Deforming arthropathy or lupus and rhupus hands in systemic lupus erythematosus

Abstract

Objective: Although deforming arthropathy in systemic lupus erythematosus (SLE) is characterised by a number of manifestations, definitive criteria for the different forms have not yet been established. To define deforming arthropathy and its different types a study was undertaken of 176 SLE patients.

Methods: Using as criterion any deviation from any of the metacarpus finger axes 17 patients (16 women, one man) were identified with clinical deforming arthropathy. These patients were evaluated according to a standardised protocol that covered all known characteristics of deforming arthropathy. By means of "Jaccoud's arthropathy index" three different forms were identified.

Results: Three patients had an erosive form of deforming arthropathy (or rhupus hand) such as those seen in frank rheumatoid arthritis (RA), eight patients were identified as having Jaccoud's arthropathy (or lupus hand), and the remaining six patients had mild deforming arthropathy. Jaccoud's arthropathy is characterised by severe deformation of the hands (ulnar deviation, swan neck deformities, and Z deformity of the thumb) and feet with multiple non-erosive subluxations, mild aching and little or no evidence of synovitis. All patients, but one, fulfilled just four criteria of the ACR classification and joint symptoms were always found to precede the diagnosis of SLE. Furthermore a remarkable association of Jaccoud's arthropathy with fetal loss, thrombosis--both venous and arterial--and the presence of antiphospholipid antibodies was found.

Conclusions: These data suggest that Jaccoud's arthropathy represents a subset of SLE. Subdivision of deforming arthropathy into several clinical forms can facilitate the clinical management of this disorder.

Figure 1

Flow diagram by which the diagnosis of the three different forms of deforming arthropathy is reached.

Figure 2

Radiograph of erosive arthropathy (rhupus hand) showing joint space narrowing and rheumatoid-like bone erosions in the intercarpal and radiocarpal joints. Mild erosive damage of some MCP joints.

Figure 3

(A) Characteristic joint deviation with MCP subluxation, ulnar deviation, swan neck deformity of the fingers, and Z deformity of both thumbs in Jaccoud's arthropathy of the hands (lupus hand). (B) Radiograph of same hands showing hook formation (arrow) and alignment disorders without noticeable erosions.

Figure 4

Radiograph of male patient with Jaccoud's arthropathy showing scapholunate dissociation leading to carpal collapse and mild ulnar translocation of the wrist.