PROGRESSIVE PSEUDORHEUMATOID DYSPLASIA - REPORT OF A FAMILY AND REVIEW

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.