A 23-year-old woman with a 7-year history of systemic lupus erythematosus (SLE) complicated by lupus nephritis presented with abdominal distension. She was found to have worsening renal function with nephritic range proteinuria and ascites. Musculoskeletal examination revealed Z-shaped thumbs, swan neck deformities of the distal interphalangeal joints, and reduced flexion at the metacarpophalangeal (MCP) joints, suggestive of Jaccoud’s arthropathy (JA) (Fig. 1). Radiographic films of bilateral hands confirmed non-erosive disease (Fig. 2).

Fig. 1
figure 1

Left hand with Jaccoud’s arthropathy

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Fig. 2
figure 2

Radiographic film of both right and left hands with subluxation of the interphalangeal joint of the first finger of the right greater than left hand; no focal bone erosions

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SLE is a chronic inflammatory disease, which can affect the skin, kidneys, nervous system, serous membranes, and other organs. Arthropathy is the most common and earliest manifestation in SLE, affecting 90% of patients with variable degree of articular involvement.1 JA is a deforming, non-erosive arthritis, occurring in 10–35% of SLE patients. While JA can involve all joints, it most commonly manifests as severe deformations of the hands, including ulnar deviation, swan neck and boutonniere deformities, and Z-deformity of the thumb, with multiple non-erosive subluxations. Ulnar drift with subluxation of the MCP joint is often the first sign, whereas swan neck, boutonniere, and Z-deformities may occur at later stages.1, 2