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.
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.
The role of C-reactive protein (CRP) in cardiovascular disease has been controversially discussed for almost two decades. Specific CRP inhibition, followed by use of CRP inhibitors in controlled clinical trials may be the only way to prove or disprove a causative role for CRP in cardiovascular disease. Endogenous and plant-derived inhibitors of the Na(+)/K(+)-ATPase, i.e. the cardiac glycosides ouabain, digoxin and digitoxin, potently inhibit CRP synthesis in human hepatoma cells and primary human hepatocytes in vitro. In the herein described single-center C-reactive protein-Digoxin Observational Study (C-DOS), 60 patients with decompensated heart failure, NYHA III-IV and severely reduced Left Ventricular Ejection Fraction (LVEF<40%), and elevated CRP plasma levels will be treated by either digoxin+conventional heart failure therapy (30 patients) or by conventional heart failure therapy alone (30 patients). Plasma CRP levels in both groups will be assessed for 21 days. Plasma CRP levels (day21-day0) will be compared by regression analysis in order to find out whether digoxin significantly lowers CRP plasma levels in humans. The trial will not answer the question whether CRP is causative in cardiovascular disease but, by following a step by step approach, investigates for the first time whether cardiac glycosides lower CRP plasma levels in humans. The study hereby serves as a pilot study for subsequent phase III trials. Importantly, it is the first trial ever that systematically uses a direct CRP synthesis inhibitor in vivo in humans.
Zusammenfassung Hintergrund Digitale Gesundheitsanwendungen (DiGA) halten in vielen Bereichen der Medizin Einzug und haben das Potenzial, die Patientenversorgung zu revolutionieren. In der Rheumatologie wäre der Einsatz bei der axialen Spondyloarthritis (axSpA) in Form einer Trainings- und Bewegungs-App denkbar. In einer repräsentativen Umfrage unter Patienten mit axSpA sollte daher ermittelt werden, ob eine solche App aktuell benötigt wird. Methodik Durchführung einer anonymen Onlinebefragung bei axSpA-Patienten der Deutschen Vereinigung Morbus Bechterew e. V. mittels eines Fragebogens; Datenauswertung mittels Excel und GraphPad Prism. Ergebnisse Es nahmen 435 axSpA-Patienten an der Befragung teil. Von den Befragten sehen 84 % die Entwicklung einer speziell auf axSpA abgestimmten Bewegungs-App als notwendig an und genauso viele wollen diese auch nutzen. Patienten unter 60 Jahre, Patienten unter 60-Jahre mit Biologika- oder Januskinase-Inhibitor-Therapie und Patienten mit häufigen Rückenschmerzen geben im Vergleich zur jeweiligen Kontrollsubgruppe einen höheren Bedarf an (jeweils p < 0,001). Schlussfolgerung Die Entwicklung einer Bewegungs-App für die axSpA wird von einem Großteil der Betroffenen als notwendig angesehen, wobei jüngere und intensiver medikamentös therapierte Patienten einen höheren Bedarf zu haben scheinen.
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