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Moreno-Martínez et al. Rare Dis Orphan Drugs J 2024;3:9 https://dx.doi.org/10.20517/rdodj.2023.51 Page 11 of 16

[122]
Recent studies suggest a protective effect of ERT after an initial latency period of 6 months . In a meta-
analysis including seven cohort studies and two randomized, controlled studies (7,513 participants: 1,471 on
ERT vs. 6,042 on native treatment), the stroke recurrence ratio in the ERT treatment group (including both
[123]
agalsidase alfa and beta) was 8.2% vs. 16% in the native treatment group (P = 0.03) .

It is worth noting that ERT has been shown to reduce both the burden of disease [124,125] and the risk of
[126]
thromboembolic events, including stroke . The beneficial effect of ER, in patients naïve to specific
treatment, on stabilizing and improving cardiomyopathy, especially with prompt initiation of therapy, also
has a preventive effect on cardioembolic stroke .
[127]
In patients with FD, classical cardiovascular risk factors (smoking, hypercholesterolemia, diabetes mellitus,
sedentarism, and hypertension) should be treated intensively, both for primary and secondary
prevention . We recommend aspirin or clopidogrel in patients who suffered an ischemic stroke or TIA.
[128]
Although no clinical trials are available regarding primary prevention, we also indicate antithrombotic
[129]
medications in FD patients with MRI lesions Fazekas grade 2 or 3 .
We usually use clopidogrel when aspirin is not tolerated and recommend statins in primary and secondary
prevention for their pleiotropic effects [17,98,130] .

Anticoagulation is reserved for patients with confirmed atrial fibrillation or cardioembolic stroke.

DECLARATIONS
Authors’ contributions
Made substantial contributions to conception and design, and participated in original draft writing and final
revision: Moreno-Martínez D, León-Cejas L, Reisin R

Availability of data and materials
Not applicable.

Financial support and sponsorship
None.

Conflicts of interest
All authors declared that there are no conflicts of interest.

Ethical approval and consent to participate
Not applicable.

Consent for publication
Not applicable.

Copyright
© The Author(s) 2024.

REFERENCES
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2. Germain DP. Fabry disease. Orphanet J Rare Dis 2010;5:30. DOI PubMed PMC
3. Mehta A, Ricci R, Widmer U, et al. Fabry disease defined: baseline clinical manifestations of 366 patients in the Fabry Outcome