Treatment of systemic sclerosis: is there any hope for the future?
Open accessAbstract
Systemic sclerosis (SSc) is an orphan connective tissue disease characterised by skin and multiorgan involvement. The following original pathological processes distinguish SSc from other connective tissue diseases: (1) microvascular modifications, initially functional and partly reversible, (2) perivascular inflammation which appears to be moderate and perhaps transitory, (3) autoimmune activation, leading to the production of specific and persistent autoantibodies and (4) fibroblast activation, producing an excess of extracellular matrix leading to fibrosis.1 Therefore, SSc is a complex disease with the implication of multiple players in its pathogenesis. SSc is a major medical challenge with high mortality and morbidity. In a meta-analysis, the pooled standardised mortality ratio (SMR) was measured as 3.53 (95% CI 3.03 to 4.11) and adjusted metaregression did not show significant changes in SMR over time.2 Nevertheless, some reports did suggest an improved survival in recent years. In an Italian study, the 10-year survival showed a clear-cut increase (81%) compared with older series (69%) from the same centre.3 However, the discrepancy may not only come from improved therapeutic management, but might be due to some changes in the natural history, to an earlier referral of the patients and, even more likely, to the better recognition of patients with milder disease. Accordingly, in the recent Italian series, there were more patients with the limited cutaneous SSc (LcSSc) than in the older study (87.5% vs 72%).3 In terms of organ involvement and progression of the disease, the outcomes in LcSSc and diffuse cutaneous SSc (DcSSc) are different. The new classification criteria will more easily identify patients with LcSSc.4 Therefore, it will become core to require the cutaneous subsetting for any scientific work on SSc and, beyond skin, subclassification or clustering is awaited to improve SSc patient risk stratification. Regarding morbidity, accumulating evidence has shown the huge …
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Most deaths in systemic sclerosis are related to pulmonary fibrosis, pulmonary arterial hypertension, or cardiac causes.
“Most deaths are related to pulmonary fibrosis, pulmonary arterial hypertension, or cardiac causes.143”
From Autoimmune diseases and immune-checkpoint inhibitors for cancer therapy: Review of the literature and personalized risk-based prevention strategy (2020) · cites this paper as [143]
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Allanore, Y., Simms, R. W., Distler, O., Trojanowska, M. E., Pope, J. E., Denton, C. P., & Varga, J. (2015) · Nature Reviews Disease Primers · doi:10.1038/nrdp.2015.2 · Cited by 934 · see citing works
Self-citationvan Laar, J. M., Farge, D., Sont, J. K., Naraghi, K., Marjanovic, Z., Larghero, J., Schuerwegh, A. J., Marijt, E. W. A., Vonk, M. C., Schattenberg, A. V. M. B., et al. (2014) · JAMA · doi:10.1001/jama.2014.6368 · Cited by 764 · see citing works
Self-citationKhanna, D., Denton, C. P., Jahreis, A., van Laar, J. M., Frech, T. M., Anderson, M. E., Baron, M., Chung, L., Fierlbeck, G., Lakshminarayanan, S., et al. (2016) · The Lancet · doi:10.1016/s0140-6736(16)00232-4 · Cited by 683 · see citing works
Self-citationTherapy for Fibrotic Diseases: Nearing the Starting Line
Friedman, S. L., Sheppard, D., Duffield, J. S., & Violette, S. (2013) · Science Translational Medicine · doi:10.1126/scitranslmed.3004700 · Cited by 655 · see citing works
Elhaï, M., Meune, C., Avouac, J., Kahan, A., & Allanore, Y. (2011) · Lara D. Veeken · doi:10.1093/rheumatology/ker269 · Cited by 459 · see citing works
Self-citationOrphan nuclear receptor NR4A1 regulates transforming growth factor-β signaling and fibrosis
Palumbo‐Zerr, K., Zerr, P., Distler, A., Fliehr, J., Mancuso, R., Huang, J., Mielenz, D., Tomčík, M., Fürnrohr, B. G., Scholtysek, C., et al. (2015) · Nature Medicine · doi:10.1038/nm.3777 · Cited by 344 · see citing works
Self-citationHuang, J., Beyer, C., Palumbo-Zerr, K., Zhang, Y., Ramming, A., Distler, A., Gelse, K., Distler, O., Schett, G., Wollin, L., et al. (2016) · Annals of the Rheumatic Diseases · doi:10.1136/annrheumdis-2014-207109 · Cited by 196 · see citing works
Self-citationNikpour, M., Hissaria, P., Byron, J., Sahhar, J., Micallef, M., Paspaliaris, W., Roddy, J., Nash, P. T., Sturgess, A., Proudman, S., et al. (2011) · Arthritis Research & Therapy · doi:10.1186/ar3544 · Cited by 164 · see citing works
Khanna, D., Berrocal, V. J., Giannini, E. H., et al. (2016) · Arthritis & Rheumatology · doi:10.1002/art.39501 · Cited by 156 · see citing works
Maurer, B., Graf, N., Michel, B. A., et al. (2014) · Annals of the Rheumatic Diseases · doi:10.1136/annrheumdis-2014-205226 · Cited by 128 · see citing works
Frantz, C., Avouac, J., Distler, O., Amrouche, F., Godard, D., Kennedy, A. T., Connolly, K., Varga, J., Matucci‐Cerinic, M., & Allanore, Y. (2016) · Seminars in Arthritis and Rheumatism · doi:10.1016/j.semarthrit.2016.02.005 · Cited by 125 · see citing works
Self-citationSobanski, V., Dauchet, L., Lefèvre, G., Lambert, M., Morell‐Dubois, S., Sy, T., HACHULLA, E., Hatron, P., Launay, D., & Dubucquoi, S. (2013) · Arthritis & Rheumatology · doi:10.1002/art.38219 · Cited by 106 · see citing works
Pan PPAR agonist IVA337 is effective in prevention and treatment of experimental skin fibrosis
Ruzehaji, N., Frantz, C., Ponsoye, M., Avouac, J., Pezet, S., Guilbert, T., Luccarini, J., Broqua, P., Junien, J., & Allanore, Y. (2016) · Annals of the Rheumatic Diseases · doi:10.1136/annrheumdis-2015-208029 · Cited by 101 · see citing works
Self-citationFerri, C., Sebastiani, M., Monaco, A. L., Iudici, M., Giuggioli, D., Furini, F., Manfredi, A., Cuomo, G., Spinella, A., Colaci, M., et al. (2014) · Autoimmunity Reviews · doi:10.1016/j.autrev.2014.08.029 · Cited by 88 · see citing works
Dobrota, R. D., Maurer, B., Graf, N., et al. (2016) · Annals of the Rheumatic Diseases · doi:10.1136/annrheumdis-2015-208024 · Cited by 85 · see citing works
Performance of the new ACR/EULAR classification criteria for systemic sclerosis in clinical practice
Jordan, S., Maurer, B., Toniolo, M., et al. (2015) · Lara D. Veeken · doi:10.1093/rheumatology/keu530 · Cited by 84 · see citing works
Hamaguchi, Y., Kodera, M., Matsushita, T., et al. (2014) · Arthritis & Rheumatology · doi:10.1002/art.38994 · Cited by 84 · see citing works
Merkel, P. A., Silliman, N., Clements, P. J., Denton, C. P., Furst, D. E., Mayes, M. D., Pope, J. E., Polisson, R. P., Streisand, J. B., & Seibold, J. R. (2012) · Arthritis & Rheumatism · doi:10.1002/art.34427 · Cited by 76 · see citing works
Bossini‐Castillo, L., López‐Isac, E., & Martin, J. J. (2015) · Journal of Autoimmunity · doi:10.1016/j.jaut.2015.07.005 · Cited by 72 · see citing works
Dees, C., Beyer, C., Distler, A., Soare, A., Zhang, Y., Palumbo‐Zerr, K., Distler, O., Schett, G. A., Sandner, P., & Distler, J. H. W. (2015) · Annals of the Rheumatic Diseases · doi:10.1136/annrheumdis-2014-206809 · Cited by 70 · see citing works
Self-citationPonsoye, M., Frantz, C., Ruzehaji, N., Nicco, C., Elhai, M., Ruiz, B., Cauvet, A., Pezet, S., Brandely, M. L., Batteux, F., et al. (2016) · Annals of the Rheumatic Diseases · doi:10.1136/annrheumdis-2015-208213 · Cited by 70 · see citing works
Self-citationRegistries in systemic sclerosis: a worldwide experience
Galluccio, F., Walker, U. A., Nihtyanova, S. I., et al. (2010) · Lara D. Veeken · doi:10.1093/rheumatology/keq355 · Cited by 58 · see citing works
Iudici, M., Codullo, V., Giuggioli, D., et al. (2013) · PubMed · Cited by 36 · see citing works
Advances in cohort enrichment shape future of trial design
Allanore, Y., & Distler, O. (2015) · Nature Reviews Rheumatology · doi:10.1038/nrrheum.2014.222 · Cited by 36 · see citing works
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Haanen, J., Ernstoff, M. S., Wang, Y., Menzies, A. M., Puzanov, I., Grivas, P., Larkin, J., Peters, S., Thompson, J. A., & Obeid, M. (2020) · Annals of Oncology · doi:10.1016/j.annonc.2020.03.285 · Cited by 222 · see citing works
- Authors
- Allanore, Y., Matucci-Cerinic, M., & Distler, O.
- Affiliations
- Hôpital Cochin (FR)
- Department of Experimental and Clinical Medicine, Division of Rheumatology AOUC, University of Florence, Florence, Italy
- University Hospital Zurich (CH)
- Corresponding author
- Allanore, Yannick
- Venue
- RMD Open
- Publisher
- BMJ
- Published
- 2016-07
- Volume
- 2
- Issue
- 2
- Pages
- e000260
- ISSN
- 2056-5933 (ISSN-L)
- PMC
- PMC4947768
- OpenAlex
- W2473350718
- Cited by
- 19
- References
- 24
- Citations by year
- 2025: 2 · 2022: 1 · 2021: 4 · 2020: 3 · 2019: 3 · 2018: 4 · 2017: 2
- FWCI
- 2.73
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Licence
- cc-by-nc
- Locations
- RMD Open (publishedVersion, cc-by-nc)
- PubMed Central (publishedVersion, cc-by-nc)
- PubMed (publishedVersion)
- Zurich Open Repository and Archive (University of Zurich) (acceptedVersion, cc-by-nc)
- Zurich Open Repository and Archive (University of Zurich) (submittedVersion, cc-by-nc)
- Zurich Open Repository and Archive (University of Zurich)
- Funders
- Bristol-Myers Squibb
- Pfizer
- Sanofi
- Biogen
- Servier
- Medac
- Active Biotech
- Genentech
- Pharmacyclics
- Topics
- Health Sciences › Medicine › Pathology and Forensic Medicine › Systemic Sclerosis and Related Diseases
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Connective tissue disorders research
- Health Sciences › Medicine › Rheumatology › Dupuytren's Contracture and Treatments
- Keywords
- systemic sclerosis, limited cutaneous systemic sclerosis, diffuse cutaneous systemic sclerosis, connective tissue diseases, microvascular dysfunction, perivascular inflammation, autoantibodies, fibroblast activation, extracellular matrix, fibrosis, mortality, survival, disease classification criteria, risk stratification, treatment
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