Presentation

A 30-year-old woman was referred to our rheumatology unit with the diagnosis of rhupus. She had a treatment compliance problem and did not use any medication for about 10 years after pregnancy. Physical examination revealed tenderness in the metacarpophalangeal joints, reducible Z-deformities of her thumbs, and swan neck deformities of her fingers (Fig. 1a). X-Ray of both the hands showed flexion deformities at the metacarpophalangeal and distal interphalangeal joints and a hyperextension deformity at the proximal interphalangeal joints (Fig. 1b). Computerized tomography (CT) of the hands documented cortical erosions and multiple subchondral degenerative cystic alterations of the carpal bones (Fig. 1c). Her erythrocyte sedimentation rate (56 mm/h) and C-reactive protein level (12.7 mg/L) were elevated. Autoimmune serology showed a strongly positive ANA (1:640, homogeneous pattern), positive anti-dsDNA (55 IU/mL), and negative extractable nuclear antigens. Complements were also normal. Both rheumatoid factor (RF) (137.4 IU/mL) and anti-cyclic citrullinated peptide antibody (110 U/mL) tests were positive.

Fig.1
figure 1

a Photograph and b radiograph of both the hands of our patient showing flexion deformities at the metacarpophalangeal and distal interphalangeal joints and a hyperextension deformity at the proximal interphalangeal joints. c Coronal CT with bone window of the wrist demonstrates cortical erosions (arrow) and multiple subchondral degenerative cystic alterations of the carpal bones (arrowhead). Narrowing of the carpomatecarpal joints and sclerosis at joint surfaces are appreciable too

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Discussion

Jaccoud’s arthropathy (JA), a correctable deformity, was originally described in cases with rheumatic fever, but is mainly seen in patients with systemic lupus erythematosus [1]. Furthermore, JA may be associated with Sjogren’s syndrome, dermatomyositis, scleroderma, sarcoidosis, HIV infection, psoriatic arthritis, inflammatory bowel disease, malignancy, and aging. We present the JA in a rhupus patient with erosive and cystic changes in the hand joints. Although JA is considered a non-erosive arthropathy, it has been reported that erosions that can not be detected in direct radiography are revealed by imaging methods such as ultrasound, CT, and magnetic resonance imaging [2]. The effects of factors such as RF positivity, elevated acute phase reactants, disease duration, arthritis duration, and presence of anti-dsDNA antibodies have been shown in JA development [3]. It has been observed that patients with erosion tend to have a higher JA index score [4].