Efficacy of rituximab in thymoma associated minimal change disease: case report

被引:1
|
作者
El Ouafi, Zhour [1 ]
Mugnier, Clovis [1 ]
Jeannet, Robin [2 ,3 ]
Danthu, Clement [1 ]
Duval, Marion [1 ]
Belle Mbou, Valere [4 ]
Toure, Fatouma [1 ,3 ]
机构
[1] CHU Limoges, Div Nephrol, Limoges, France
[2] CHU Limoges, Div Immunol, Limoges, France
[3] Univ Limoges, CNRS, CRIBL, INSERM U1262, Limoges, France
[4] CHU Limoges, Div Anatomopathol, Limoges, France
关键词
Glomerulonephritis; Minimal change disease; Thymoma; Rituximab; NEPHROTIC SYNDROME;
D O I
10.1186/s12882-021-02479-8
中图分类号
R5 [内科学]; R69 [泌尿科学(泌尿生殖系疾病)];
学科分类号
1002 ; 100201 ;
摘要
Background Thymomas have been associated with a broad spectrum of autoimmune diseases. Minimal change disease (MCD) is the most frequent pathological lesion reported. Pathophysiological mechanisms involved in secondary MCD, and linking MCD to thymoma are not yet fully explained, although the hypothesis of T cell dysfunction has been suggested. The fundamental therapeutic principles are steroids and surgical treatment of thymoma, but failures and relapses often require immunosuppressant combinations. Case presentation A 62-year-old female was admitted in our unit for a nephrotic syndrome associated with a thymoma. The diagnosis of thymoma associated MCD was confirmed by kidney biopsy. After surgical resection of the thymoma and steroid therapy, no remission was observed. Immunosuppressive therapy was then intensified with introduction of rituximab. Here, we report a steroid-resistant nephrotic syndrome secondary to MCD associated thymoma, which achieved complete remission after rituximab therapy. To the best of our knowledge, this is the first report of the use and efficacy of rituximab therapy in this pathology. Conclusions Our case report suggests that primary and secondary MCD may share similar pathophysiological mechanisms. It does not allow us to draw any conclusions about the mechanism of action of rituximab, but we believe this report argues for the safety and efficacy of rituximab use in thymoma-associated MCD, and therefore constitutes a rationale for future studies.
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