A 45-year-old Japanese female was hospitalized due to severe abdominal discomfort, bloody diarrhea, arthritis in the limbs, nodular erythema, and high fever. She had no particular history or family history of inflammatory episodes. Despite upper endoscopy and small-bowel follow-through showing no obvious abnormalities, ileocolonoscopy revealed deep discrete longitudinal ulcers accompanied by cobblestone appearance in the colon, together with severe anal fissures. Noncaseating granuloma was detected in the biopsies from the colonic mucosa. The patient was diagnosed with CD, with colonic involvement and extraintestinal joint manifestation. Because she had a complicating anal lesion and had left her disabled child behind, we decided to treat her with an anti-TNFalpha antibody, hoping to shorten her hospital stay. She received subcutaneous adalimumab: 160 mg at week 0, 80 mg at week 2, and thereafter 40 mg every 2 weeks. Her symptoms improved, and the patient was discharged. Sulfasalazine was stopped after the discharge, due to signs of pancreatitis associated with sulfasalazine. After the fifth adalimumab shot, she visited our outpatient clinic with complaints of a tender shoulder and left clavicle and acne spreading over her trunk, limbs, and face (Figure 1A). Two weeks later, both submandibular saliva glands were swollen and tender. She had low-grade fever and could not raise her arms, due to unbearable pain in the bilateral acromioclavicular joints. Her anterior chest pain was painful in the sternoclavicular, and sternocostal joints.
Laboratory tests showed elevated CRP of 0.73 mg/dL, serum amylase of 248 IU/L, and erythrocyte-sedimentation rate of 40 mm/hour without elevated white blood-cell count. Serum antinuclear antibody, anticyclic citrullinated peptide antibody, rheumatoid factor, and anti-Sjogren's syndrome A and B antibodies were negative and IgG4 was within the normal range. Similarly, human leukocyte antigen B27 blood culture, procalcitonin, 1,3-beta-d-glucan, and IFNgamma-release assays (QuantiFeron -TB Gold; Quest Diagnostics, Madison, NJ, US) were negative. Further, because NSAIDs showed inadequate efficacy, we added 20 mg/day prednisolone orally, but the syndrome reappeared when the dose of prednisolone was reduced to 15 mg/day. Additionally, the pain in her low back was diagnosed to be bilateral sacroiliitis. Oral minocycline and corticosteroid ointment seemed to be effective on acne, but ineffective on other symptoms.
Because we had assumed that her cutaneous, bone, and joint manifestations were adverse effects of adalimumab, the anti-TNF was discontinued after the fifth shot, but her cutaneous and articular symptoms continued to exacerbate. Ileocolonoscopy was undertaken again, and showed mucosal healing in the colon and at the anal lesion. Fourteen weeks after the cessation of adalimumab, pustulosis appeared on her palms and soles. The patient was diagnosed to have developed cutaneous lesions like acne and palmoplantar pustulosis, together with articular features like anterior chest pain and sacroiliitis, which appeared after the administration of adalimumab and were consistent with SAPHO syndrome (Figure 1B and C). She was referred to a rheumatologist in a tertiary hospital and received radiological examination, which supported the diagnosis of SAPHO syndrome. Computerized tomography showed bone erosions with edema in the bilateral sternoclavicular joints (Figure 2). Additionally, bone-scintigraphy findings showed extensive uptake of radiopharmaceutical 99mTc at the sternoclavicular joints and sternum, which is called a "bull's head" sign (Figure 3). Intensive uptake was also observed in the bilateral sacroiliac joints. She started receiving low-dose methotrexate (6 mg per week), which did not induce adequate efficacy; it was increased to 12 mg/week 3 months later to induce and maintain clinical remission.
Written informed consent was provided by the patient after explaining the purpose, the nature of the treatments, and the procedures involved. Further, she agreed to the use of the information, including all images to appear in this case report, stating that she fully understood this journal to be a publication that is freely accessible through the Internet. The patient's anonymity should be protected in this report.