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Arthropathy, progressive pseudorheumatoid, of childhood MeSH Supplementary Concept Data 2024


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 Number
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 Preferred
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