MeSH Browser

MeSH Supplementary: Arthropathy, progressive pseudorheumatoid, of childhood
Unique ID: C535387
RDF Unique Identifier: http://id.nlm.nih.gov/mesh/C535387
Entry Term(s): Progressive pseudorheumatoid arthropathy of childhood; Progressive pseudorheumatoid dysplasia; SEDT-PA; Spondyloepiphyseal dysplasia tarda with progressive arthropathy
Registry Numbers: 0
Heading Mapped to: Joint Diseases / congenital
Frequency: 48
Note: A hereditary autosomal recessive arthropathy with onset typically by 3 years of age. It is characterized by joint stiffness that first affects the hips, swelling of the fingers, and morning stiffness and decreased mobility of the cervical spine similar to RHEUMATOID ARTHRITIS. However, normal sedimentation rate, negative RHEUMATOID FACTOR tests, and histologically normal SYNOIVUM exclude rheumatoid disease. Patients do not respond to snti-rheumatic drugs and also exhibit radiologic changes indicative of bone dysplasia. Mutations in the WISP3 gene have been identified. OMIM: 208230
Date of Entry: 2010/08/25
Revision Date: 2019/06/19

Arthropathy, progressive pseudorheumatoid, of childhood MeSH Supplementary Concept Data 2025