Abstract
Background
Weber-Christian disease (WCD) is an uncommon, idiopathic disease that is challenging to diagnose and has an unclear treatment protocol. We reviewed thirteen patients with WCD and analyzed their clinical characteristics. The purpose of this article was to improve the understanding of this rare disorder.
Methods
Thirteen cases of WCD were analyzed retrospectively regarding their clinical manifestation, laboratory results, misdiagnoses, therapy, and outcome.
Results
Of the thirteen patients diagnosed with WCD, the majority were female (male to female ratio, 2:11), with a mean patient age of 50.1 years. Subcutaneous nodules were the most commonly reported symptom, followed by fever, arthralgias/arthritis, and myalgia. The laboratory results were typically nonspecific. In total, 61.5% patients were misdiagnosed before pathology confirmed the diagnosis of WCD. Most patients were treated with corticosteroids and/or immunosuppressants. Two patients were treated surgically. While long-term remission was successfully achieved in some patients, others had recurrent symptoms.
Conclusion
WCD was predominantly observed in female patients in our cohort. Subcutaneous nodules and fever were the most common clinical characteristics. In addition, the patients’ laboratory test results were nonspecific, which led to a high misdiagnosis rate. In this study population, corticosteroid and/or immunosuppressant treatments were efficacious therapeutic interventions for WCD.
Key Points •Subcutaneous nodules and fever were the most common clinical characteristics in Weber-Christian panniculitis. •Misdiagnosis rate was higher in Weber-Christian panniculitis patient; tumors, bacterial infections and rheumatic diseases were the most common misdiagnoses. •Corticosteroid and/or immunosuppressant therapy was effective in most Weber-Christian panniculitis patients. |
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Acknowledgments
We would like to thank Lili Yu (Department of Pathology, Zhejiang Provincial People’s Hospital, People’s Hospital of Hangzhou Medical College, Hangzhou, China) for his assistance in identifying Weber-Christian panniculitis for the present study.
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Wei Zheng had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis. Wei Zheng, Wen yuan Song, and Hongying Pan were involved in the design of the study; authors Wei Zheng and Wen yuan Song jointly completed the writing, and Qingqing Wu, Qiaoqiao Yin, and Chaolan Pan were substantially involved in revising the article.
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Because this study was based on the retrospective analysis of existing administrative and clinical data, the need to obtain informed patient consent was waived by the Ethics Review Committee of Zhejiang Provincial People’s Hospital.
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Zheng, W., Song, W., Wu, Q. et al. Analysis of the clinical characteristics of thirteen patients with Weber-Christian panniculitis. Clin Rheumatol 38, 3635–3641 (2019). https://doi.org/10.1007/s10067-019-04722-y
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DOI: https://doi.org/10.1007/s10067-019-04722-y