Severe Jaccoud’s arthropathy in systemic lupus erythematosus

Abstract

Jaccoud's arthropathy (JA) is a clinical situation nowadays present mostly in systemic lupus erythematosus (SLE). It 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. From our cohort of SLE patients with JA, we selected a subgroup with a more severe form of this arthropathy and looked at their clinical and laboratory profile as well as studied the magnetic resonance imaging (MRI) findings or ultrasound (US) obtained from the hand with most evident deformities. Seven SLE patients with a severe form of JA were identified. All seven patients have "swan neck," ulnar deviation and "Z-thumb" deformities. Two out of seven had "mutilans-type JA" and four had fixed deformities in the metacarpophalangeal (MCP) joints. The MRI of the hand with more evident deformity clinically performed in six cases and US performed in one case showed mild synovitis in five and moderate synovitis in two patients, mild flexor tenosynovitis in six and severe tenosynovitis in one. Only two small bone erosions were observed in the second and third MCP joints of one patient with moderate synovitis. Severe JA compromises the functional capacity of the joints and imposes the risk of misdiagnosis of RA. With the improvement of the survival rate of SLE and the lack of specific prophylactic or therapeutical measures for JA, it is reasonable to assume that more and more cases of severe JA are going to be identified.

Case report: Joint deformity associated with systemic lupus erythematosus

“…Our report confirmed that although bone erosion can be detected more accurately using ultrasound and MRI than with radiography, there are still some patients with JA that show no bone erosion. In addition to our report, there have been several studies describing JA patients without bone erosion based on MRI findings 16–19 . This may indicate that joint deformities with or without bone erosion are two different types of diseases.…”

Jaccoud-type lupus arthropathy

“…However, the spectrum of deformity in JA is very wide, varying from a simple 'reducible' ulnar deviation to a more complicated 'severe' or 'mutilans-type' deformity with fixed joints and subluxations. 6 Thus, a modified set of criteria for JA in SLE contemplating 'severe' forms of the disease has been recently published. 31 The importance of having classification criteria for JA lies in two points: a) standardize patients for future clinical trials; b) distinguish SLE cases with JA from those with RA, as the therapeutic approach is different.…”

“…This is probably secondary to soft tissue fibrosis, disuse of the involved joints and muscle hypotrophy. 6 Curiously, based on Bywaters' report in 1950, the original patient described by Jaccoud was probably an example of the severe form of this arthropathy in whom 'deformity of the hands developed for the first time in the third month of the fifth attack and was initially correctable, only later becoming permanent'. 20 Other authors have confirmed the changing character of the deformities from reducible to fixed.…”

Jaccoud-type lupus arthropathy: practical classification criteria

“…Although a 'reducible' pattern of arthropathy is observed in the majority of JA cases, in most advanced cases, the joints may become fixed, sometimes referred as to 'severe JA', 2 which is clinically difficult to distinguish from RA. In such situations, misdiagnosis is common, with dangerous consequences.…”