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Limited systemic sclerosis

MedGen UID:
220934
•Concept ID:
C1290138
•
Disease or Syndrome
Synonyms: limited systemic sclerosis; progressive systemic sclerosis sine scleroderma; Scleroderma, sine; SSC without Skin Involvement; SSC without skin involvement; Systemic Sclerosis sine Scleroderma; Systemic sclerosis sine scleroderma; systemic sclerosis sine scleroderma; Systemic Sclerosis without Skin Involvement; systemic sclerosis without skin involvement; Systemic sclerosis without skin thickening
SNOMED CT: Systemic sclerosis without skin thickening (128461001); Systemic sclerosis sine scleroderma (128461001)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0016359
Orphanet: ORPHA220407

Definition

Limited systemic sclerosis (lSSc) (or SSc sine scleroderma) is a subset of systemic sclerosis (SSc; see this term) characterized by organ involvement in the absence of fibrosis of the skin. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLimited systemic sclerosis

Professional guidelines

PubMed

Treatment of complications associated with systemic sclerosis.
Moore SC, Desantis ER
Am J Health Syst Pharm 2008 Feb 15;65(4):315-21. doi: 10.2146/ajhp070024. PMID: 18238768
Treatment of cutaneous calcinosis in limited systemic sclerosis with minocycline.
Robertson LP, Marshall RW, Hickling P
Ann Rheum Dis 2003 Mar;62(3):267-9. doi: 10.1136/ard.62.3.267. PMID: 12594118Free PMC Article
Digital calcification in systemic sclerosis: effective treatment with good tissue preservation using the carbon dioxide laser.
Bottomley WW, Goodfield MJ, Sheehan-Dare RA
Br J Dermatol 1996 Aug;135(2):302-4. PMID: 8881679

Recent clinical studies

Etiology

Native T1 mapping in early diffuse and limited systemic sclerosis, and its association with diastolic function.
Purevsuren M, Uehara M, Ishizuka M, Suzuki Y, Shimbo M, Kakuda N, Ishii S, Sumida H, Miyazaki M, Yamashita T, Yoshizaki A, Asano Y, Sato S, Hatano M, Komuro I
J Cardiol 2023 Aug;82(2):100-107. Epub 2023 Mar 13 doi: 10.1016/j.jjcc.2023.03.003. PMID: 36921691
Acroosteolysis and bone metabolism parameters distinguish female patients with limited systemic sclerosis with and without calcinosis: a case control study.
Sampaio-Barros MM, Castelo Branco LCM, Takayama L, Pontes Filho MAG, Sampaio-Barros PD, Pereira RMR
Clin Rheumatol 2019 Nov;38(11):3189-3193. Epub 2019 Jun 19 doi: 10.1007/s10067-019-04637-8. PMID: 31218481
Association between Skin Thickness Measurements with Corneal Biomechanical Properties and Dry Eye Tests in Systemic Sclerosis.
Ayhan Z, Kaya M, Ozturk T, Arikan G, Birlik M
Ocul Immunol Inflamm 2019;27(7):1138-1143. Epub 2018 Aug 15 doi: 10.1080/09273948.2018.1501496. PMID: 30110190
Poly(ADP-ribose) polymerase activity in systemic lupus erythematosus and systemic sclerosis.
Cerboni B, Morozzi G, Galeazzi M, Bellisai F, Micheli V, Pompucci G, Sestini S
Hum Immunol 2009 Jul;70(7):487-91. Epub 2009 Apr 17 doi: 10.1016/j.humimm.2009.04.021. PMID: 19376176
Connective tissue diseases and pregnancy.
Saar P, Hermann W, Müller-Ladner U
Rheumatology (Oxford) 2006 Oct;45 Suppl 3:iii30-2. doi: 10.1093/rheumatology/kel288. PMID: 16987830

Diagnosis

Native T1 mapping in early diffuse and limited systemic sclerosis, and its association with diastolic function.
Purevsuren M, Uehara M, Ishizuka M, Suzuki Y, Shimbo M, Kakuda N, Ishii S, Sumida H, Miyazaki M, Yamashita T, Yoshizaki A, Asano Y, Sato S, Hatano M, Komuro I
J Cardiol 2023 Aug;82(2):100-107. Epub 2023 Mar 13 doi: 10.1016/j.jjcc.2023.03.003. PMID: 36921691
Sine Scleroderma.
Kaur G, Banka S, Meena B, Kulkarni A, Prajapat R, Dhaka J
J Assoc Physicians India 2020 Apr;68(4):68-70. PMID: 32610852
L-selectin and skin damage in systemic sclerosis.
Dunne JV, van Eeden SF, Keen KJ
PLoS One 2012;7(9):e44814. Epub 2012 Sep 13 doi: 10.1371/journal.pone.0044814. PMID: 23028631Free PMC Article
Palisaded neutrophilic and granulomatous dermatitis associated with limited systemic sclerosis.
Hantash BM, Chiang D, Kohler S, Fiorentino D
J Am Acad Dermatol 2008 Apr;58(4):661-4. Epub 2007 Oct 22 doi: 10.1016/j.jaad.2007.09.019. PMID: 17945384
Localized and systemic scleroderma.
Hawk A, English JC 3rd
Semin Cutan Med Surg 2001 Mar;20(1):27-37. doi: 10.1053/sder.2001.23093. PMID: 11308134

Therapy

Limited Systemic Sclerosis-Associated Calcinosis Cutis of the Fingers Treated Successfully With Ablative Continuous-Wave Carbon Dioxide Laser and Curettage.
McTighe SP, Simpson M
Dermatol Surg 2023 Apr 1;49(4):424-425. Epub 2023 Feb 28 doi: 10.1097/DSS.0000000000003729. PMID: 36857163
Juvenile systemic sclerosis: experience from a tertiary care center from India.
Bagri NK, Raj D, Kaur J, Punia H, Saini I, Lodha R, Kabra SK
Rheumatol Int 2017 Oct;37(10):1687-1691. Epub 2017 Aug 22 doi: 10.1007/s00296-017-3793-3. PMID: 28831595
Limited systemic sclerosis initially presenting with mesenteric panniculitis.
Arroyo-Ávila M, Vilá LM
BMJ Case Rep 2014 Oct 17;2014 doi: 10.1136/bcr-2014-206961. PMID: 25326572Free PMC Article
Treatment of cutaneous calcinosis in limited systemic sclerosis with minocycline.
Robertson LP, Marshall RW, Hickling P
Ann Rheum Dis 2003 Mar;62(3):267-9. doi: 10.1136/ard.62.3.267. PMID: 12594118Free PMC Article
Localized and systemic scleroderma.
Hawk A, English JC 3rd
Semin Cutan Med Surg 2001 Mar;20(1):27-37. doi: 10.1053/sder.2001.23093. PMID: 11308134

Prognosis

Native T1 mapping in early diffuse and limited systemic sclerosis, and its association with diastolic function.
Purevsuren M, Uehara M, Ishizuka M, Suzuki Y, Shimbo M, Kakuda N, Ishii S, Sumida H, Miyazaki M, Yamashita T, Yoshizaki A, Asano Y, Sato S, Hatano M, Komuro I
J Cardiol 2023 Aug;82(2):100-107. Epub 2023 Mar 13 doi: 10.1016/j.jjcc.2023.03.003. PMID: 36921691
Association between Skin Thickness Measurements with Corneal Biomechanical Properties and Dry Eye Tests in Systemic Sclerosis.
Ayhan Z, Kaya M, Ozturk T, Arikan G, Birlik M
Ocul Immunol Inflamm 2019;27(7):1138-1143. Epub 2018 Aug 15 doi: 10.1080/09273948.2018.1501496. PMID: 30110190
Cardio-pulmonary involvement in systemic sclerosis: A study at a tertiary care center.
Arakkal G, Chintagunta SR, Chandika V, Damarla SV, Manchala S, Kumar BU
Indian J Dermatol Venereol Leprol 2017 Nov-Dec;83(6):677-682. doi: 10.4103/0378-6323.198453. PMID: 29035287
Localized and systemic scleroderma.
Hawk A, English JC 3rd
Semin Cutan Med Surg 2001 Mar;20(1):27-37. doi: 10.1053/sder.2001.23093. PMID: 11308134
Cardiac involvement in limited systemic sclerosis: non-invasive assessment in asymptomatic patients.
Handa R, Gupta K, Malhotra A, Jain P, Kamath PK, Aggarwal P, Dwivedi SN, Wali JP
Clin Rheumatol 1999;18(2):136-9. doi: 10.1007/s100670050071. PMID: 10357119

Clinical prediction guides

Native T1 mapping in early diffuse and limited systemic sclerosis, and its association with diastolic function.
Purevsuren M, Uehara M, Ishizuka M, Suzuki Y, Shimbo M, Kakuda N, Ishii S, Sumida H, Miyazaki M, Yamashita T, Yoshizaki A, Asano Y, Sato S, Hatano M, Komuro I
J Cardiol 2023 Aug;82(2):100-107. Epub 2023 Mar 13 doi: 10.1016/j.jjcc.2023.03.003. PMID: 36921691
Periodontal disease and its association to endothelial dysfunction and clinical changes in limited systemic sclerosis: A case-control study.
Jud P, Wimmer G, Meinitzer A, Strohmaier H, Schwantzer G, Moazedi-Fürst F, Schweiger L, Brodmann M, Hafner F, Arefnia B
J Periodontal Res 2023 Jun;58(3):621-633. Epub 2023 Mar 15 doi: 10.1111/jre.13111. PMID: 36919705
Serum microRNAs in Systemic Sclerosis, Associations with Digital Vasculopathy and Lung Involvement.
Wajda A, Walczyk M, Dudek E, Stypińska B, Lewandowska A, Romanowska-Próchnicka K, Chojnowski M, Olesińska M, Paradowska-Gorycka A
Int J Mol Sci 2022 Sep 14;23(18) doi: 10.3390/ijms231810731. PMID: 36142646Free PMC Article
Evaluation of endothelial dysfunction and clinical events in patients with early-stage vasculopathy in limited systemic sclerosis.
Jud P, Meinitzer A, Strohmaier H, Schwantzer G, Foris V, Kovacs G, Avian A, Odler B, Moazedi-Fürst F, Brodmann M, Hafner F
Clin Exp Rheumatol 2021 Jul-Aug;39 Suppl 131(4):57-65. Epub 2021 Jul 28 doi: 10.55563/clinexprheumatol/243mpp. PMID: 34323684
L-selectin and skin damage in systemic sclerosis.
Dunne JV, van Eeden SF, Keen KJ
PLoS One 2012;7(9):e44814. Epub 2012 Sep 13 doi: 10.1371/journal.pone.0044814. PMID: 23028631Free PMC Article

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