Early-onset primary osteoarthritis and mild chondrodysplasia : radiographic and pathologic studies with an analysis of cartilage proteoglycans

Three generations of a nonconsanguineous family with premature onset of primary (idiopathic) osteoarthritis (OA) were studied for clues to the etiopathogenesis of their disorder. Articular symptoms began in their second and third decades of life and involved multiple joints, both typical and atypica...

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Bibliographic Details
Published in:Arthritis and rheumatism 1990-05, Vol.33 (5), p.674-684
Main Authors: KATZENSTEIN, P. L, MALEMUD, C. J, PATHRIA, M. N, CARTER, J. R, SHEON, R. P, MOSKOWITZ, R. W
Format: Article
Language:English
Subjects:
Biological and medical sciences
Cartilage - metabolism
Cartilage Diseases - diagnostic imaging
Cartilage Diseases - metabolism
Cartilage Diseases - pathology
Diseases of the osteoarticular system
Femur Head - pathology
Humans
Medical sciences
Osteoarthritis
Osteoarthritis - diagnostic imaging
Osteoarthritis - metabolism
Osteoarthritis - pathology
Pedigree
Proteoglycans - metabolism
Radiography
Spinal Diseases - diagnostic imaging
Spinal Diseases - metabolism
Spinal Diseases - pathology
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Summary:Three generations of a nonconsanguineous family with premature onset of primary (idiopathic) osteoarthritis (OA) were studied for clues to the etiopathogenesis of their disorder. Articular symptoms began in their second and third decades of life and involved multiple joints, both typical and atypical for primary OA. Radiographs of the majority of involved peripheral joints showed abnormalities typical of primary OA. Evidence of chondrodysplasia was found in the spines. Pathologic examination of femoral heads obtained at total hip arthroplasty from 3 affected family members showed moderate to severe OA. Articular cartilage proteoglycans from these specimens were evaluated for aggregatability with hyaluronic acid, levels of chondroitin sulfate and keratan sulfate, and core protein structure. The results from each patient's specimen differed from the results of the other specimens. We conclude that this family's disorder, primary OA associated with a mild chondrodysplasia, was a late-onset overlap form of an epiphyseal dysplasia, that a defect common to hyaline articular and physeal cartilage was primary, and that a single structural proteoglycan abnormality was not likely to be the underlying cause.
ISSN:0004-3591
1529-0131