progressive pseudorheumatoid arthropathy of childhood
MeSH: C535387ORPHA: 1159
Overview
Progressive pseudorheumatoid arthropathy (dysplasia) of childhood (PPAC; PPD) presents as spondyloepiphyseal dysplasia (SED) tarda with progressive arthropathy and is described as a specific autosomal recessive subtype of SED
Available Treatments (0)
No treatments linked yet
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Clinical Presentation
Signs and symptoms associated with progressive pseudorheumatoid arthropathy of childhood, sourced from HPO and Orphanet clinical annotations.
PlatyspondylyJoint swellingWaddling gaitSpondyloepiphyseal dysplasiaIrregular vertebral endplatesNarrow small joints of the handPolyarticular arthropathyWrist swellingMuscle weaknessLimitation of joint mobilityAbnormal hip joint morphologyScoliosisAbnormality of the kneeArthralgiaEasy fatigabilityThoracolumbar kyphoscoliosisShort statureIrregularity of vertebral bodiesHyperconvex vertebral body endplatesAbnormality of hand joint mobilityVertebral wedgingJoint contracture of the handAbnormality of the elbowInfancy onset short-trunk short statureCamptodactylyAbnormality of the neckCoxa varaGenu valgumAbnormal ilium morphologyGenu varumAbnormal shoulder morphologyHyperlordosisFlat capital femoral epiphysisEnlargement of the proximal femoral epiphysisBeaking of vertebral bodiesEnlarged interphalangeal jointsBroad femoral neckIrregular acetabular roofEnlarged epiphysesPeriarticular calcificationGeneralized osteoporosisShort femoral neck
Classification & Codes
MeSH Code
C535387Orphanet Code
ORPHA:1159progressive pseudorheumatoid arthropathy of childhood
| MeSH | C535387 |
| Orphanet | ORPHA:1159 |
| Treatments | 0 drug(s) |
| Symptoms on record | 42 signs |
| Status | published |
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO