Analysis of the clinical characteristics of thirteen patients with Weber-Christian panniculitis

• Published in: Clinical rheumatology Vol. 38; no. 12; pp. 3635 - 3641
• Main Authors: Zheng, Wei, Song, Wenyuan, Wu, Qingqing, Yin, Qiaoqiao, Pan, Chaolan, Pan, Hongying
• Format: Journal Article
• Language: English
• Published: London Springer London 01.12.2019; Springer Nature B.V
• Subjects: Arthritis; Corticosteroids; Female; Fever; Humans; Immunosuppressive agents; Laboratories; Male; Medicine; Medicine & Public Health; Middle Aged; Myalgia; Nodules; Original Article; Panniculitis, Nodular Nonsuppurative; Patients; Population studies; Remission; Retrospective Studies; Rheumatic diseases; Rheumatology; Therapeutic applications; Tumors; Clinical features; Immunosuppressants; Corticosteroids; Diagnosis; Weber-Christian disease
• ISSN: 0770-3198; 1434-9949
• DOI: 10.1007/s10067-019-04722-y

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.