Jaccoud arthropathy

Jaccoud’s arthropathy is a chronic and non-erosive deforming arthropathy, usually affecting the hands characterized by ulnar deviation of the second to fifth fingers and subluxation of the metacarpophalangeal joints ¹. Joint subluxations are voluntarily correctable by the patients. Toes may also be affected. Jaccoud’s arthropathy may be seen in association with several rheumatic and non-rheumatic disorders, such as systemic lupus erythematosus (SLE) ², recurrent rheumatic fever ³, other connective tissue diseases ⁴, psoriatic arthritis ⁵, inflammatory bowel disease arthritis ⁶ and malignancy ⁷. It has been reported that about 5% of patients with systemic lupus erythematosus (SLE) have Jaccoud’s arthropathy ⁸.

Jaccoud’s arthropathy is characterized by the presence of joint deformities such as “swan neck,” ulnar deviation and “Z-thumb” resembling rheumatoid arthritis (RA) but that are passively correctable and without bone erosion on plain radiographs ⁹.

On imaging, ulnar deviation of the second to fifth fingers and subluxation of the metacarpophalangeal joints is seen.

Definite criteria for the diagnosis of Jaccoud’s arthropathy have not yet been established, and the pathogenesis is understood incompletely ¹⁰. However, an inflammatory process localized in the periarticular soft tissues (including the tendons and joint capsules) has been indicated as the primary cause ².

Figure 1. Jaccoud’s arthritis

Jaccoud arthropathy causes

Jaccoud’s arthropathy was initially described as a complication of recurrent rheumatic fever ³. Jaccoud’s arthropathy may be seen in association with several rheumatic and non-rheumatic disorders, such as systemic lupus erythematosus (SLE), recurrent rheumatic fever, other connective tissue diseases ⁴, psoriatic arthritis ⁵, inflammatory bowel disease arthritis ⁶ and malignancy ⁷. It has been reported that about 5% of patients with systemic lupus erythematosus (SLE) have Jaccoud’s arthropathy ⁸. Jaccoud’s arthropathy has also been reported in patients without active arthritis ¹¹. Jaccoud arthropathy is thought to be related to ligamentous laxity ¹².

Jaccoud’s arthropathy pathophysiology is poorly known but involves periarticular structures such as tendons and the joint capsule.

Jaccoud arthropathy symptoms

Common presentation of Jaccoud’s arthropathy is involvement of the wrist, metacarpophalangeal joints, and joints of the feet. Jaccoud arthropathy is characterized by marked ulnar subluxation and deviation at the metacarpophalangeal joints that is correctable or reducible with physical manipulation ¹². Typical features include characteristic joint deviation with metacarpophalangeal subluxation, ulnar deviation, swan neck deformity of the fingers, Z deformity of both thumbs and hook formation, and alignment disorders without noticeable erosions on radiographs ¹³. Deformity of the long bones is generally rare.

In contrast to rheumatoid arthritis, the gradual development of deformity of the hands or feet occurs without symptoms, with little evidence of active synovitis, and with the maintenance of functional capacity ¹⁴.

Jaccoud arthropathy diagnosis

Jaccoud’s arthropathy has been described as severe joint deformity with no or minimal osseous destruction ¹⁵. Although it has been reported that bone erosions do not occur in Jaccoud’s arthropathy, recent findings from ultrasound and magnetic resonance imaging have shown otherwise ¹⁶.

Radiologically, the earliest bone change is erosion of the metacarpal head on the palmar and radial part of their circumference in an anteroposterior projection producing a hook like erosion ¹⁷. “Hook” erosions may occur on the radial palmar aspect of the metacarpal heads and can be differentiated from the marginal erosions of rheumatoid arthritis ¹⁸. Jaccoud’s arthropathy is not associated with swelling, obliteration of joint spaces, or destruction of articular surfaces ¹¹. Erythrocyte sedimentation rate (ESR) and rheumatoid factor tests are generally negative in patients with Jaccoud’s arthropathy. Although the hands are the most common site of involvement, this condition has also been reported to involve the feet ¹¹.

One important differential diagnosis that should be considered is Madelung’s deformity. Madelung’s deformity is characterized by ulnar and dorsal curvature of the distal radius due to deficient growth of the volar and ulnar aspect of the distal radial physis, increased inclination of the distal radial joint surface, triangulation of the corpus with proximal and volar migration, and prominent dorsal subluxation of the ulnar head ¹⁹. Madelung’s deformity usually presents in late childhood or adolescence, is more common in females, and is usually bilateral ²⁰. A number of anatomical changes occur in Madelung deformity, which result in biomechanical alterations leading to a decreased range of movement, decreased grip strength, and often pain ²⁰. In most patients, there is a hereditary component. In either case, presentation of
Jaccoud’s arthropathy is expected to be similar.

Jaccoud arthropathy treatment

Jaccoud’s arthropathy optimal therapeutic approach remains to be established. Presently, the therapy for Jaccoud’s arthropathy is conservative and based on the use of non-hormonal anti-inflammatory drugs, low doses of corticosteroids, methotrexate and antimalarials ²¹. The role of surgery through either the realignment of soft tissue around the joint–or more aggressive procedures such as arthrodesis, silastic implant and arthroplasty–needs to be proven ²¹. No benefit from either prednisone or a combination of prednisone and methotrexate was obtained ²².

References

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