TY  - JOUR
A1  - Strunz, Patrick-Pascal
A1  - Froehlich, Matthias
A1  - Gernert, Michael
A1  - Schwaneck, Eva Christina
A1  - Fleischer, Anna
A1  - Pecher, Ann-Christin
A1  - Tony, Hans-Peter
A1  - Henes, Joerg Christoph
A1  - Schmalzing, Marc
T1  - Immunological Adverse Events After Autologous Hematopoietic Stem Cell Transplantation in Systemic Sclerosis Patients
JF  - Frontiers in Immunology
N2  - 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.
KW  - scleroderma
KW  - fever
KW  - autoimmune disease
KW  - Grave’s disease
KW  - modified Rodnan skin score (mRSS)
KW  - risk factor analysis
KW  - engraftment syndrome
KW  - Sjögren’s syndrome
Y1  - 2021
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-245574
SN  - 1664-3224
VL  - 12
ER  - 
TY  - JOUR
A1  - Froehlich, Matthias
A1  - Schwaneck, Eva C.
A1  - Gernert, Michael
A1  - Gadeholt, Ottar
A1  - Strunz, Patrick-Pascal
A1  - Morbach, Henner
A1  - Tony, Hans-Peter
A1  - Schmalzing, Marc
T1  - Autologous Stem Cell Transplantation in Common Variable Immunodeficiency: A Case of Successful Treatment of Severe Refractory Autoimmune Encephalitis
JF  - Frontiers in Immunology
N2  - 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.
KW  - common variable immunodeficiency
KW  - primary immunodeficiencies
KW  - autoimmunity
KW  - autologous stem cell transplantation
KW  - autoimmune encephalitis
Y1  - 2020
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-206972
SN  - 1664-3224
VL  - 11
IS  - 1317
ER  -