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2016 ; 11
(1
): 88
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Multicenter Female Fabry Study (MFFS) - clinical survey on current treatment of
females with Fabry disease
#MMPMID27356758
Lenders M
; Hennermann JB
; Kurschat C
; Rolfs A
; Canaan-Kühl S
; Sommer C
; Üçeyler N
; Kampmann C
; Karabul N
; Giese AK
; Duning T
; Stypmann J
; Krämer J
; Weidemann F
; Brand SM
; Wanner C
; Brand E
Orphanet J Rare Dis
2016[Jun]; 11
(1
): 88
PMID27356758
show ga
BACKGROUND: The aim of the present study was to assess manifestations of and
applied treatment concepts for females with Fabry disease (FD) according to the
current European Fabry Guidelines. METHODS: Between 10/2008 and 12/2014, data
from the most recent visit of 261 adult female FD patients from six German Fabry
centers were retrospectively analyzed. Clinical presentation and laboratory data,
including plasma lyso-Gb3 levels were assessed. RESULTS: Fifty-five percent of
females were on enzyme replacement therapy (ERT), according to recent European FD
guidelines. Thirty-three percent of females were untreated although criteria for
ERT initiation were fulfilled. In general, the presence of left ventricular
hypertrophy (LVH) seemed to impact more on ERT initiation than impaired renal
function. In ERT-naïve females RAAS blockers were more often prescribed if LVH
was present rather than albuminuria. Affected females with missense mutations
showed a similar disease burden compared to females with nonsense mutations.
Elevated plasma lyso-Gb3 levels in ERT-naïve females seem to be a marker of
disease burden, since patients showed comparable incidences of organ
manifestations even if they were ~8 years younger than females with normal
lyso-Gb3 levels. CONCLUSION: The treatment of the majority of females with FD in
Germany is in line with the current European FD guidelines. However, a relevant
number of females remain untreated despite organ involvement, necessitating a
careful reevaluation of these females.
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