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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Alabama Coll Osteopath Med, Osteopath Med, Dothan, AL 36303 USAAlabama Coll Osteopath Med, Osteopath Med, Dothan, AL 36303 USA
Wynn, Austin B.
Metuku, Rajesh
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Alabama Coll Osteopath Med, Osteopath Med, Dothan, AL 36303 USAAlabama Coll Osteopath Med, Osteopath Med, Dothan, AL 36303 USA
Metuku, Rajesh
Santos, Raul
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Archbold Mem Hosp, Nephrol, Thomasville, GA USA
Archbold Mem Hosp, Med Educ, Thomasville, GA USAAlabama Coll Osteopath Med, Osteopath Med, Dothan, AL 36303 USA
机构:
Washington Univ, Sch Med, Dept Med, Div Nephrol,Barnes Jewish Hosp, St Louis, MO 63110 USAWashington Univ, Sch Med, Dept Med, Div Nephrol,Barnes Jewish Hosp, St Louis, MO 63110 USA
Edrees, Fahad
Black, Robert M.
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St Vincent Hosp, Div Renal Med, Worcester, MA 01604 USA
Reliant Med Grp, Worcester, MA USAWashington Univ, Sch Med, Dept Med, Div Nephrol,Barnes Jewish Hosp, St Louis, MO 63110 USA
Black, Robert M.
Leb, Laszlo
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St Vincent Hosp, Dept Hematol Oncol, Worcester, MA 01604 USA
Reliant Med Grp, Worcester, MA USAWashington Univ, Sch Med, Dept Med, Div Nephrol,Barnes Jewish Hosp, St Louis, MO 63110 USA
Leb, Laszlo
Rennke, Helmut
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Harvard Med Sch, Brigham & Womens Hosp, Dept Renal Pathol, Boston, MA USAWashington Univ, Sch Med, Dept Med, Div Nephrol,Barnes Jewish Hosp, St Louis, MO 63110 USA