TY  - JOUR
A1  - Krämer, Johannes
A1  - Bijnens, Bart
A1  - Störk, Stefan
A1  - Ritter, Christian O.
A1  - Liu, Dan
A1  - Ertl, Georg
A1  - Wanner, Christoph
A1  - Weidemann, Frank
T1  - Left ventricular geometry and blood pressure as predictors of adverse progression of Fabry cardiomyopathy
JF  - PLoS ONE
N2  - Background 
In spite of several research studies help to describe the heart in Fabry disease (FD), the cardiomyopathy is not entirely understood. In addition, the impact of blood pressure and alterations in geometry have not been systematically evaluated. 

Methods 
In 74 FD patients (mean age 36±12 years; 45 females) the extent of myocardial fibrosis and its progression were quantified using cardiac magnetic-resonance-imaging with late enhancement technique (LE). Results were compared to standard echocardiography complemented by 2D-speckle-tracking, 3D-sphericity-index (SI) and standardized blood pressure measurement. At baseline, no patient received enzyme replacement therapy (ERT). After 51±24 months, a follow-up examination was performed. 

Results 
Systolic blood pressure (SBP) was higher in patients with vs. without LE: 123±17 mmHg vs. 115±13 mmHg; P = 0.04. A positive correlation was found between SI and the amount of LE-positive myocardium (r = 0.51; P<0.001) indicating an association of higher SI in more advanced stages of the cardiomyopathy. SI at baseline was positively associated with the increase of LE-positive myocardium during follow-up. The highest SBP (125±19 mmHg) and also the highest SI (0.32±0.05) was found in the subgroup with a rapidly increasing LE (ie, ≥0.2% per year; n = 16; P = 0.04). Multivariate logistic regression analysis including SI, SBP, EF, left ventricular volumes, wall thickness and NT-proBNP adjusted for age and sex showed SI as the most powerful parameter to detect rapid progression of LE (AUC = 0.785; P<0.05). 

Conclusions 
LV geometry as assessed by the sphericity index is altered in relation to the stage of the Fabry cardiomyopathy. Although patients with FD are not hypertensive, the SBP has a clear impact on the progression of the cardiomyopathy.
KW  - cardiovascular magnetic resonance
KW  - clinical manifestations
KW  - disease
KW  - identification
KW  - fibrosis
KW  - 2-dimensional speckle tracking
KW  - myocardial infarction
KW  - therapy
KW  - diagnosis
KW  - impact
Y1  - 2015
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-145131
VL  - 10
IS  - 11
ER  - 
TY  - JOUR
A1  - Kraemer, Markus
A1  - Becker, Jana
A1  - Bley, Thorsten Alexander
A1  - Steinbrecher, Andreas
A1  - Minnerup, Jens
A1  - Hellmich, Bernhard
T1  - Diagnostik und Therapie der Riesenzellarteriitis
T1  - Diagnostics and treatment of giant cell arteritis
JF  - Der Nervenarzt
N2  - Die Riesenzellarteriitis (RZA) ist in der Altersgruppe der über 50-Jährigen die häufigste idiopathische systemische Vaskulitis. Die Erkrankung bedarf einer zeitnahen Diagnostik und Therapie, um schwere Komplikationen wie eine Erblindung oder einen Schlaganfall zu vermeiden. Die Rezidivneigung erfordert eine mehrjährige, zum Teil lebenslange Glukokortikoid(GC)-Therapie, was das Risiko GC-induzierter Langzeitnebenwirkungen erhöht. Daher wird bei der Mehrzahl der Patienten eine additive GC-einsparende Therapie empfohlen. Hierzu steht der Anti-IL-6-Rezeptor-Antikörper Tocilizumab in subkutaner Applikation als zugelassene Substanz zur Verfügung, alternativ kann Methotrexat (MTX) eingesetzt werden (off-label).
N2  - Giant cell arteritis (GCA) is the most common idiopathic systemic vasculitis in the age group over 50 years. It requires prompt diagnostics and treatment to avoid severe complications, such as visual loss or stroke. The tendency to relapse makes a glucocorticoid (GC) treatment necessary for several years and sometimes lifelong, which increases the risk of GC-induced long-term side effects. Therefore, additive GC-sparing treatment is recommended in the majority of patients. For this purpose, the anti-IL‑6 receptor antibody tocilizumab is available as an approved substance for subcutaneous application; alternatively, methotrexate (MTX) can be used (off-label).
KW  - Riesenzellarteriitis
KW  - Diagnose
KW  - Therapie
KW  - Glukokortikoide
KW  - Glukokortikoideinsparende Therapie
KW  - Tocilizumab
KW  - Methotrexat
KW  - giant cell arteritis
KW  - diagnosis
KW  - therapy
KW  - glucocorticoids
KW  - glucocorticoid-sparing agents
KW  - Tocilizumab
KW  - Methotrexate
Y1  - 2022
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-307771
SN  - 0028-2804
SN  - 1433-0407
VL  - 93
IS  - 8
ER  -