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A Chinese case series of Schnitzler syndrome and complete remission in one tocilizumab-treated patient

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Abstract

Schnitzler syndrome (SchS) is a rare acquired systemic autoinflammatory disease. The major clinical features of SchS are urticarial rash and monoclonal gammopathy, accompanied by fever, joint pain, and lymphadenopathy. There were few reports about SchS in Chinese population. Herein, we describe two patients with SchS in China and conducted a systematic literature review about SchS. Two Chinese Han patients were diagnosed as SchS in our department from 2017 to 2019. Their phenotype and genotype were carefully documented and studied. We also conducted a systematic literature review about SchS. There was one man (66 years old) and one woman (49 years old). Recurrent fever and urticarial rash occurred in both of them during the febrile attacks and normalized in asymptomatic intervals. Other manifestations included arthralgia, lymphadenopathy, and hearing loss. Hepatic cirrhosis and epilepsy were seen in the male patient. None of them had bone pain or family histories. Serum monoclonal IgM gammopathy was found in both patients. MyD88 gene mutation L258P was identified in the female patient. They were treated with tocilizumab and tripterygium wilfordii Hook F (TwHF) respectively, and both showed good response. The rarity and diversity of SchS make it difficult to be recognized. Anti-IL-6 agents may be alternative therapies when anti-IL-1 therapy is unresponsive or unavailable. Due to the case report, the effect of TwHF in the treatment of SchS should be further studied.

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Acknowledgments

We appreciate all supports from the patients and their families.

Funding

This work was supported by the Natural Science Foundation of Beijing (grant number 7192170), the Chinese Academy of Medical Sciences (CAMS) Innovation Fund for Medical Sciences (CIFMS) (grant number 2017-I2M-3-001), and the National Key Research and Development Program of China (grant numbers 2016YFC0901500; 2016YFC0901501).

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Authors and Affiliations

  1. Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China

    Ruyu Yan & Min Shen

  2. Department of Rheumatology, the Second Affiliated Hospital, Chongqing Medical University, Chongqing, China

    Ruyu Yan

  3. Department of Infectious Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China

    Wei Cao & Xinchao Liu

  4. Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China

    Feng Li

  5. Department of Rheumatology, Peking Union Medical College Hospital, No.1 Shuaifuyuan, Dongcheng District, Beijing, 100730, China

    Min Shen

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  1. Ruyu Yan
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  2. Wei Cao
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  4. Feng Li
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Contributions

Ruyu Yan, Wei Cao, Xinchao Liu, Feng Li, and Min Shen were treating physicians for the patients and had contributed suggestions for the diagnosis and management of the patients. Ruyu Yan drafted the manuscript. Min Shen designed the study and revised the manuscript. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Min Shen.

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Ethical approval for this study was approved by the Institutional Review Board of Peking Union Medical College Hospital and performed according to the Declaration of Helsinki.

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Yan, R., Cao, W., Liu, X. et al. A Chinese case series of Schnitzler syndrome and complete remission in one tocilizumab-treated patient. Clin Rheumatol 39, 3847–3852 (2020). https://doi.org/10.1007/s10067-020-05204-2

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  • DOI: https://doi.org/10.1007/s10067-020-05204-2

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