CASE REPORT article
Front. Immunol.
Sec. Autoimmune and Autoinflammatory Disorders: Autoinflammatory Disorders
Volume 16 - 2025 | doi: 10.3389/fimmu.2025.1563542
SAPHO syndrome complicated with IgG4-related ophthalmic disease: A case report and literature review
Provisionally accepted
- 1Suzhou Municipal Hospital, Suzhou, China
- 2Department of Endocrinology, The Affiliated Suzhou Hospital of Nanjing Medical University, Suzhou, Liaoning Province, China
- 3Department of Endocrinology, Nanjing Meishan Hospital, Nanjing, China
- 4Department of Pathology, First Affiliated Hospital of Nanjing Medical University, Nanjing, Jiangsu Province, China
- 5Department of Rheumatology and Immunology, The First Affiliated Hospital of Nanjing Medical University, Nanjing, China
- 6Department of Radiology, First Affiliated Hospital, Nanjing Medical University, Nanjing, Jiangsu Province, China
- 7Department of Endocrinology, First Affiliated Hospital, Nanjing Medical University, Nanjing, Liaoning Province, China
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Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is an extremely rare condition with nonspecific clinical signs and symptoms. Here, we present the case of a 54-year-old Chinese woman with an 8-year history of recurrent furuncles and a 6-year history of clavicular pain. Initially, computed tomography (CT) showed nonspecific changes. The patient was treated with nonsteroidal anti-inflammatory drugs for symptomatic relief; however, clavicular symptoms recurred intermittently. Two years ago, the patient had experienced ocular discomfort from bilateral lacrimal gland enlargement and extraocular muscle thickening. She exhibited elevated IgG4 levels and was diagnosed with IgG4-related ophthalmic disease. Treatment with low-dose glucocorticoids slightly improved her clavicular symptoms. However, over the past month, her clavicular pain worsened. Recent CT and magnetic resonance imaging revealed a deformity of the right clavicular cortex near the end, with poor bone quality and continuity, and bone scintigraphy revealed intense radiotracer uptake in the sternoclavicular region. Consequently, the patient was diagnosed with SAPHO syndrome. We conclude that while clinical manifestations and imaging are helpful in narrowing the differential diagnosis, biopsy and histopathological examinations are necessary to confirm SAPHO syndrome. In patients with an initial presentation of abnormal IgG4 levels, physicians must maintain a high index of suspicion to ensure appropriate treatment.
Keywords: SAPHO syndrome, IgG4, Ophthalmic disease, TNF-a (tumor necrosis factor a), Immune disorders
Received: 21 Jan 2025; Accepted: 20 Mar 2025.
Copyright: © 2025 Liu, Chen, Wang, Wang, Hu and Chen. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence:
Chen xiao Liu, Suzhou Municipal Hospital, Suzhou, China
Huan huan Chen, Department of Endocrinology, First Affiliated Hospital, Nanjing Medical University, Nanjing, Liaoning Province, China
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