A 42 year old Japanese woman presented to our clinic complaining of severe pain in her neck, both shoulders, and lumbar spine. She had had pustulosis palmaris and plantaris from age 16 and recurrent painful swelling of the clavicles, the sternoclavicular joints, and the sternum from age 25. She reported multiple visits to the doctor for similar symptoms, and she was treated with non-steroidal anti-inflammatory drugs.

On initial examination at our clinic, her lumbar and cervical spine mobility was limited considerably. She had an oedematous face and subcutaneous collateral circulation on chest and abdomen. Laboratory testing showed a white cell count of 13.5×10⁹/l and a C reactive protein of 142 mg/l (normal <5). Antinuclear antibodies, antineutrophil cytoplasmic antibodies, anticardiolipin antibodies, lupus anticoagulants, and rheumatoid factors were all negative. Prothrombin time and …