[Systemic sclerosis].
Systemische Sklerose.
Connective tissue hyperplasia
Interdisciplinary health team
Organ-specific treatment
Quality of life
Scleroderma
Journal
Dermatologie (Heidelberg, Germany)
ISSN: 2731-7013
Titre abrégé: Dermatologie (Heidelb)
Pays: Germany
ID NLM: 9918384885206676
Informations de publication
Date de publication:
16 Feb 2024
16 Feb 2024
Historique:
accepted:
10
01
2024
medline:
17
2
2024
pubmed:
17
2
2024
entrez:
17
2
2024
Statut:
aheadofprint
Résumé
Systemic sclerosis (SSc) is a very heterogeneous, chronic, rare, but socioeconomically important disease with a severe disease course and severe impairment of the quality of life of affected patients. Overview of the current state of research on the pathogenesis, diagnosis and therapy of SSc. A literature search was performed. The pathogenesis of SSc is not fully understood. ACR/EULAR criteria allow the diagnosis of early forms of SSc. Classification into limited cutaneous SSc and diffuse cutaneous SSc is of prognostic and therapeutic relevance. New organ-specific treatment options for SSc have led to improved quality of life and prognosis. HINTERGRUND: Die systemische Sklerose (SSc) stellt eine sehr heterogene, chronische, zwar seltene, jedoch sozioökonomisch bedeutsame Erkrankung mit schwerem Krankheitsverlauf und starker Einschränkung der Lebensqualität betroffener Patienten dar. Es wird eine Übersicht zum aktuellen Forschungsstand zu Pathogenese, Diagnostik und Therapie der SSc gegeben. Es erfolgte eine Literaturrecherche. Die Pathogenese der SSc bleibt bislang weiterhin nicht gänzlich geklärt. ACR(American College of Rheumatology)/EULAR(European League Against Rheumatism)-Kriterien erlauben eine Diagnose auch früher SSc-Formen. Die Klassifikation in limitierte kutane systemische Sklerose (lcSSc) und diffuse kutane systemische Sklerose (dcSSc) ist von prognostischer und therapeutischer Relevanz. Neue organspezifische Therapiemöglichkeiten der SSc haben zu einer verbesserten Lebensqualität und Prognose geführt.
Sections du résumé
BACKGROUND
BACKGROUND
Systemic sclerosis (SSc) is a very heterogeneous, chronic, rare, but socioeconomically important disease with a severe disease course and severe impairment of the quality of life of affected patients.
OBJECTIVES
OBJECTIVE
Overview of the current state of research on the pathogenesis, diagnosis and therapy of SSc.
METHODS
METHODS
A literature search was performed.
RESULTS
RESULTS
The pathogenesis of SSc is not fully understood. ACR/EULAR criteria allow the diagnosis of early forms of SSc. Classification into limited cutaneous SSc and diffuse cutaneous SSc is of prognostic and therapeutic relevance. New organ-specific treatment options for SSc have led to improved quality of life and prognosis.
ZUSAMMENFASSUNG
UNASSIGNED
HINTERGRUND: Die systemische Sklerose (SSc) stellt eine sehr heterogene, chronische, zwar seltene, jedoch sozioökonomisch bedeutsame Erkrankung mit schwerem Krankheitsverlauf und starker Einschränkung der Lebensqualität betroffener Patienten dar.
FRAGESTELLUNG
UNASSIGNED
Es wird eine Übersicht zum aktuellen Forschungsstand zu Pathogenese, Diagnostik und Therapie der SSc gegeben.
MATERIAL UND METHODE
UNASSIGNED
Es erfolgte eine Literaturrecherche.
ERGEBNISSE
UNASSIGNED
Die Pathogenese der SSc bleibt bislang weiterhin nicht gänzlich geklärt. ACR(American College of Rheumatology)/EULAR(European League Against Rheumatism)-Kriterien erlauben eine Diagnose auch früher SSc-Formen. Die Klassifikation in limitierte kutane systemische Sklerose (lcSSc) und diffuse kutane systemische Sklerose (dcSSc) ist von prognostischer und therapeutischer Relevanz. Neue organspezifische Therapiemöglichkeiten der SSc haben zu einer verbesserten Lebensqualität und Prognose geführt.
Autres résumés
Type: Publisher
(ger)
HINTERGRUND: Die systemische Sklerose (SSc) stellt eine sehr heterogene, chronische, zwar seltene, jedoch sozioökonomisch bedeutsame Erkrankung mit schwerem Krankheitsverlauf und starker Einschränkung der Lebensqualität betroffener Patienten dar.
Identifiants
pubmed: 38366245
doi: 10.1007/s00105-024-05298-8
pii: 10.1007/s00105-024-05298-8
doi:
Types de publication
English Abstract
Journal Article
Review
Langues
ger
Sous-ensembles de citation
IM
Informations de copyright
© 2024. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.
Références
Bergamasco A et al (2019) Epidemiology of systemic sclerosis and systemic sclerosis-associated interstitial lung disease. Clin Epidemiol 11:257–273
pubmed: 31114386
pmcid: 6497473
doi: 10.2147/CLEP.S191418
Jerjen R et al (2022) Systemic sclerosis in adults. Part I: clinical features and pathogenesis. J Am Acad Dermatol 87(5):937–954
pubmed: 35131402
doi: 10.1016/j.jaad.2021.10.065
Arnett FC et al (2001) Familial occurrence frequencies and relative risks for systemic sclerosis (scleroderma) in three United States cohorts. Arthritis Rheum 44(6):1359–1362
pubmed: 11407695
doi: 10.1002/1529-0131(200106)44:6<1359::AID-ART228>3.0.CO;2-S
Rosendahl AH, Schonborn K, Krieg T (2022) Pathophysiology of systemic sclerosis (scleroderma). Kaohsiung J Med Sci 38(3):187–195
pubmed: 35234358
doi: 10.1002/kjm2.12505
De Martinis M et al (2016) An overview of environmental risk factors in systemic sclerosis. Expert Rev Clin Immunol 12(4):465–478
pubmed: 26610037
doi: 10.1586/1744666X.2016.1125782
Katsumoto TR, Whitfield ML, Connolly MK (2011) The pathogenesis of systemic sclerosis. Annu Rev Pathol 6:509–537
pubmed: 21090968
doi: 10.1146/annurev-pathol-011110-130312
van den Hoogen F et al (2013) 2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative. Ann Rheum Dis 72(11):1747–1755
pubmed: 24092682
doi: 10.1136/annrheumdis-2013-204424
LeRoy EC et al (1988) Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 15(2):202–205
pubmed: 3361530
LeRoy EC, Medsger TA Jr. (2001) Criteria for the classification of early systemic sclerosis. J Rheumatol 28(7):1573–1576
pubmed: 11469464
Sticherling M (2019) Systemic sclerosis—the dermatological perspective. J Dtsch Dermatol Ges 17(7):716–728
pubmed: 31364296
Harvey GR et al (1999) Clinical and serological associations with anti-RNA polymerase antibodies in systemic sclerosis. Clin Exp Immunol 117(2):395–402
pubmed: 10444276
pmcid: 1905338
doi: 10.1046/j.1365-2249.1999.00964.x
Knobler R et al (2017) European dermatology forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, part 1: localized scleroderma, systemic sclerosis and overlap syndromes. J Eur Acad Dermatol Venereol 31(9):1401–1424
pubmed: 28792092
doi: 10.1111/jdv.14458
Bellando-Randone S, Matucci-Cerinic M (2019) Very early systemic sclerosis. Best Pract Res Clin Rheumatol 33(4):101428
pubmed: 31810547
doi: 10.1016/j.berh.2019.101428
Hunzelmann N et al (2008) The registry of the German network for systemic scleroderma: frequency of disease subsets and patterns of organ involvement. Rheumatology 47(8):1185–1192
pubmed: 18515867
pmcid: 2468885
doi: 10.1093/rheumatology/ken179
Sticherling M (2019) Systemische sklerodermie – die dermatologische sicht. J Dtsch Dermatol Ges 17(7):716–729
pubmed: 31364296
Devgire V, Hughes M (2019) Raynaud’s phenomenon. Br J Hosp Med 80(11):658–664
doi: 10.12968/hmed.2019.80.11.658
Adigun R, Goyal A, Hariz A (2023) Systemic Sclerosis, in StatPearls. In: Treasure Island (FL) ineligible companies. Disclosure: Amandeep Goyal declares no relevant financial relationships with ineligible companies. Disclosure: Anis Hariz declares no relevant financial relationships with ineligible companies
Vitiello M et al (2012) An update on the treatment of the cutaneous manifestations of systemic sclerosis: the dermatologist’s point of view. J Clin Aesthet Dermatol 5(7):33–43
pubmed: 22798974
pmcid: 3396456
Cruz-Dominguez MP et al (2017) Clinical, biochemical, and radiological characterization of the calcinosis in a cohort of mexican patients with systemic sclerosis. Clin Rheumatol 36(1):111–117
pubmed: 27718018
doi: 10.1007/s10067-016-3412-9
Belz D, Hunzelmann N, Moinzadeh P (2014) Digital ulcers in systemic scleroderma. Hautarzt 65(11):944–948
pubmed: 25336296
doi: 10.1007/s00105-014-3528-2
Hachulla E et al (2007) Natural history of ischemic digital ulcers in systemic sclerosis: single-center retrospective longitudinal study. J Rheumatol 34(12):2423–2430
pubmed: 17985402
Amanzi L et al (2010) Digital ulcers in scleroderma: staging, characteristics and sub-setting through observation of 1614 digital lesions. Rheumatology 49(7):1374–1382
pubmed: 20400463
doi: 10.1093/rheumatology/keq097
Sunderkotter C et al (2009) Comparison of patients with and without digital ulcers in systemic sclerosis: detection of possible risk factors. Br J Dermatol 160(4):835–843
pubmed: 19183180
doi: 10.1111/j.1365-2133.2008.09004.x
Hughes M et al (2021) Digital ulcers in systemic sclerosis. Presse Med 50(1):104064
pubmed: 33548375
doi: 10.1016/j.lpm.2021.104064
Mihai C et al (2016) Digital ulcers predict a worse disease course in patients with systemic sclerosis. Ann Rheum Dis 75(4):681–686
pubmed: 25688073
doi: 10.1136/annrheumdis-2014-205897
Bruni C et al (2015) Digital ulcers as a sentinel sign for early internal organ involvement in very early systemic sclerosis. Rheumatology 54(1):72–76
pubmed: 25065009
doi: 10.1093/rheumatology/keu296
Giuggioli D et al (2012) Scleroderma digital ulcers complicated by infection with fecal pathogens. Arthritis Care Res 64(2):295–297
doi: 10.1002/acr.20673
Sticherling M (2012) Systemic sclerosis-dermatological aspects. Part 1: pathogenesis, epidemiology, clinical findings. J Dtsch Dermatol Ges 10(10):705–718 (quiz 716)
pubmed: 22913330
Odonwodo A, Badri T, Hariz A (2023) Scleroderma, in StatPearls. In: Treasure Island (FL) ineligible companies. Disclosure: Talel Badri declares no relevant financial relationships with ineligible companies. Disclosure: Anis Hariz declares no relevant financial relationships with ineligible companies
Moinzadeh P et al (2023) Impact of systemic sclerosis-associated interstitial lung disease with and without pulmonary hypertension on survival: a large cohort study of the German network for systemic sclerosis. Chest
Lescoat A et al (2023) Systemic sclerosis-associated interstitial lung disease in the EUSTAR database: analysis by region. Rheumatology 62(6):2178–2188
pubmed: 36222557
doi: 10.1093/rheumatology/keac576
Nihtyanova SI et al (2014) Prediction of pulmonary complications and long term survival in systemic sclerosis. Arthritis Rheumatol
Moinzadeh P et al (2014) Disease progression in systemic sclerosis-overlap syndrome is significantly different from limited and diffuse cutaneous systemic sclerosis. Ann Rheum Dis
Denton CP, Khanna D (2017) Systemic sclerosis. Lancet 390(10103):1685–1699
pubmed: 28413064
doi: 10.1016/S0140-6736(17)30933-9
Ntusi NA et al (2014) Subclinical myocardial inflammation and diffuse fibrosis are common in systemic sclerosis—a clinical study using myocardial T1-mapping and extracellular volume quantification. J Cardiovasc Magn Reson 16:21
pubmed: 24593856
pmcid: 3996013
doi: 10.1186/1532-429X-16-21
Balaj R, Poanta L, Rednic S (2012) Cardiac involvement in systemic sclerosis. Rom J Intern Med 50(4):269–274
pubmed: 23610974
Poudel DR, Derk CT (2018) Mortality and survival in systemic sclerosis: a review of recent literature. Curr Opin Rheumatol 30(6):588–593
pubmed: 30148801
doi: 10.1097/BOR.0000000000000551
Moinzadeh P et al (2020) Scleroderma renal crisis: risk factors for an increasingly rare organ complication. J Rheumatol 47(2):241–248
pubmed: 30936287
doi: 10.3899/jrheum.180582
Cutolo M et al (2000) Nailfold videocapillaroscopy assessment of microvascular damage in systemic sclerosis. J Rheumatol 27(1):155–160
pubmed: 10648032
Hasseli-Frabel R et al (2022) Nailfold capillaroscopy-principles and clinical application. Z Rheumatol 81(4):313–322
pubmed: 35445832
pmcid: 9022415
Mierau R et al (2011) Frequency of disease-associated and other nuclear autoantibodies in patients of the German network for systemic scleroderma: correlation with characteristic clinical features. Arthritis Res Ther 13(5):R172
pubmed: 22018289
pmcid: 3308107
doi: 10.1186/ar3495
Moinzadeh P et al (2014) Association of anti-RNA polymerase III autoantibodies and cancer in scleroderma. Arthritis Res Ther 16(1):R53
pubmed: 24524733
pmcid: 3978927
doi: 10.1186/ar4486
Gabrielli A, Avvedimento EV, Krieg T (2009) Scleroderma. N Engl J Med 360(19):1989–2003
pubmed: 19420368
doi: 10.1056/NEJMra0806188
Van Praet JT et al (2011) Histopathological cutaneous alterations in systemic sclerosis: a clinicopathological study. Arthritis Res Ther 13(1):R35
pubmed: 21356083
pmcid: 3241379
doi: 10.1186/ar3267
Hunzelmann N (2018) Current treatment of systemic scleroderma. Hautarzt 69(11):901–907
pubmed: 30143809
doi: 10.1007/s00105-018-4258-7
Hunzelmann N et al (2008) Organ-specific diagnosis in patients with systemic sclerosis: Recommendations of the German Network for Systemic Sclerosis (DNSS). Z Rheumatol 67(4):334–336 (337–40)
pubmed: 18418613
doi: 10.1007/s00393-008-0291-8
Siqueira VS et al (2022) Predictors of progression to systemic sclerosis: analysis of very early diagnosis of systemic sclerosis in a large single-centre cohort. Rheumatology 61(9):3686–3692
pubmed: 35020814
doi: 10.1093/rheumatology/keac006
Hughes M, Herrick AL (2019) Systemic sclerosis. Br J Hosp Med 80(9):530–536
doi: 10.12968/hmed.2019.80.9.530
Kowal-Bielecka O et al (2009) EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group (EUSTAR). Ann Rheum Dis 68(5):620–628
pubmed: 19147617
doi: 10.1136/ard.2008.096677
Hughes M et al (2015) Consensus best practice pathway of the UK scleroderma study group: digital vasculopathy in systemic sclerosis. Rheumatology 54(11):2015–2024
pubmed: 26116156
doi: 10.1093/rheumatology/kev201
Kowal-Bielecka O et al (2017) Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis 76(8):1327–1339
pubmed: 27941129
doi: 10.1136/annrheumdis-2016-209909
Juche A et al (2020) Reality of inpatient vasoactive treatment with prostacyclin derivatives in patients with acral circulation disorders due to systemic sclerosis in Germany. Z Rheumatol 79(10):1057–1066
pubmed: 32040755
pmcid: 7708340
doi: 10.1007/s00393-019-00743-9
Matucci-Cerinic M et al (2011) Bosentan treatment of digital ulcers related to systemic sclerosis: results from the RAPIDS‑2 randomised, double-blind, placebo-controlled trial. Ann Rheum Dis 70(1):32–38
pubmed: 20805294
doi: 10.1136/ard.2010.130658
Hughes M, Khanna D, Pauling JD (2020) Drug initiation and escalation strategies of vasodilator therapies for raynaud’s phenomenon: can we treat to target? Rheumatology 59(3):464–466
pubmed: 31670800
doi: 10.1093/rheumatology/kez522
Jerjen R et al (2022) Systemic sclerosis in adults. Part II: management and therapeutics. J Am Acad Dermatol 87(5):957–978
pubmed: 35131401
doi: 10.1016/j.jaad.2021.10.066
Saddic N et al (2009) Surgical debridement of painful fingertip calcinosis cutis in CREST syndrome. Arch Dermatol 145(2):212–213
pubmed: 19221282
doi: 10.1001/archderm.145.2.212-b
Kreuter A et al (2004) Low-dose UVA1 phototherapy in systemic sclerosis: effects on acrosclerosis. J Am Acad Dermatol 50(5):740–747
pubmed: 15097958
doi: 10.1016/j.jaad.2003.08.026
Das SN et al (2005) Placebo controlled trial of methotrexate in systemic sclerosis. Mymensingh Med J 14(1):71–74
pubmed: 15695960
Sullivan KM et al (2018) Myeloablative autologous stem-cell transplantation for severe scleroderma. N Engl J Med 378(1):35–47
pubmed: 29298160
pmcid: 5846574
doi: 10.1056/NEJMoa1703327
Blank N et al (2022) Autologous hematopoietic stem cell transplantation improves long-term survival-data from a national registry. Arthritis Res Ther 24(1):258
pubmed: 36424638
pmcid: 9685870
doi: 10.1186/s13075-022-02948-x
Steen VD, Medsger TA Jr. (1998) Case-control study of corticosteroids and other drugs that either precipitate or protect from the development of scleroderma renal crisis. Arthritis Rheum 41(9):1613–1619
pubmed: 9751093
doi: 10.1002/1529-0131(199809)41:9<1613::AID-ART11>3.0.CO;2-O
Volkmann ER, McMahan Z (2022) Gastrointestinal involvement in systemic sclerosis: pathogenesis, assessment and treatment. Curr Opin Rheumatol 34(6):328–336
pubmed: 35993874
pmcid: 9547962
doi: 10.1097/BOR.0000000000000899
Distler O et al (2019) Nintedanib for systemic sclerosis-associated interstitial lung disease. N Engl J Med 380(26):2518–2528
pubmed: 31112379
doi: 10.1056/NEJMoa1903076
Solomon JJ et al (2013) Scleroderma lung disease. Eur Respir Rev 22(127):6–19
pubmed: 23457159
pmcid: 4103193
doi: 10.1183/09059180.00005512