, Teresita, additional, Benjamin, Michael, additional, Beresford, Michael W., additional, Berman, Brian, additional, Bermas, Bonnie Lee, additional, Bertsias, George, additional, Bilezikian, John P., additional, Bilginer, Yelda, additional, Birnbaum, Julius, additional, Bishop, Felicity L., additional, Bleasel, Jane F., additional, Böhm, Markus, additional, Bolster, Marcy B., additional, Bombardieri, Stefano, additional, Bonelli, Michael, additional, Bonnick, Sydney L., additional, Boumpas, Dimitrios T., additional, Bozec, Aline, additional, Brasington, Richard D., additional, Braun, Juergen, additional, Brown, Matthew A., additional, Bruce, Ian N., additional, Bugbee, William D., additional, Bukhari, Marwan A.S., additional, Burgos-Vargas, Rubén, additional, Burmester, Gerd-Rüdiger, additional, Burns, Jane C., additional, Burr, David B., additional, Buttgereit, Frank, additional, Bykerk, Vivian P., additional, Calabrese, Leonard H., additional, Callen, Jeffrey P., additional, Cavallo, Sabrina, additional, Cawston, Tim E., additional, Chandran, Vinod, additional, Chard, Michael Denis, additional, Chaudhary, Prateek, additional, Chen, Lan X., additional, Choi, Hyon K., additional, Choy, Ernest H., additional, Christopher-Stine, Lisa, additional, Chu, Alvina D., additional, Clauw, Daniel J., additional, Clements, Philip J., additional, Clowse, Megan E.B., additional, Coghlan, J. Gerry, additional, Conaghan, Philip G., additional, Cooper, Cyrus, additional, Costenbader, Karen H., additional, Creamer, Paul, additional, Crispín, José C., additional, Cronstein, Bruce N., additional, Cross, Raymond, additional, Cusano, Natalie E., additional, Cutolo, Maurizio, additional, D'Adamo, Chris, additional, D'Agati, Vivette, additional, D'Cruz, David P., additional, Dagfinrud, Hanne, additional, Daikh, David I., additional, Dalbeth, Nicola, additional, Dalton, Seamus E., additional, Dass, Shouvik, additional, Davis, Aileen M., additional, De Ceulaer, Karel, additional, Deal, Chad L., additional, Deane, Kevin D., additional, Della Rossa, Alessandra, additional, Dellaripa, Paul F., additional, Dennison, Elaine, additional, Denton, Christopher P., additional, Dieppe, Paul, additional, Doherty, Michael, additional, Dolan, Patricia, additional, Donn, Rachelle, additional, Dvorkina, Olga, additional, Dyer, George S.M., additional, Eastell, Richard, additional, Edwards, N. Lawrence, additional, Emery, Paul, additional, Erturan, Gurhan, additional, Espinoza, Luis R., additional, Eyre, Steve, additional, Fanouriakis, Antonios C., additional, Farber, Joshua, additional, Farrokhi, Shawn, additional, Fasth, Anders, additional, Feist, Eugen, additional, Feldman, Debbie, additional, Felson, David T., additional, Fisk, John D., additional, Fitzgerald, G. Kelley, additional, Flores, Raymond H., additional, Fox, David A., additional, Francomano, Clair A., additional, Frangos, Jennifer, additional, Freemont, Anthony J., additional, Fricka, Kevin B., additional, Furst, Daniel E., additional, Gabay, Cem, additional, Gadina, Massimo, additional, Gaston, J.S. Hill, additional, Gay, Steffen, additional, Gensler, Lianne S., additional, Geraldino-Pardilla, Laura, additional, Gerlag, Danielle M., additional, Ginzler, Ellen M., additional, Gizinski, Alison M., additional, Gladman, Dafna D., additional, Gold, Garry E., additional, Goldbach-Mansky, Raphaela, additional, Goldingay, Sarah, additional, Gordon, Sharon M., additional, Gorodkin, Rachel, additional, Goronzy, Jörg J., additional, Görtz, Simon, additional, Grahame, Rodney, additional, Grainger, Andrew J., additional, Gravallese, Ellen M., additional, Greenberg, Jeffrey D., additional, Gujar, Bansari, additional, Hagan, Matilda, additional, Hagley, Karlene, additional, Hakim, Alan J., additional, Hall, John C., additional, Hamuryudan, Vedat, additional, Hanly, John G., additional, Hanson, Eric P., additional, Haraoui, Boulos, additional, Harley, John B., additional, Hashkes, Philip J., additional, Hawker, Gillian A., additional, Hawkins, Philip N., additional, Heiberg, Turid, additional, Heinegård, Dick, additional, Helfgott, Simon M., additional, Ho, Pauline Y.P., additional, Hochberg, Marc C., additional, Hochman, Jacqueline, additional, Hodgkiss-Harlow, Chelsea J., additional, Hoffman, Robert W., additional, Hoffmann, Markus, additional, Holers, V. Michael, additional, Holick, Michael F., additional, Holroyd, Christopher, additional, Hübscher, Osvaldo, additional, Hunter, David J., additional, Husni, M. Elaine, additional, Inman, Robert D., additional, Isaac, Zacharia, additional, Iversen, Maura D., additional, Jabs, Douglas A., additional, Jackson, William, additional, Jaimungal, Sarada, additional, James, Judith A., additional, Javier, Rose-Marie, additional, Johnsen, Alyssa K., additional, Jordan, Joanne M., additional, Kaisho, Tsuneyasu, additional, Kallenberg, Cees G.M., additional, Kane, David, additional, Kapoor, Mohit, additional, Karlson, Elizabeth W., additional, Kassimos, Dimitrios G., additional, Kastner, Daniel L., additional, Katz, Jeffrey N., additional, Kay, Jonathan, additional, Kelly, Jennifer A., additional, Keystone, Edward, additional, Khamashta, Munther A., additional, Khanna, Dinesh, additional, Kjeken, Ingvild, additional, Koch, Alisa E., additional, Koff, Matthew F., additional, Kottyan, Leah, additional, Koutsogeorgopoulou, Loukia A., additional, Kraus, Virginia Byers, additional, Kumar, Pradeep, additional, Kvien, Tore K., additional, Lafyatis, Robert, additional, Landewé, Robert B.M., additional, Langford, Carol A., additional, Lau, Arthur N., additional, Laxer, Ronald M., additional, Learch, Thomas J., additional, Lewith, George, additional, Li, Yi, additional, Liao, Katherine P., additional, Littlejohn, Geoffrey, additional, Lorenzo, Pilar, additional, Luger, Thomas A., additional, Lundberg, Ingrid E., additional, Lundberg, Karin, additional, Machold, Klaus P., additional, MacKenzie, C. Ronald, additional, Mahr, Alfred D., additional, Manheimer, Eric, additional, Marini, Joan C., additional, Marquez, Javier, additional, Marsden, Debbie, additional, Martel-Pelletier, Johanne, additional, Martín-Mola, Emilio, additional, Martínez-Lavín, Manuel, additional, Massarotti, Elena M., additional, Matteson, Eric L., additional, Matzat, Stephen J., additional, Mayahi, Reza, additional, Mayes, Maureen Davidica, additional, McAlindon, Timothy, additional, McBain, Hayley, additional, McCarberg, Bill, additional, McCarthy, Edward F., additional, McCarthy, Geraldine, additional, McDermott, Michael F., additional, McGonagle, Dennis, additional, McLean, Lachy, additional, Merkel, Peter A., additional, Mikdashi, Jamal A., additional, Miller, Frederick W., additional, Miller, Paul D., additional, Minden, Kirsten, additional, Monach, Paul A., additional, Mulligan, Kathleen, additional, Namur, Gauthier, additional, Naredo, Esperanza, additional, Naylor, Kim E., additional, Nelson, Amanda E., additional, Newman, Stanton P., additional, Nordal, Ellen, additional, Nöth, Ulrich, additional, Ntatsaki, Eleana, additional, O'Shea, John J., additional, Oddis, Chester V., additional, Olivé, Alejandro, additional, Omair, Mohammed A., additional, Ombrello, Michael J., additional, Omisade, Antonina, additional, Ong, Voon H., additional, Önnerfjord, Patrik, additional, Orcel, Philippe, additional, Ospelt, Caroline, additional, Ozen, Seza, additional, Paget, Stephen A., additional, Patel, Dipak R., additional, Patrono, Carlo, additional, Pelletier, Jean-Pierre, additional, Pereira, Rosa Maria Rodrigues, additional, Pilkington, Clarissa A., additional, Pillinger, Michael H., additional, Pineda, Carlos, additional, Plenge, Robert M., additional, Price, Andrew, additional, Pricop, Luminita, additional, Rackwitz, Lars, additional, Ravelli, Angelo, additional, Redmond, Anthony C., additional, Reeves, Westley H., additional, Remmers, Elaine F., additional, Requena, Luis, additional, Ribbens, Clio, additional, Richardson, Bruce C., additional, Riera Alonso, Elena, additional, Riley, Graham, additional, Ritchlin, Christopher, additional, Ritter, Susan Y., additional, Rosas, Ivan O., additional, Rowan, Drew D., additional, Rudwaleit, Martin, additional, Rull, Marina, additional, Rygg, Marite, additional, Saag, Kenneth G., additional, Salmon, Jane E., additional, Salter, Donald M., additional, Salzberg, Daniel J., additional, Sambrook, Philip N., additional, Saxne, Tore, additional, Schaible, Hans-Georg, additional, Scher, Jose U., additional, Schett, Georg, additional, Schmitz, Nicole, additional, Schreiber, Benjamin, additional, Schumacher, H. Ralph, additional, Schwartz, Daniella Muallem, additional, Scott, David G.I., additional, Seton, Margaret, additional, Shapiro, Lauren M., additional, Sharby, Nancy, additional, Siegel, Jeffrey, additional, Siegel, Richard M., additional, Sieper, Joachim, additional, Silver, Richard M., additional, Silverberg, Shonni J., additional, Simard, Julia F., additional, Simmons, Barry P., additional, Simms, Robert W., additional, Singer, Nora G., additional, Smith, Malcolm D., additional, Smith, Stacy E., additional, Smolen, Josef S., additional, Spector, Tim D., additional, Steen, Virginia D., additional, Steere, Allen C., additional, Steiner, Günter, additional, Steinert, Andre F., additional, Stojan, George, additional, Stone, John H., additional, Strand, Vibeke, additional, Straub, Rainer H., additional, Streeten, Elizabeth A., additional, Superti-Furga, Giulio, additional, Symmons, Deborah P.M., additional, Szekanecz, Zoltan, additional, Tak, Paul P., additional, Tavoni, Antonio, additional, Taylor, Peter C., additional, Terkeltaub, Robert, additional, Thomas, Sarah S., additional, Thorne, Jennifer E., additional, Tobias, Jonathan H., additional, Tremoulet, Adriana H., additional, Tsokos, George C., additional, Tuan, Rocky S., additional, Turesson, Carl, additional, Unizony, Sebastian H., additional, Valdes, Ana M., additional, van den Berg, Wim B., additional, van der Heijde, Désirée, additional, van Vollenhoven, Ronald Frits, additional, Varga, John, additional, Vassilopoulos, Dimitrios, additional, Vital, Edward M., additional, Walker-Bone, Karen, additional, Wallace, Daniel J., additional, Warburton, Gary, additional, Ward, Robert J., additional, Watts, Richard, additional, Wechalekar, Mihir D., additional, Wedderburn, Lucy R., additional, Weinblatt, Michael E., additional, Weir, Matthew R., additional, Wenham, Claire Y.J., additional, West, Sterling G., additional, Weyand, Cornelia M., additional, White, Kenneth E., additional, Winthrop, Kevin L., additional, Wong, John B., additional, Woolf, Anthony D., additional, Worthington, Jane, additional, Xu, Huji, additional, Yazici, Hasan, additional, Young, D.A., additional, Yurdakul, Sebahattin, additional, Zhang, Yuqing, additional, and Zhuang, Haoyang, additional

Published
2015
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3. Geographical heterogeneity of clinical and serological phenotypes of systemic sclerosis observed at tertiary referral centres. The experience of the Italian SIR-SPRING registry and review of the world literature.

Author

Ferri C, De Angelis R, Giuggioli D, Bajocchi G, Dagna L, Zanframundo G, Foti R, Cacciapaglia F, Cuomo G, Ariani A, Rosato E, Guiducci S, Girelli F, Riccieri V, Zanatta E, Bosello S, Cavazzana I, Ingegnoli F, De Santis M, Murdaca G, Abignano G, Romeo N, Della Rossa A, Caminiti M, Iuliano A, Ciano G, Beretta L, Bagnato G, Lubrano E, De Andres I, Giollo A, Saracco M, Agnes C, Lumetti F, Spinella A, Magnani L, Campochiaro C, De Luca G, Codullo V, Visalli E, Masini F, Gigante A, Bellando-Randone S, Pellegrino G, Pigatto E, Lazzaroni MG, Franceschini F, Generali E, Mennillo G, Barsotti S, Mariano GP, Calabrese F, Furini F, Vultaggio L, Parisi S, Peroni CL, Rozza D, Zanetti A, Carrara G, Landolfi G, Scirè CA, Bianchi G, Fusaro E, Sebastiani GD, Govoni M, D'Angelo S, Cozzi F, Doria A, Iannone F, Salvarani C, and Matucci-Cerinic M

Subjects
Antibodies, Antinuclear, Humans, Italy epidemiology, Phenotype, Registries, Tertiary Care Centers, Rheumatology, Scleroderma, Systemic diagnosis
Abstract

Introduction: Systemic sclerosis (SSc) is characterized by a complex etiopathogenesis encompassing both host genetic and environmental -infectious/toxic- factors responsible for altered fibrogenesis and diffuse microangiopathy. A wide spectrum of clinical phenotypes may be observed in patients' populations from different geographical areas. We investigated the prevalence of specific clinical and serological phenotypes in patients with definite SSc enrolled at tertiary referral centres in different Italian geographical macro-areas. The observed findings were compared with those reported in the world literature., Materials and Methods: The clinical features of 1538 patients (161 M, 10.5%; mean age 59.8 ± 26.9 yrs.; mean disease duration 8.9 ± 7.7 yrs) with definite SSc recruited in 38 tertiary referral centres of the SPRING (Systemic sclerosis Progression INvestiGation Group) registry promoted by Italian Society of Rheumatology (SIR) were obtained and clustered according to Italian geographical macroareas., Results: Patients living in Southern Italy were characterized by more severe clinical and/or serological SSc phenotypes compared to those in Northern and Central Italy; namely, they show increased percentages of diffuse cutaneous SSc, digital ulcers, sicca syndrome, muscle involvement, arthritis, cardiopulmonary symptoms, interstitial lung involvement at HRCT, as well increased prevalence of serum anti-Scl70 autoantibodies. In the same SSc population immunusppressive drugs were frequently employed. The review of the literature underlined the geographical heterogeneity of SSc phenotypes, even if the observed findings are scarcely comparable due to the variability of methodological approaches., Conclusion: The phenotypical differences among SSc patients' subgroups from Italian macro-areas might be correlated to genetic/environmental co-factors, and possibly to a not equally distributed national network of information and healthcare facilities., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2022. Published by Elsevier B.V.)

Published
2022
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4. Prognostic Value of Lung Ultrasound B-Lines in Systemic Sclerosis.

Author

Gargani L, Bruni C, Romei C, Frumento P, Moreo A, Agoston G, Guiducci S, Bellando-Randone S, Lepri G, Belloli L, Della Rossa A, Delle Sedie A, Stagnaro C, De Nes M, Salvadori S, Mosca M, Falaschi F, Epis O, Picano E, and Matucci-Cerinic M

Subjects
Adult, Aged, Female, Humans, Male, Middle Aged, Prognosis, Prospective Studies, Ultrasonography, Lung Diseases, Interstitial diagnostic imaging, Lung Diseases, Interstitial etiology, Scleroderma, Systemic complications, Scleroderma, Systemic diagnostic imaging
Abstract

Background: A high percentage of systemic sclerosis (SSc) patients experience interstitial lung disease (ILD) during the disease course. Recent data have shown that lung ultrasound (LUS) can assess ILD by the evaluation of B-lines, the sonographic sign of pulmonary interstitial involvement., Research Question: To establish the prognostic value of B-lines in a large number of patients with SSc., Study Design and Methods: A total of 396 consecutive patients with SSc, who were enrolled at three Rheumatology Departments, underwent a comprehensive LUS examination on the anterolateral and posterior chest for a total of 58 scanning sites. All available clinical, imaging, and functional data were recorded. Patients were followed after enrolment to establish the prognostic role of LUS., Results: The median number of B-lines was higher in patients with the diffuse cutaneous subset (44 vs 17 B-lines; P < .0001), topoisomerase I autoantibodies (39 vs 16 B-lines; P < .0001), and the presence of ILD at chest high-resolution CT (45 vs 9 B-lines; P < .0001). At multivariable analysis, the number of posterior B-lines ≥5 was associated with new development or worsening ILD (hazard ratio, 3.378; 95% CI, 1.137-9.994; P = .028), with additional value over topoisomerase I positivity. The prognostic value was further confirmed in the subgroup of patients with known ILD at baseline (hazard ratio, 1.010; 95% CI, 1.003-1.018; P = .008)., Interpretation: Lung ultrasound B-lines are associated with worsening or development of pulmonary deterioration. In the near future, LUS might become part of the diagnostic and prognostic armamentarium in patients with SSc, which would allow a more sustainable and user-friendly approach to this very fragile population., (Copyright © 2020 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.)

Published
2020
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5. Clinical manifestations and treatment of Churg-Strauss syndrome.

Author

Baldini C, Talarico R, Della Rossa A, and Bombardieri S

Subjects
Adrenal Cortex Hormones administration & dosage, Antibodies, Monoclonal therapeutic use, Asthma complications, Asthma immunology, Churg-Strauss Syndrome complications, Churg-Strauss Syndrome drug therapy, Drug Therapy, Combination, Eosinophilia complications, Eosinophilia drug therapy, Eosinophilia immunology, Humans, Immunologic Factors therapeutic use, Immunosuppressive Agents administration & dosage, Remission Induction, Antibodies, Antineutrophil Cytoplasmic immunology, Churg-Strauss Syndrome immunology
Abstract

Churg-Strauss syndrome (CSS) is a systemic necrotizing vasculitis affecting small to medium-sized vessels, and characterized by asthma, blood hypereosinophilia, and eosinophil-rich granulomatous inflammation of the respiratory tract. In the past few years the pathogenesis of the disease and its clinical manifestations have been clarified, fostering important advances in the treatment of CSS. Systemic corticosteroids are still considered the cornerstone of treatment. Many issues need to be addressed, such as how to maintain remission, prevent disease relapses, and treat refractory disease. This review provides a clinical overview of CSS and a summary of the current treatments and novel therapies., (Copyright 2010 Elsevier Inc. All rights reserved.)

Published
2010
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