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Anti-PM-Scl antibody positivity

MedGen UID:
1783571
•Concept ID:
C5539685
•
Laboratory or Test Result
Synonym: Anti-PM/Scl antibody positivity
 
HPO: HP:0033560

Definition

Anti-PM-Scl antibodies target components of RNA-processing exosome complex in the nucleolus. There are ten proteins in this complex and antibodies to eight of them are found at varying frequencies; PM/Scl-100, PM/Scl-75, hRrp4, hRrp42, hRrp46, hCs14, hRrp41, and hRrp40. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAnti-PM-Scl antibody positivity

Professional guidelines

PubMed

Interstitial lung disease and myositis-specific and associated autoantibodies: Clinical manifestations, survival and the performance of the new ATS/ERS criteria for interstitial pneumonia with autoimmune features (IPAF).
Mejía M, Herrera-Bringas D, Pérez-Román DI, Rivero H, Mateos-Toledo H, Castorena-García P, Figueroa JE, Rojas-Serrano J
Respir Med 2017 Feb;123:79-86. Epub 2016 Dec 23 doi: 10.1016/j.rmed.2016.12.014. PMID: 28137500
Calcinosis in poly-dermatomyositis: clinical and laboratory predictors and treatment options.
Fredi M, Bartoli F, Cavazzana I, Ceribelli A, Carabellese N, Tincani A, Satoh M, Franceschini F
Clin Exp Rheumatol 2017 Mar-Apr;35(2):303-308. Epub 2016 Nov 14 PMID: 27908312
Calcinosis: pathophysiology and management.
Valenzuela A, Chung L
Curr Opin Rheumatol 2015 Nov;27(6):542-8. doi: 10.1097/BOR.0000000000000220. PMID: 26352733

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