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Systemic sclerosis sine scleroderma

MedGen UID:
220934
•Concept ID:
C1290138
•
Disease or Syndrome
Synonym: Limited systemic sclerosis
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  
  • CROGVSystemic sclerosis sine scleroderma

Professional guidelines

PubMed

Prevalence of the limited vs. extensive scleroderma-related interstitial lung disease at the time of diagnosis of SSc-ILD based on Goh et al. criteria. Systematic review and meta-analysis.
Rubio-Rivas M, Pestaña-Fernández M
Rev Clin Esp (Barc) 2024 Apr;224(4):189-196. Epub 2024 Feb 20 doi: 10.1016/j.rceng.2024.02.008. PMID: 38387499
French recommendations for the management of systemic sclerosis.
Hachulla E, Agard C, Allanore Y, Avouac J, Bader-Meunier B, Belot A, Berezne A, Bouthors AS, Condette-Wojtasik G, Constans J, De Groote P, Diot E, Dumas F, Jego P, Joly F, Launay D, Le Guern V, Le Quintrec JS, Lescaille G, Meune C, Moulin B, Nguyen C, Omeish N, Pene F, Richard MA, Rochefort J, Roren A, Sitbon O, Sobanski V, Truchetet ME, Mouthon L; Collaborators
Orphanet J Rare Dis 2021 Jul 26;16(Suppl 2):322. doi: 10.1186/s13023-021-01844-y. PMID: 34304732Free PMC Article
Applying the ACR/EULAR Systemic Sclerosis Classification Criteria to the Spanish Scleroderma Registry Cohort.
Sáez-Comet L, Simeón-Aznar CP, Pérez-Conesa M, Vallejo-Rodríguez C, Tolosa-Vilella C, Iniesta-Arandia N, Colunga-Argüelles D, Egurbide-Arberas MV, Ortego-Centeno N, Vargas-Hitos JA, Freire-Dapena M, Rubio-Rivas M, Ríos-Blanco JJ, Trapiella-Martínez L, Fonollosa-Pla V; RESCLE Investigators
J Rheumatol 2015 Dec;42(12):2327-31. Epub 2015 Oct 15 doi: 10.3899/jrheum.150144. PMID: 26472418

Recent clinical studies

Etiology

Systemic sclerosis sine scleroderma is more aggressive in children than in adults.
Tirelli F, Zanatta E, Moccaldi B, Binda M, Martini G, Giraudo C, Vittadello F, Meneghel A, Zulian F
Rheumatology (Oxford) 2024 Sep 1;63(SI2):SI215-SI218. doi: 10.1093/rheumatology/keae304. PMID: 38775723
Systemic sclerosis sine scleroderma in children.
Zulian F, Lanzoni G, Castaldi B, Meneghel A, Tirelli F, Zanatta E, Martini G
Rheumatology (Oxford) 2022 May 30;61(6):2555-2562. doi: 10.1093/rheumatology/keab738. PMID: 34605913Free PMC Article
Systemic sclerosis sine scleroderma.
Kucharz EJ, Kopeć-Mędrek M
Adv Clin Exp Med 2017 Aug;26(5):875-880. doi: 10.17219/acem/64334. PMID: 29068586
Scleroderma: nomenclature, etiology, pathogenesis, prognosis, and treatments: facts and controversies.
Fett N
Clin Dermatol 2013 Jul-Aug;31(4):432-437. doi: 10.1016/j.clindermatol.2013.01.010. PMID: 23806160
Systemic sclerosis sine scleroderma: is it always the same disease? Report of three patients and discussion.
Slobodin G, Rosner I, Rozenbaum M, Naschitz JE, Zuckerman E, Yeshurun D
Rheumatol Int 2002 Aug;22(4):170-2. Epub 2002 Jul 2 doi: 10.1007/s00296-002-0222-y. PMID: 12172958

Diagnosis

Cutaneous Manifestations, Clinical Characteristics, and Prognosis of Patients With Systemic Sclerosis Sine Scleroderma: Data From the International EUSTAR Database.
Lescoat A, Huang S, Carreira PE, Siegert E, de Vries-Bouwstra J, Distler JHW, Smith V, Del Galdo F, Anic B, Damjanov N, Rednic S, Ribi C, Bancel DF, Hoffmann-Vold AM, Gabrielli A, Distler O, Khanna D, Allanore Y; EUSTAR collaborators
JAMA Dermatol 2023 Aug 1;159(8):837-847. doi: 10.1001/jamadermatol.2023.1729. PMID: 37378994Free PMC Article
Systemic sclerosis sine scleroderma in children.
Zulian F, Lanzoni G, Castaldi B, Meneghel A, Tirelli F, Zanatta E, Martini G
Rheumatology (Oxford) 2022 May 30;61(6):2555-2562. doi: 10.1093/rheumatology/keab738. PMID: 34605913Free PMC Article
Pauci-Immune Necrotizing Glomerulonephritis as a Manifestation of Systemic Sclerosis Sine Scleroderma.
Shabaka A, Gatius S, Vian J, Pascual A, Rodriguez-Moreno A
J Clin Rheumatol 2021 Jan 1;27(1):e20-e21. doi: 10.1097/RHU.0000000000001208. PMID: 31789998
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
Systemic sclerosis sine scleroderma.
Kucharz EJ, Kopeć-Mędrek M
Adv Clin Exp Med 2017 Aug;26(5):875-880. doi: 10.17219/acem/64334. PMID: 29068586

Therapy

Immunotactoid glomerulopathy - an enigmatic case in the setting of nodal marginal zone lymphoma and systemic sclerosis sine scleroderma.
Mohamed MW, Al-Hammadi M, Hussein AM, Alarab D, Albreak HA, Tungekar MF, Dandi B
BMC Nephrol 2022 Mar 15;23(1):103. doi: 10.1186/s12882-022-02730-w. PMID: 35291980Free PMC Article
Digital Ulcers, Systemic Sclerosis Sine Scleroderma and Paraneoplastic Phenomena Responding to Bosentan Therapy.
Sharabi I, Tanay A, Zandman-Goddard G
Isr Med Assoc J 2015 Feb;17(2):126-7. PMID: 26223094
Systemic sclerosis sine scleroderma: a case report of anterior uveitis.
Borges T, Vilaça J, Ferreira S, Chora I, Silva S, Dias C
Reumatismo 2015 Jun 30;67(1):21-5. doi: 10.4081/reumatismo.2015.818. PMID: 26150271
Scleroderma: nomenclature, etiology, pathogenesis, prognosis, and treatments: facts and controversies.
Fett N
Clin Dermatol 2013 Jul-Aug;31(4):432-437. doi: 10.1016/j.clindermatol.2013.01.010. PMID: 23806160
Non-Hodgkin's lymphoma in systemic sclerosis: case and literature review.
Vettori S, Staibano S, Mascolo M, Ilardi G, Valentini G
Clin Rheumatol 2010 Jan;29(1):1-6. Epub 2009 Oct 7 doi: 10.1007/s10067-009-1286-9. PMID: 19809778

Prognosis

Systemic Sclerosis Sine Scleroderma: A Time of Reappraisal.
Makris A, Panagiotopoulos A, Distler O, Sfikakis PP
J Rheumatol 2024 Nov 1;51(11):1060-1068. doi: 10.3899/jrheum.2023-1113. PMID: 38950948
Systemic sclerosis sine scleroderma is more aggressive in children than in adults.
Tirelli F, Zanatta E, Moccaldi B, Binda M, Martini G, Giraudo C, Vittadello F, Meneghel A, Zulian F
Rheumatology (Oxford) 2024 Sep 1;63(SI2):SI215-SI218. doi: 10.1093/rheumatology/keae304. PMID: 38775723
Cutaneous Manifestations, Clinical Characteristics, and Prognosis of Patients With Systemic Sclerosis Sine Scleroderma: Data From the International EUSTAR Database.
Lescoat A, Huang S, Carreira PE, Siegert E, de Vries-Bouwstra J, Distler JHW, Smith V, Del Galdo F, Anic B, Damjanov N, Rednic S, Ribi C, Bancel DF, Hoffmann-Vold AM, Gabrielli A, Distler O, Khanna D, Allanore Y; EUSTAR collaborators
JAMA Dermatol 2023 Aug 1;159(8):837-847. doi: 10.1001/jamadermatol.2023.1729. PMID: 37378994Free PMC Article
Systemic sclerosis sine scleroderma in children.
Zulian F, Lanzoni G, Castaldi B, Meneghel A, Tirelli F, Zanatta E, Martini G
Rheumatology (Oxford) 2022 May 30;61(6):2555-2562. doi: 10.1093/rheumatology/keab738. PMID: 34605913Free PMC Article
Systemic sclerosis sine scleroderma.
Kucharz EJ, Kopeć-Mędrek M
Adv Clin Exp Med 2017 Aug;26(5):875-880. doi: 10.17219/acem/64334. PMID: 29068586

Clinical prediction guides

Cutaneous Manifestations, Clinical Characteristics, and Prognosis of Patients With Systemic Sclerosis Sine Scleroderma: Data From the International EUSTAR Database.
Lescoat A, Huang S, Carreira PE, Siegert E, de Vries-Bouwstra J, Distler JHW, Smith V, Del Galdo F, Anic B, Damjanov N, Rednic S, Ribi C, Bancel DF, Hoffmann-Vold AM, Gabrielli A, Distler O, Khanna D, Allanore Y; EUSTAR collaborators
JAMA Dermatol 2023 Aug 1;159(8):837-847. doi: 10.1001/jamadermatol.2023.1729. PMID: 37378994Free PMC Article
Systemic sclerosis sine scleroderma: a multicenter study of 1417 subjects.
Diab S, Dostrovsky N, Hudson M, Tatibouet S, Fritzler MJ, Baron M, Khalidi N; Canadian Scleroderma Research Group
J Rheumatol 2014 Nov;41(11):2179-85. Epub 2014 Oct 1 doi: 10.3899/jrheum.140236. PMID: 25274885
Systemic sclerosis sine scleroderma: demographic, clinical, and serologic features and survival in forty-eight patients.
Poormoghim H, Lucas M, Fertig N, Medsger TA Jr
Arthritis Rheum 2000 Feb;43(2):444-51. doi: 10.1002/1529-0131(200002)43:2<444::AID-ANR27>3.0.CO;2-G. PMID: 10693887

Recent systematic reviews

Systemic Sclerosis Sine Scleroderma: A Time of Reappraisal.
Makris A, Panagiotopoulos A, Distler O, Sfikakis PP
J Rheumatol 2024 Nov 1;51(11):1060-1068. doi: 10.3899/jrheum.2023-1113. PMID: 38950948
COVID-19 and exacerbation of dermatological diseases: A review of the available literature.
Aram K, Patil A, Goldust M, Rajabi F
Dermatol Ther 2021 Nov;34(6):e15113. Epub 2021 Sep 7 doi: 10.1111/dth.15113. PMID: 34453380Free PMC Article

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