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Systemic sclerosis

MedGen UID:
19897
•Concept ID:
C0036421
•
Disease or Syndrome
Synonyms: Scleroderma, Systemic; Sclerosis, Systemic; Systemic Scleroderma; Systemic Sclerosis
SNOMED CT: Systemic sclerosis (89155008); Systemic scleroderma (89155008); SS - Systemic sclerosis (89155008)
Modes of inheritance:
Non-Mendelian inheritance
MedGen UID:
109109
•Concept ID:
C0600599
•
Genetic Function
Source: Orphanet
A mode of inheritance that depends on genetic determinants in more than one gene.
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Non-Mendelian inheritance (Orphanet)
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0005100
Orphanet: ORPHA90291

Definition

Systemic sclerosis (SSc) is a generalized disorder of small arteries, microvessels and connective tissue, characterized by fibrosis and vascular obliteration in the skin and organs, particularly the lungs, heart, and digestive tract. There are two main subsets of SSc: diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc (lcSSc) (see these terms). A third subset of SSc has also been observed, called limited Systemic Sclerosis (lSSc) or systemic sclerosis sine scleroderma (see these terms). [from ORDO]

Professional guidelines

PubMed

State-of-the-art evidence in the treatment of systemic sclerosis.
Pope JE, Denton CP, Johnson SR, Fernandez-Codina A, Hudson M, Nevskaya T
Nat Rev Rheumatol 2023 Apr;19(4):212-226. Epub 2023 Feb 27 doi: 10.1038/s41584-023-00909-5. PMID: 36849541Free PMC Article
Update of EULAR recommendations for the treatment of systemic sclerosis.
Kowal-Bielecka O, Fransen J, Avouac J, Becker M, Kulak A, Allanore Y, Distler O, Clements P, Cutolo M, Czirjak L, Damjanov N, Del Galdo F, Denton CP, Distler JHW, Foeldvari I, Figelstone K, Frerix M, Furst DE, Guiducci S, Hunzelmann N, Khanna D, Matucci-Cerinic M, Herrick AL, van den Hoogen F, van Laar JM, Riemekasten G, Silver R, Smith V, Sulli A, Tarner I, Tyndall A, Welling J, Wigley F, Valentini G, Walker UA, Zulian F, Müller-Ladner U; EUSTAR Coauthors
Ann Rheum Dis 2017 Aug;76(8):1327-1339. Epub 2016 Nov 9 doi: 10.1136/annrheumdis-2016-209909. PMID: 27941129
2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative.
van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, Matucci-Cerinic M, Naden RP, Medsger TA Jr, Carreira PE, Riemekasten G, Clements PJ, Denton CP, Distler O, Allanore Y, Furst DE, Gabrielli A, Mayes MD, van Laar JM, Seibold JR, Czirjak L, Steen VD, Inanc M, Kowal-Bielecka O, Müller-Ladner U, Valentini G, Veale DJ, Vonk MC, Walker UA, Chung L, Collier DH, Ellen Csuka M, Fessler BJ, Guiducci S, Herrick A, Hsu VM, Jimenez S, Kahaleh B, Merkel PA, Sierakowski S, Silver RM, Simms RW, Varga J, Pope JE
Ann Rheum Dis 2013 Nov;72(11):1747-55. doi: 10.1136/annrheumdis-2013-204424. PMID: 24092682

Recent clinical studies

Etiology

Metabolomics in systemic sclerosis.
Gogulska Z, Smolenska Z, Turyn J, Zdrojewski Z, Chmielewski M
Rheumatol Int 2024 Oct;44(10):1813-1822. Epub 2024 Jul 9 doi: 10.1007/s00296-024-05628-y. PMID: 38981905Free PMC Article
Systemic sclerosis.
Volkmann ER, Andréasson K, Smith V
Lancet 2023 Jan 28;401(10373):304-318. Epub 2022 Nov 25 doi: 10.1016/S0140-6736(22)01692-0. PMID: 36442487Free PMC Article
Environmental Risks for Systemic Sclerosis.
Alahmari H, Ahmad Z, Johnson SR
Rheum Dis Clin North Am 2022 Nov;48(4):845-860. doi: 10.1016/j.rdc.2022.06.006. PMID: 36332999
Employment outcomes in systemic sclerosis.
Lee JJY, Gignac MAM, Johnson SR
Best Pract Res Clin Rheumatol 2021 Sep;35(3):101667. Epub 2021 Mar 10 doi: 10.1016/j.berh.2021.101667. PMID: 33714678
Racial Disparities in Systemic Sclerosis.
Moore DF, Steen VD
Rheum Dis Clin North Am 2020 Nov;46(4):705-712. Epub 2020 Sep 8 doi: 10.1016/j.rdc.2020.07.009. PMID: 32981647

Diagnosis

Systemic sclerosis: one year in review 2023.
Di Battista M, Lepri G, Codullo V, Da Rio M, Fiorentini E, Della Rossa A, Guiducci S
Clin Exp Rheumatol 2023 Aug;41(8):1567-1574. Epub 2023 May 17 doi: 10.55563/clinexprheumatol/ki76s5. PMID: 37199215
Systemic sclerosis.
Volkmann ER, Andréasson K, Smith V
Lancet 2023 Jan 28;401(10373):304-318. Epub 2022 Nov 25 doi: 10.1016/S0140-6736(22)01692-0. PMID: 36442487Free PMC Article
Systemic sclerosis: one year in review 2022.
Lepri G, Orlandi M, Di Battista M, De Mattia G, Da Rio M, Codullo V, Guiducci S, Della Rossa A
Clin Exp Rheumatol 2022 Oct;40(10):1911-1920. Epub 2022 Sep 21 doi: 10.55563/clinexprheumatol/3401fl. PMID: 36135958
Systemic sclerosis.
Denton CP, Khanna D
Lancet 2017 Oct 7;390(10103):1685-1699. Epub 2017 Apr 13 doi: 10.1016/S0140-6736(17)30933-9. PMID: 28413064
Systemic sclerosis.
Hughes M, Herrick A
Br J Hosp Med (Lond) 2012 Sep;73(9):509-10, 511-6. doi: 10.12968/hmed.2012.73.9.509. PMID: 23124403

Therapy

Novel Therapeutic Approaches in Connective Tissue Disease-Associated Interstitial Lung Disease.
Mulcaire-Jones E, Pugashetti JV, Oldham JM, Khanna D
Semin Respir Crit Care Med 2024 Jun;45(3):435-448. Epub 2024 May 13 doi: 10.1055/s-0044-1786155. PMID: 38740369
State-of-the-art evidence in the treatment of systemic sclerosis.
Pope JE, Denton CP, Johnson SR, Fernandez-Codina A, Hudson M, Nevskaya T
Nat Rev Rheumatol 2023 Apr;19(4):212-226. Epub 2023 Feb 27 doi: 10.1038/s41584-023-00909-5. PMID: 36849541Free PMC Article
Treatment for systemic sclerosis-associated interstitial lung disease.
Roofeh D, Lescoat A, Khanna D
Curr Opin Rheumatol 2021 May 1;33(3):240-248. doi: 10.1097/BOR.0000000000000795. PMID: 33741803Free PMC Article
Nintedanib for Systemic Sclerosis-Associated Interstitial Lung Disease.
Distler O, Highland KB, Gahlemann M, Azuma A, Fischer A, Mayes MD, Raghu G, Sauter W, Girard M, Alves M, Clerisme-Beaty E, Stowasser S, Tetzlaff K, Kuwana M, Maher TM; SENSCIS Trial Investigators
N Engl J Med 2019 Jun 27;380(26):2518-2528. Epub 2019 May 20 doi: 10.1056/NEJMoa1903076. PMID: 31112379
Immunosuppressive drug therapy.
McCune WJ, Vallance DK, Lynch JP 3rd
Curr Opin Rheumatol 1994 May;6(3):262-72. doi: 10.1097/00002281-199405000-00004. PMID: 8060760

Prognosis

Cancer and scleroderma: recent insights.
Morrisroe K, Nikpour M
Curr Opin Rheumatol 2020 Nov;32(6):479-487. doi: 10.1097/BOR.0000000000000755. PMID: 33002949
Lung transplantation for scleroderma: Strength in numbers.
Gelber AC
Joint Bone Spine 2018 Jan;85(1):5-7. Epub 2017 Sep 14 doi: 10.1016/j.jbspin.2017.09.002. PMID: 28917997
Systemic sclerosis.
Hughes M, Herrick A
Br J Hosp Med (Lond) 2012 Sep;73(9):509-10, 511-6. doi: 10.12968/hmed.2012.73.9.509. PMID: 23124403
Epidemiology of systemic sclerosis.
Nikpour M, Stevens WM, Herrick AL, Proudman SM
Best Pract Res Clin Rheumatol 2010 Dec;24(6):857-69. doi: 10.1016/j.berh.2010.10.007. PMID: 21665131
Gastrointestinal manifestations of systemic sclerosis.
Abu-Shakra M, Guillemin F, Lee P
Semin Arthritis Rheum 1994 Aug;24(1):29-39. doi: 10.1016/0049-0172(94)90097-3. PMID: 7985035

Clinical prediction guides

Moving in the "right" direction: exposing subclinical right ventricular disease with exercise in patients with systemic sclerosis.
Hale S, Freed BH
Int J Cardiovasc Imaging 2021 Jul;37(7):2149-2150. doi: 10.1007/s10554-021-02269-1. PMID: 33999352
Giant calcinosis revealing systemic sclerosis.
Meriglier E, Lafourcade F, Gombert B, Landron C
Int J Rheum Dis 2019 Sep;22(9):1787-1788. Epub 2019 Jul 2 doi: 10.1111/1756-185X.13648. PMID: 31267677
Imaging Risk in Multisystem Inflammatory Diseases.
Ikonomidis I, Makavos G, Katsimbri P, Boumpas DT, Parissis J, Iliodromitis E
JACC Cardiovasc Imaging 2019 Dec;12(12):2517-2537. Epub 2019 Mar 13 doi: 10.1016/j.jcmg.2018.06.033. PMID: 30878436
ESVM guidelines - the diagnosis and management of Raynaud's phenomenon.
Belch J, Carlizza A, Carpentier PH, Constans J, Khan F, Wautrecht JC, Visona A, Heiss C, Brodeman M, Pécsvárady Z, Roztocil K, Colgan MP, Vasic D, Gottsäter A, Amann-Vesti B, Chraim A, Poredoš P, Olinic DM, Madaric J, Nikol S, Herrick AL, Sprynger M, Klein-Weigel P, Hafner F, Staub D, Zeman Z
Vasa 2017 Oct;46(6):413-423. Epub 2017 Sep 12 doi: 10.1024/0301-1526/a000661. PMID: 28895508
Pulmonary arterial hypertension associated with systemic sclerosis.
Mathai SC, Hassoun PM
Expert Rev Respir Med 2011 Apr;5(2):267-79. doi: 10.1586/ers.11.18. PMID: 21510736Free PMC Article

Recent systematic reviews

Prevalence, imaging patterns and risk factors of interstitial lung disease in connective tissue disease: a systematic review and meta-analysis.
Joy GM, Arbiv OA, Wong CK, Lok SD, Adderley NA, Dobosz KM, Johannson KA, Ryerson CJ
Eur Respir Rev 2023 Mar 31;32(167) Epub 2023 Mar 8 doi: 10.1183/16000617.0210-2022. PMID: 36889782Free PMC Article
JAK inhibitors and systemic sclerosis: A systematic review of the literature.
Moriana C, Moulinet T, Jaussaud R, Decker P
Autoimmun Rev 2022 Oct;21(10):103168. Epub 2022 Aug 6 doi: 10.1016/j.autrev.2022.103168. PMID: 35944611
Treatment of calcinosis cutis in systemic sclerosis and dermatomyositis: A review of the literature.
Traineau H, Aggarwal R, Monfort JB, Senet P, Oddis CV, Chizzolini C, Barbaud A, Francès C, Arnaud L, Chasset F
J Am Acad Dermatol 2020 Feb;82(2):317-325. Epub 2019 Jul 11 doi: 10.1016/j.jaad.2019.07.006. PMID: 31302187
Assessment of skin involvement in systemic sclerosis.
Kumánovics G, Péntek M, Bae S, Opris D, Khanna D, Furst DE, Czirják L
Rheumatology (Oxford) 2017 Sep 1;56(suppl_5):v53-v66. doi: 10.1093/rheumatology/kex202. PMID: 28992173Free PMC Article
Update of EULAR recommendations for the treatment of systemic sclerosis.
Kowal-Bielecka O, Fransen J, Avouac J, Becker M, Kulak A, Allanore Y, Distler O, Clements P, Cutolo M, Czirjak L, Damjanov N, Del Galdo F, Denton CP, Distler JHW, Foeldvari I, Figelstone K, Frerix M, Furst DE, Guiducci S, Hunzelmann N, Khanna D, Matucci-Cerinic M, Herrick AL, van den Hoogen F, van Laar JM, Riemekasten G, Silver R, Smith V, Sulli A, Tarner I, Tyndall A, Welling J, Wigley F, Valentini G, Walker UA, Zulian F, Müller-Ladner U; EUSTAR Coauthors
Ann Rheum Dis 2017 Aug;76(8):1327-1339. Epub 2016 Nov 9 doi: 10.1136/annrheumdis-2016-209909. PMID: 27941129

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