Skip to main content
SpringerLink
Log in

Progressive pseudorheumatoid arthropathy of childhood

  • Clinical Brief
  • Published:
The Indian Journal of Pediatrics Aims and scope Submit manuscript

Abstract

Progressive pseudorheumatoid arthropathy of childhood (PPAC) described by Sprangeret al is a rare autosomal recessive disorder.

An 11 year-old girl was diagnosed as having PPAC at Ege University, Faculty of Medicine, Department of Paediatrics. Her complaints of painful joints, difficulty in walking and joint contractures began at the age of 3 years and she was treated for juvenile rheumatoid arthritis for 8 years. Her symptoms did not respond to nonsteroid anti-inflammatory treatment. During her last hospitalisation period, she was reinvestigated. Radiological examination showed spondyloepiphyseal dysplasia, severe acetabular irregularity and osteoporosis. All the laboratory test results for rheumatoid arthritis were negative. The clinical and radiological findings of the patient are illustrated.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

References

  1. Al-Awadi SA, Farag Tl, Naguib Ket al. Spondyloephyseal dysplasia with progressive arthropathy.J Med Genet 1984; 21: 193–196.

    Article  PubMed  CAS  Google Scholar 

  2. Archik SG, Kamat RD. Progressive pseudorheumatoid chondrodysplasia simulating juvenile rheumatoid arthritis.Indian J Pediatr 1990; 57: 785–788.

    Article  PubMed  CAS  Google Scholar 

  3. Mc Kusick VA.Mendelian inheritance in Man. 10th ed. Baltimore and London: The Johns Hopkins University Press. 1992: 1232.

    Google Scholar 

  4. Perri G. The radiological features of a new bone dysplasia.Pediatr Radiol 1981; 11: 109–113.

    Article  PubMed  CAS  Google Scholar 

  5. Spranger J, Albert J, Schilling F, Bartsocas C. Progressive pseudoromatoid arthritis of Childhood (PPAC): a hereditary disorder simulating rheumatoid arthritis.Europ J Pediatr 1993: 140: 34–40.

    Article  Google Scholar 

  6. Winter RM, Baraitser M.Multiple Congentital Anomalies. 1st ed. Cambridge, 1991: 504.

  7. Wynne-Davies R, Hall C, Ansell BM. Spondylo-epiphyseal dysplasia tarda with progressive arthropathy. A ‘new’ disorder of autosomal recessive inheritance.J Bone Joint Surg 1982; 64: 442–445.

    CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Faculty of Medicine, Department of Paediatrics, Ege University, 35100, Bornova-Izmir, Turkey

    Ozgur Cogulu MD (Paediatrician), Cihangir Ozkinay & Helda Binnur Deveci

  2. Department of Medical Biology, Ege University Faculty of Medicine, 35100, Bornova-Izmir, Turkey

    Ferda Ozkinay & Gül Sapmaz

  3. Department of Radiology, Ege University, Faculty of Medicine, 35100, Bornova-Izmir, Turkey

    Osman Yalman

Authors
  1. Ozgur Cogulu MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Ferda Ozkinay
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Cihangir Ozkinay
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Gül Sapmaz
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Osman Yalman
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Helda Binnur Deveci
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Cogulu, O., Ozkinay, F., Ozkinay, C. et al. Progressive pseudorheumatoid arthropathy of childhood. Indian J Pediatr 66, 455–460 (1999). https://doi.org/10.1007/BF02845540

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF02845540

Key words

Search

Navigation