@article{StrunzFroehlichGernertetal.2021,
  author    = {Strunz, Patrick-Pascal and Froehlich, Matthias and Gernert, Michael and Schwaneck, Eva Christina and Fleischer, Anna and Pecher, Ann-Christin and Tony, Hans-Peter and Henes, Joerg Christoph and Schmalzing, Marc},
  title     = {Immunological Adverse Events After Autologous Hematopoietic Stem Cell Transplantation in Systemic Sclerosis Patients},
  series = {Frontiers in Immunology},
  volume    = {12},
  journal   = {Frontiers in Immunology},
  issn      = {1664-3224},
  doi       = {10.3389/fimmu.2021.723349},
  url       = {http://nbn-resolving.de/urn:nbn:de:bvb:20-opus-245574},
  year      = {2021},
  abstract  = {Autologous hematopoietic stem cell transplantation (aHSCT) represents an effective treatment for systemic sclerosis (SSc), but it also can cause immunological adverse events (iAEs). Therefore, we aimed to determine the frequency of iAEs [engraftment syndrome (ES) and secondary autoimmune disorder (sAD)] and to identify potential risk factors for their development in a retrospective analysis on 22 patients similarly transplanted due to SSc. While nine patients (41\%) suffered from ESs, seven sADs occurred in six patients (27\%). Patients who developed ES were older in our cohort (52.45 vs. 42.58 years, p = .0433, Cohen's d = 0.86), and cardiac involvement by SSc was associated with development of ES (OR = 40.11, p = .0017). Patients with manifestation of sAD had a higher modified Rodnan skin score (mRSS) reduction after aHSCT (90.50\% vs. 60.00\%, p = .0064, r = .65). Thus, IAEs are common after aHSCT for SSc and can occur in different stages during and after aHSCT with characteristic clinical manifestations. Good cutaneous response after aHSCT might be considered as a risk factor for sAD, and higher age at aHSCT and cardiac involvement might be considered as risk factors for the development of ES.},
  language  = {en}
}
@article{FroehlichSchwaneckGernertetal.2020,
  author    = {Froehlich, Matthias and Schwaneck, Eva C. and Gernert, Michael and Gadeholt, Ottar and Strunz, Patrick-Pascal and Morbach, Henner and Tony, Hans-Peter and Schmalzing, Marc},
  title     = {Autologous Stem Cell Transplantation in Common Variable Immunodeficiency: A Case of Successful Treatment of Severe Refractory Autoimmune Encephalitis},
  series = {Frontiers in Immunology},
  volume    = {11},
  journal   = {Frontiers in Immunology},
  number    = {1317},
  issn      = {1664-3224},
  doi       = {10.3389/fimmu.2020.01317},
  url       = {http://nbn-resolving.de/urn:nbn:de:bvb:20-opus-206972},
  year      = {2020},
  abstract  = {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.},
  language  = {en}
}