Autologous Stem Cell Transplantation in Common Variable Immunodeficiency: A Case of Successful Treatment of Severe Refractory Autoimmune Encephalitis
Common variable immunodeficiency (CVID) is the most common primary immunodeficiency in adults. It is associated with hypogammaglobulinemia, recurring infections and autoimmune phenomena. Treatment includes immunoglobulin substitution and immunosuppressants. Autoimmune neurological manifestations of...
Main Authors: | , , , , , , , |
---|---|
Format: | Article |
Language: | English |
Published: |
Frontiers Media S.A.
2020-06-01
|
Series: | Frontiers in Immunology |
Subjects: | |
Online Access: | https://www.frontiersin.org/article/10.3389/fimmu.2020.01317/full |
id |
doaj-2f43c75d553c49b4892bbebccf86ca29 |
---|---|
record_format |
Article |
spelling |
doaj-2f43c75d553c49b4892bbebccf86ca292020-11-25T02:45:02ZengFrontiers Media S.A.Frontiers in Immunology1664-32242020-06-011110.3389/fimmu.2020.01317548315Autologous Stem Cell Transplantation in Common Variable Immunodeficiency: A Case of Successful Treatment of Severe Refractory Autoimmune EncephalitisMatthias Froehlich0Eva C. Schwaneck1Michael Gernert2Ottar Gadeholt3Patrick-Pascal Strunz4Henner Morbach5Hans-Peter Tony6Marc Schmalzing7Schwerpunkt Rheumatologie/Klinische Immunologie, Medizinische Klinik und Poliklinik II, Universität Würzburg, Würzburg, GermanySchwerpunkt Rheumatologie/Klinische Immunologie, Medizinische Klinik und Poliklinik II, Universität Würzburg, Würzburg, GermanySchwerpunkt Rheumatologie/Klinische Immunologie, Medizinische Klinik und Poliklinik II, Universität Würzburg, Würzburg, GermanyRheumatologische Schwerpunktpraxis Würzburg, Würzburg, GermanySchwerpunkt Rheumatologie/Klinische Immunologie, Medizinische Klinik und Poliklinik II, Universität Würzburg, Würzburg, GermanyKinderklinik und Poliklinik, Universität Würzburg, Würzburg, GermanySchwerpunkt Rheumatologie/Klinische Immunologie, Medizinische Klinik und Poliklinik II, Universität Würzburg, Würzburg, GermanySchwerpunkt Rheumatologie/Klinische Immunologie, Medizinische Klinik und Poliklinik II, Universität Würzburg, Würzburg, GermanyCommon variable immunodeficiency (CVID) is the most common primary immunodeficiency in adults. It is associated with hypogammaglobulinemia, recurring infections and autoimmune phenomena. Treatment includes immunoglobulin substitution and immunosuppressants. Autoimmune neurological manifestations of CVID are rare and occur predominantly as granulomatous disease. We report the case of a 35-year-old woman with CVID who developed autoimmune encephalitis as demonstrated by double cerebral biopsy. Infectious or malignant causes could be excluded. Despite intensive immunosuppressive therapy with common regimens no significant improvement could be achieved. Ultimately, an autologous hematopoietic stem cell transplantation (HSCT) was performed, resulting in lasting complete remission of the encephalitis. To our knowledge, this is the first report of refractory autoimmune phenomena in CVID treated by autologous HSCT.https://www.frontiersin.org/article/10.3389/fimmu.2020.01317/fullcommon variable immunodeficiencyprimary immunodeficienciesautoimmunityautologous stem cell transplantationautoimmune encephalitis |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Matthias Froehlich Eva C. Schwaneck Michael Gernert Ottar Gadeholt Patrick-Pascal Strunz Henner Morbach Hans-Peter Tony Marc Schmalzing |
spellingShingle |
Matthias Froehlich Eva C. Schwaneck Michael Gernert Ottar Gadeholt Patrick-Pascal Strunz Henner Morbach Hans-Peter Tony Marc Schmalzing Autologous Stem Cell Transplantation in Common Variable Immunodeficiency: A Case of Successful Treatment of Severe Refractory Autoimmune Encephalitis Frontiers in Immunology common variable immunodeficiency primary immunodeficiencies autoimmunity autologous stem cell transplantation autoimmune encephalitis |
author_facet |
Matthias Froehlich Eva C. Schwaneck Michael Gernert Ottar Gadeholt Patrick-Pascal Strunz Henner Morbach Hans-Peter Tony Marc Schmalzing |
author_sort |
Matthias Froehlich |
title |
Autologous Stem Cell Transplantation in Common Variable Immunodeficiency: A Case of Successful Treatment of Severe Refractory Autoimmune Encephalitis |
title_short |
Autologous Stem Cell Transplantation in Common Variable Immunodeficiency: A Case of Successful Treatment of Severe Refractory Autoimmune Encephalitis |
title_full |
Autologous Stem Cell Transplantation in Common Variable Immunodeficiency: A Case of Successful Treatment of Severe Refractory Autoimmune Encephalitis |
title_fullStr |
Autologous Stem Cell Transplantation in Common Variable Immunodeficiency: A Case of Successful Treatment of Severe Refractory Autoimmune Encephalitis |
title_full_unstemmed |
Autologous Stem Cell Transplantation in Common Variable Immunodeficiency: A Case of Successful Treatment of Severe Refractory Autoimmune Encephalitis |
title_sort |
autologous stem cell transplantation in common variable immunodeficiency: a case of successful treatment of severe refractory autoimmune encephalitis |
publisher |
Frontiers Media S.A. |
series |
Frontiers in Immunology |
issn |
1664-3224 |
publishDate |
2020-06-01 |
description |
Common variable immunodeficiency (CVID) is the most common primary immunodeficiency in adults. It is associated with hypogammaglobulinemia, recurring infections and autoimmune phenomena. Treatment includes immunoglobulin substitution and immunosuppressants. Autoimmune neurological manifestations of CVID are rare and occur predominantly as granulomatous disease. We report the case of a 35-year-old woman with CVID who developed autoimmune encephalitis as demonstrated by double cerebral biopsy. Infectious or malignant causes could be excluded. Despite intensive immunosuppressive therapy with common regimens no significant improvement could be achieved. Ultimately, an autologous hematopoietic stem cell transplantation (HSCT) was performed, resulting in lasting complete remission of the encephalitis. To our knowledge, this is the first report of refractory autoimmune phenomena in CVID treated by autologous HSCT. |
topic |
common variable immunodeficiency primary immunodeficiencies autoimmunity autologous stem cell transplantation autoimmune encephalitis |
url |
https://www.frontiersin.org/article/10.3389/fimmu.2020.01317/full |
work_keys_str_mv |
AT matthiasfroehlich autologousstemcelltransplantationincommonvariableimmunodeficiencyacaseofsuccessfultreatmentofsevererefractoryautoimmuneencephalitis AT evacschwaneck autologousstemcelltransplantationincommonvariableimmunodeficiencyacaseofsuccessfultreatmentofsevererefractoryautoimmuneencephalitis AT michaelgernert autologousstemcelltransplantationincommonvariableimmunodeficiencyacaseofsuccessfultreatmentofsevererefractoryautoimmuneencephalitis AT ottargadeholt autologousstemcelltransplantationincommonvariableimmunodeficiencyacaseofsuccessfultreatmentofsevererefractoryautoimmuneencephalitis AT patrickpascalstrunz autologousstemcelltransplantationincommonvariableimmunodeficiencyacaseofsuccessfultreatmentofsevererefractoryautoimmuneencephalitis AT hennermorbach autologousstemcelltransplantationincommonvariableimmunodeficiencyacaseofsuccessfultreatmentofsevererefractoryautoimmuneencephalitis AT hanspetertony autologousstemcelltransplantationincommonvariableimmunodeficiencyacaseofsuccessfultreatmentofsevererefractoryautoimmuneencephalitis AT marcschmalzing autologousstemcelltransplantationincommonvariableimmunodeficiencyacaseofsuccessfultreatmentofsevererefractoryautoimmuneencephalitis |
_version_ |
1724764556736069632 |