Successful combined treatment with thymectomy, rituximab and tocilizumab for severe thymoma-associated multi autoimmune syndrome

Olafur Sveinsson; Fredrik Piehl; Oskar Aspegren; Max Albert Hietala

doi:10.1016/j.jneuroim.2019.577028

Successful combined treatment with thymectomy, rituximab and tocilizumab for severe thymoma-associated multi autoimmune syndrome

Olafur Sveinsson^*, Fredrik Piehl, Oskar Aspegren, Max Albert Hietala

^*Corresponding author for this work

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2 Citations (Scopus)

Abstract

We present a 53-year-old woman who presented simultaneously with acute inflammatory demyelinating polyneuropathy, Graves' disease, leukocytoclastic vasculitis, elevated acetylcholine antibody receptor antibodies and a mediastinal mass. Thymectomy was performed and revealed a type A thymoma and the clinical picture and paraclinical findings were consistent with a thymoma-associated multi-autoimmune syndrome (TAMA). Beside prednisolone and plasmapheresis, the patient was treated with tocilizumab and rituximab. After surgical and immunomodulatory treatment with tocilizumab and rituximab the patient's condition slowly started to improve. TAMA is associated with a spectrum of autoantibodies and immune-mediated damage to multiple organs. Even if thymectomy is crucial for long term prognosis, aggressive immunomodulation should be considered early in the disease course, especially in cases showing involvement of the peripheral and/or central nervous system.

Original language	English
Article number	577028
Journal	Journal of Neuroimmunology
Volume	336
DOIs	https://doi.org/10.1016/j.jneuroim.2019.577028
Publication status	Published - 15 Nov 2019

Bibliographical note

Funding Information:
OS, OA, MAH do not report any conflicts of interest. FP has received research grants from Biogen, Genzyme, Merck KGaA and Novartis, and fees for serving as Chair of DMC in clinical trials with Parexel, all outside the submitted work. The reporting of clinical data was approved by the patient.

Publisher Copyright:
© 2019 Elsevier B.V.

Other keywords

Rituximab
Thymectomy
Thymoma
Thymoma-associated multi-autoimmune syndrome
Tocilizumab

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10.1016/j.jneuroim.2019.577028

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title = "Successful combined treatment with thymectomy, rituximab and tocilizumab for severe thymoma-associated multi autoimmune syndrome",

abstract = "We present a 53-year-old woman who presented simultaneously with acute inflammatory demyelinating polyneuropathy, Graves' disease, leukocytoclastic vasculitis, elevated acetylcholine antibody receptor antibodies and a mediastinal mass. Thymectomy was performed and revealed a type A thymoma and the clinical picture and paraclinical findings were consistent with a thymoma-associated multi-autoimmune syndrome (TAMA). Beside prednisolone and plasmapheresis, the patient was treated with tocilizumab and rituximab. After surgical and immunomodulatory treatment with tocilizumab and rituximab the patient's condition slowly started to improve. TAMA is associated with a spectrum of autoantibodies and immune-mediated damage to multiple organs. Even if thymectomy is crucial for long term prognosis, aggressive immunomodulation should be considered early in the disease course, especially in cases showing involvement of the peripheral and/or central nervous system.",

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author = "Olafur Sveinsson and Fredrik Piehl and Oskar Aspegren and Hietala, {Max Albert}",

note = "Funding Information: OS, OA, MAH do not report any conflicts of interest. FP has received research grants from Biogen, Genzyme, Merck KGaA and Novartis, and fees for serving as Chair of DMC in clinical trials with Parexel, all outside the submitted work. The reporting of clinical data was approved by the patient. Publisher Copyright: {\textcopyright} 2019 Elsevier B.V.",

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AU - Piehl, Fredrik

AU - Aspegren, Oskar

AU - Hietala, Max Albert

N1 - Funding Information: OS, OA, MAH do not report any conflicts of interest. FP has received research grants from Biogen, Genzyme, Merck KGaA and Novartis, and fees for serving as Chair of DMC in clinical trials with Parexel, all outside the submitted work. The reporting of clinical data was approved by the patient. Publisher Copyright: © 2019 Elsevier B.V.

PY - 2019/11/15

Y1 - 2019/11/15

N2 - We present a 53-year-old woman who presented simultaneously with acute inflammatory demyelinating polyneuropathy, Graves' disease, leukocytoclastic vasculitis, elevated acetylcholine antibody receptor antibodies and a mediastinal mass. Thymectomy was performed and revealed a type A thymoma and the clinical picture and paraclinical findings were consistent with a thymoma-associated multi-autoimmune syndrome (TAMA). Beside prednisolone and plasmapheresis, the patient was treated with tocilizumab and rituximab. After surgical and immunomodulatory treatment with tocilizumab and rituximab the patient's condition slowly started to improve. TAMA is associated with a spectrum of autoantibodies and immune-mediated damage to multiple organs. Even if thymectomy is crucial for long term prognosis, aggressive immunomodulation should be considered early in the disease course, especially in cases showing involvement of the peripheral and/or central nervous system.

AB - We present a 53-year-old woman who presented simultaneously with acute inflammatory demyelinating polyneuropathy, Graves' disease, leukocytoclastic vasculitis, elevated acetylcholine antibody receptor antibodies and a mediastinal mass. Thymectomy was performed and revealed a type A thymoma and the clinical picture and paraclinical findings were consistent with a thymoma-associated multi-autoimmune syndrome (TAMA). Beside prednisolone and plasmapheresis, the patient was treated with tocilizumab and rituximab. After surgical and immunomodulatory treatment with tocilizumab and rituximab the patient's condition slowly started to improve. TAMA is associated with a spectrum of autoantibodies and immune-mediated damage to multiple organs. Even if thymectomy is crucial for long term prognosis, aggressive immunomodulation should be considered early in the disease course, especially in cases showing involvement of the peripheral and/or central nervous system.

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KW - Thymoma-associated multi-autoimmune syndrome

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M1 - 577028

ER -

Successful combined treatment with thymectomy, rituximab and tocilizumab for severe thymoma-associated multi autoimmune syndrome

Abstract

Bibliographical note

Other keywords

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