Cutaneous Manifestations, Clinical Characteristics, and Prognosis of Patients With Systemic Sclerosis Sine Scleroderma: Data From the International EUSTAR Database.
Détails
ID Serval
serval:BIB_6D3F9BC1AB36
Type
Article: article d'un périodique ou d'un magazine.
Collection
Publications
Institution
Titre
Cutaneous Manifestations, Clinical Characteristics, and Prognosis of Patients With Systemic Sclerosis Sine Scleroderma: Data From the International EUSTAR Database.
Périodique
JAMA dermatology
Collaborateur⸱rice⸱s
EUSTAR collaborators
Contributeur⸱rice⸱s
Matucci Cerinic M., Walker U., Iannone F., Becvar R., Kowal Bielecka O., Pizzorni C., Ciccia F., Siegert E., Rednic S., Vlachoyiannopoulos P.G., Stork J., Inanc M., Carreira P.E., Novak S., Czirják L., Iudici M., Kucharz E.J., Perdan-Pirkmajer K., Coleiro B., Moroncini G., Farge Bancel D., Hesselstrand R., Radic M., Balbir-Gurman A., Lo Monaco A., Pellerito R., Giollo A., Morovic-Vergles J., Denton C., Vonk M., Damjanov N., Henes J., Ortiz Santamaria V., Heitmann S., Krasowska D., Hasler P., Kohm M., Foeldvari I., Bajocchi G., João Salvador M., Stamenkovic B., Selmi C.F., Tikly M., Ananieva L.P., Herrick A., Müller-Ladner U., Søndergaard K., Puppo F., Engelhart M., Szücs G., de la Puente C., Riccieri V., Ionescu R.M., Sha A., Gheorghiu A.M., Sunderkötter C., Distler J., Ingegnoli F., Mouthon L., Smith V., Paolo Cantatore F., Ullman S., von Mühlen C.A., Pozzi M.R., Eyerich K., Wiland P., Vanthuyne M., Alegre-Sancho J.J., Herrmann K., De Langhe E., Anic B., Baresic M., Mayer M., Üprus M., Otsa K., Yavuz S., Granel B., de Souza Müller C., Agachi S., Stebbings S., Mathieu A., Vacca A., Sampaio-Barros P.D., Stamp L., Solanki K., Veale D., Loyo E., Tineo C., Toloza S., Li M., Mohamed WAAA, Olas J., Oksel F., Yargucu F., Tanaseanu C.M., Foti R., Ancuta C., Furst D.E., Maurer B., van Laar J., Olesinska M., Kayser C., Fathi N., de la Peña Lefebvre P.G., Martin JJG, Carpentier P., Imbert B., Francès C., Senet P., Sibilia J., Litinsky I., Senécal J.L., Koenig M., Joval F., Tamara G., Del Galdo F., Seskute G., Saketkoo L.A., Kerzberg E., Bianchi W., Valdetaro Bianchi B., Castellví I., Milas-Ahic J., Visevic R., Limonta M., Rimar D., Couto M., Spertini F., Marcoccia A., Kahl S., Hsu V.M., Martin T., Moiseev S., Novikov P., Chung L.S., Schmeiser T., Majewski D., Zdrojewski Z., Martínez-Barrio J., Khanna D., Bernardino V., Santo L., Levy Y., Rezus E., Nuri Pamuk O., Brito de Araujo D., Sarzi Puttini P., Brzosko M., Poormoghim H., Maman M., Kötter I., Cuomo G., Gaches F., Belloli L., Sfikakis P., Markus J., Furst D., Ramazan A.M., Truchetet M.E., Jego P., Dagna L., van Laar J.M., Voigt L., Oliveira S., Atzeni F., Kuwana M., Mekinian A., Martin M., Tanaka Y., Yasuoka H., Simeón-Aznar C.P., Atsumi T., Parvu M., Cordeiro I., Del Papa N., Karonitsch T., Bazela-Ostromecka A., Selvi E., Kawaguchi Y., Soukup T., Rodriguez-Pinto I., Geroldinger-Simic M., Espinosa G., Voigt K., Kubacki T., Garmish O., Mosca M., Gerth U., Antonenko L., Mendoza F.A., Airò P., de Vries-Bouwstra J., Riemekasten G., Hachulla E., Doria A., Rosato E., Hunzelmann N., Montecucco C., Gabrielli A., Hoffmann-Vold A.M., Distler O., Ben Shimol J., Cutolo M., Allanore Y.
ISSN
2168-6084 (Electronic)
ISSN-L
2168-6068
Statut éditorial
Publié
Date de publication
01/08/2023
Peer-reviewed
Oui
Volume
159
Numéro
8
Pages
837-847
Langue
anglais
Notes
Publication types: Observational Study ; Journal Article
Publication Status: ppublish
Publication Status: ppublish
Résumé
Systemic sclerosis (SSc) sine scleroderma (ssSSc) is a subset of SSc defined by the absence of skin fibrosis. Little is known about the natural history and skin manifestations among patients with ssSSc.
To characterize the clinical phenotype of patients with ssSSc compared with patients with limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc (dcSSc) within the EUSTAR database.
This longitudinal observational cohort study based on the international EUSTAR database included all patients fulfilling the classification criteria for SSc assessed by the modified Rodnan Skin score (mRSS) at inclusion and with at least 1 follow-up visit; ssSSc was defined by the absence of skin fibrosis (mRSS = 0 and no sclerodactyly) at all available visits. Data extraction was performed in November 2020, and data analysis was performed from April 2021 to April 2023.
Main outcomes were survival and skin manifestations (onset of skin fibrosis, digital ulcers, telangiectasias, puffy fingers).
Among the 4263 patients fulfilling the inclusion criteria, 376 (8.8%) were classified as having ssSSc (mean [SD] age, 55.3 [13.9] years; 345 [91.8%] were female). At last available visit, in comparison with 708 patients with lcSSc and 708 patients with dcSSc with the same disease duration, patients with ssSSc had a lower prevalence of previous or current digital ulcers (28.2% vs 53.1% in lcSSc; P < .001; and 68.3% in dcSSc; P < .001) and puffy fingers (63.8% vs 82.4% in lcSSc; P < .001; and 87.6% in dcSSc; P < .001). By contrast, the prevalence of interstitial lung disease was similar in ssSSc and lcSSc (49.8% and 57.1%; P = .03) but significantly higher in dcSSc (75.0%; P < .001). Skin telangiectasias were associated with diastolic dysfunction in patients with ssSSc (odds ratio, 4.778; 95% CI, 2.060-11.081; P < .001). The only independent factor for the onset of skin fibrosis in ssSSc was the positivity for anti-Scl-70 antibodies (odds ratio, 3.078; 95% CI, 1.227-7.725; P = .02). Survival rate was higher in patients with ssSSc (92.4%) compared with lcSSc (69.4%; P = .06) and dcSSc (55.5%; P < .001) after up to 15 years of follow-up.
Systemic sclerosis sine scleroderma should not be neglected considering the high prevalence of interstitial lung disease (>40%) and SSc renal crisis (almost 3%). Patients with ssSSc had a higher survival than other subsets. Dermatologists should be aware that cutaneous findings in this subgroup may be associated with internal organ dysfunction. In particular, skin telangiectasias in ssSSc were associated with diastolic heart dysfunction.
To characterize the clinical phenotype of patients with ssSSc compared with patients with limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc (dcSSc) within the EUSTAR database.
This longitudinal observational cohort study based on the international EUSTAR database included all patients fulfilling the classification criteria for SSc assessed by the modified Rodnan Skin score (mRSS) at inclusion and with at least 1 follow-up visit; ssSSc was defined by the absence of skin fibrosis (mRSS = 0 and no sclerodactyly) at all available visits. Data extraction was performed in November 2020, and data analysis was performed from April 2021 to April 2023.
Main outcomes were survival and skin manifestations (onset of skin fibrosis, digital ulcers, telangiectasias, puffy fingers).
Among the 4263 patients fulfilling the inclusion criteria, 376 (8.8%) were classified as having ssSSc (mean [SD] age, 55.3 [13.9] years; 345 [91.8%] were female). At last available visit, in comparison with 708 patients with lcSSc and 708 patients with dcSSc with the same disease duration, patients with ssSSc had a lower prevalence of previous or current digital ulcers (28.2% vs 53.1% in lcSSc; P < .001; and 68.3% in dcSSc; P < .001) and puffy fingers (63.8% vs 82.4% in lcSSc; P < .001; and 87.6% in dcSSc; P < .001). By contrast, the prevalence of interstitial lung disease was similar in ssSSc and lcSSc (49.8% and 57.1%; P = .03) but significantly higher in dcSSc (75.0%; P < .001). Skin telangiectasias were associated with diastolic dysfunction in patients with ssSSc (odds ratio, 4.778; 95% CI, 2.060-11.081; P < .001). The only independent factor for the onset of skin fibrosis in ssSSc was the positivity for anti-Scl-70 antibodies (odds ratio, 3.078; 95% CI, 1.227-7.725; P = .02). Survival rate was higher in patients with ssSSc (92.4%) compared with lcSSc (69.4%; P = .06) and dcSSc (55.5%; P < .001) after up to 15 years of follow-up.
Systemic sclerosis sine scleroderma should not be neglected considering the high prevalence of interstitial lung disease (>40%) and SSc renal crisis (almost 3%). Patients with ssSSc had a higher survival than other subsets. Dermatologists should be aware that cutaneous findings in this subgroup may be associated with internal organ dysfunction. In particular, skin telangiectasias in ssSSc were associated with diastolic heart dysfunction.
Mots-clé
Female, Male, Humans, Scleroderma, Systemic/complications, Scleroderma, Systemic/epidemiology, Scleroderma, Diffuse/complications, Scleroderma, Diffuse/diagnosis, Scleroderma, Diffuse/epidemiology, Lung Diseases, Interstitial/complications, Fibrosis, Prognosis, Telangiectasis/etiology, Telangiectasis/complications
Pubmed
Web of science
Création de la notice
29/06/2023 14:03
Dernière modification de la notice
19/12/2023 7:15
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