1. Prevalence of Raynaud Phenomenon and Nailfold Capillaroscopic Abnormalities in Fabry Disease
- Author
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Achille Aouba, Roland Jaussaud, Jean-Jacques Parienti, Samuel Deshayes, Nathalie Triclin, Laurent Auboire, Bernard Imbert, Boris Bienvenu, and Olivier Lidove
- Subjects
Male ,Pathology ,medicine.medical_specialty ,Cross-sectional study ,Observational Study ,Article ,Microscopic Angioscopy ,Microcirculation ,Raynaud phenomenon ,Sex Factors ,Prevalence ,Humans ,Medicine ,Nailfold Capillaroscopy ,integumentary system ,business.industry ,Raynaud Disease ,General Medicine ,Middle Aged ,medicine.disease ,Fabry disease ,Dermatology ,Capillaries ,Cross-Sectional Studies ,Nails ,Case-Control Studies ,Concomitant ,Fabry Disease ,Female ,business ,Secondary Raynaud phenomenon - Abstract
Fabry disease (FD) is a lysosomal disorder leading to progressive systemic involvement, including microvascular damage that leads to neurological and cardiovascular disorders. We hypothesize that the latter could be documented at an early stage by performing a microcirculation study with nailfold capillaroscopy and evaluation of Raynaud phenomenon. The objective was to measure the prevalence of Raynaud phenomenon and nailfold capillaroscopic abnormalities in FD. This cross-sectional study included a standardized questionnaire and a nailfold capillaroscopy that assessed previously reported patterns in FD (dystrophic and giant capillaries, avascular fields, irregular architecture, dilatation and density of capillaries, hemorrhage), and was conducted on 32 Fabry patients and 39 controls. Capillaroscopic photographs were reviewed by 2 independent blinded investigators. Twelve Fabry patients (38%) suffered from Raynaud phenomenon, 5 were males (ie, 50% of male Fabry patients), compared with 2 controls (13%) (P
- Published
- 2015