Progressive Pseudorheumatoid Arthropathy

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A case of progressive pseudorheumatoid arthropathy of `childhood' with the

diagnosis delayed to the fifth decade

http://www.ingentaconnect.com/content/bsc/ijcp/2006/00000060/00000010/art00030

Authors: CEFLE, A.1; CEFLE, K.2; TUNACI, M.3; OZTURK, S.2; PALANDUZ, S.2

Source: International Journal of Clinical Practice, Volume 60, Number 10,

October 2006, pp. 1306-1309(4)

Abstract: Summary

Progressive pseudorheumatoid arthropathy of childhood (PPAC) is a rare

single gene disorder which is frequently misdiagnosed as juvenile rheumatoid

arthritis.

It is characterised with arthralgia, joint contractures, bony swelling of

metacarpophalangeal and interphalangeal joints and platyspondyly.

Clinical and laboratory signs of joint inflammation such as synovitis, a

high erythrocyte sedimentation rate and an elevated C-reactive protein level

are usually absent.

Although the disease begins early in life (usually between 3 and 8 years of

age), the diagnosis may be delayed.

In the present case report, we describe a male patient diagnosed with PPAC

at the age of 46 years, although he had been exhibiting the typical

radiological and clinical features of the disease since the age of 7 years.

Document Type: Research article

DOI: 10.1111/j.1742-1241.2005.00662.x

Affiliations: 1: Medical Faculty, Department of Internal Medicine, Division

of Rheumatology, Kocaeli, Istanbul University 2: Istanbul Medical Faculty,

Department of Internal Medicine, Division of Medical Genetics, Istanbul

University 3: Istanbul Medical Faculty, Department of Radiology, Istanbul,

Turkey

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