Progressive pseudorheumatoid dysplasia is an inherited skeletal dyspla
sia with radiographic changes notably in the spine, similar to spondyl
oepiphyseal dysplasia tarda. There is also articular cartilage involve
ment which gives it some clinical resemblance to rheumatoid arthritis.
We report here on six subjects from one inbred family from Jordan. Ba
sed on previously published reports and this one, we review the clinic
al and radiological features and discuss the genetics and differential
diagnosis of the disorder. We suggest the addition of the word ((spon
dyloepiphyseal)) to the name adopted by the International Working Grou
p on Constitutional Diseases of Bone, to become ''progressive pseudorh
eumatoid spondyloepiphyseal dysplasia'', We also speculate on candidat
e genes for this disorder.