Synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome and psoriatic arthritis share some clinical characteristics despite of unclear pathogenesis of SAPHO syndrome. The standard treatment of SAPHO syndrome is not established because of the rare occurrence of the syndrome [1]. As empirical treatments of SAPHO syndrome refer to the treatments of psoriatic arthritis, anti-inflammatory therapy and succeeding tumor necrosis factor monoclonal antibody (anti-TNF-α) therapy for refractory cases should be evaluated [2]. Effectiveness and safety profiles of adalimumab in treatment of psoriatic arthritis are well established [3]. Few cases of SAPHO syndrome are reported in Korea [4, 5] with a solitary case with etanercept treatment [4]. We experienced a SAPHO case presenting with venous engorgement in anterior chest and was treated successfully with adalimumab.

A 60-year-old woman visited our clinic due to slowly progressive venous engorgement in her left chest, which was accompanied by intermittent pain and swelling. On physical examination, small pustular-like lesions were found in both soles and tenderness and bony swelling was observed in left clavicle. Venous engorgement was observed in left anterior chest. Erythrocyte sedimentation rate (ESR) was 69 mm/h and C-reactive protein (CRP) level was 4.33 mg/dL. A computed tomography (CT) venography showed a remarkable hyperostosis of the clavicular head at the level of the thoracic outlet between her clavicle and her first rib, compressing the venous return in her left subclavian vein (Figure 1). Collateral circulations have developed from her left subclavian vein to left and right internal jugular vein across the sternum. The tender swelling partially responded to the treatment with sulfasalazine (1 g twice daily), methotrexate (7.5 mg weekly) and prednisolone (5 mg daily). ESR and CRP level also decreased to 27 mm/h and 0.11 mg/dL.

Figure 1 Compressed left subclavian vein between the clavicle (arrowhead) and first rib (arrow) on a coronal thin-slab volume rendering image. Click for larger imageDownload as PowerPoint slide

After 20 months, pain and swelling were aggravated in the clavicle. ESR and CRP level increased to 77 mm/h and 3.55 mg/dL. She suffered from recurrent oral mucositis due to methotrexate. Adalimumab 40 mg injection began to be administered per 2 weeks.

Following 1 month treatment with adalimumab, left clavicular pain and tenderness disappeared. Venous engorgement was significantly decreased. ESR and CRP level decreased to 14 mm/h and 0.03 mg/dL respectively. She could discontinue nonsteroidal anti-inflammatory drugs and sulfasalazine. She is now maintaining adalimumab (40 mg biweekly) with methotrexate (7.5 mg weekly) and aspirin (100 mg daily). Ten months after adalimumab treatment, three-dimensional reconstruction CT scan was performed. The diameter of left clavicle and the first rib has significantly decreased from 40.53 mm to 37.55 mm (Figure 2).

Figure 2 (A) Sclerotic change and hyperostosis (40.53 mm) of the clavicle and first rib. Before adalimumab treatment. (B) Less prominent sclerotic change of the clavicle and decreased hyperostosis (37.55 mm). During adalimumab treatment. Click for larger imageDownload as PowerPoint slide

In adult SAPHO patient, anterior chest region is the most common site of skeletal manifestation, which appears mostly as sternoclavicular joint hyperostosis and sclerotic change [1]. But hyperostosis and hypertrophy of surrounding structures rarely lead to subclavian vein or superior vena cava obstruction. This is the first SAPHO case in Korea, in whom progressive hyperostosis of clavicle resulted in left subclavian vein obstruction. Collateral circulations from her left subclavian vein to left and right internal jugular vein developed into venous engorgement.

In this case, we experienced an unusual case of SAPHO who presented with venous engorgement caused by bony swelling of left clavicle. She was successfully treated with anti-TNF-α therapy.