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Published on: June 14, 2016
Arterial fibrodysplasia. Histopathologic character and current etiologic concepts
Insights
Arterial fibrodysplasia, a vascular disease, affects numerous arteries, particularly renal arteries. This study identifies a new form of perimedial dysplasia and discusses potential causes like hormonal influences.
Area of Science:
- Vascular Medicine
- Pathology
- Genetics
Background:
- Arterial fibrodysplasia (AFD) is a non-atherosclerotic vascular disease.
- It affects various arteries, with renal artery involvement being common.
- Existing classifications include intimal fibroplasia and medial hyperplasia, with medical fibroplasia representing a disease continuum.
Purpose of the Study:
- To characterize the spectrum of arterial fibrodysplasia.
- To identify and describe a previously unrecognized form of perimedial dysplasia.
- To explore potential contributing factors to AFD development.
Main Methods:
- Retrospective analysis of 196 patients with arterial fibrodysplasia.
- Detailed pathological examination of 316 affected vessels.
- Histological assessment to identify distinct pathological entities.
Main Results:
- Arterial fibrodysplasia affected 196 patients (172 females, 24 males) with 316 diseased vessels.
- Renal artery lesions were most frequent (152 adults, 25 pediatric).
- A novel form of perimedial dysplasia, marked by peripheral medial elastic tissue accumulation, was identified.
Conclusions:
- Medical fibroplasia encompasses a range of pathological processes.
- A distinct entity of perimedial dysplasia is recognized.
- Hormonal influences, mechanical stresses, and vasa vasorum patterns may predispose individuals to arterial fibrodysplasia.
Abstract:
Arterial fibrodysplasia affected 196 patients (172 females, 24 males) harboring a total of 316 diseased vessels. Renal artery lesions were documented in 152 adult and 25 pediatric patients. Superior mesenteric, celiac, common hepatic, and external iliac arteries were occasionally involved. Intimal fibroplasia and medial hyperplasia are uncommon types of arterial dysplasia. Medical fibroplasia represents a continum of disease, including pathologic processes heretofore categorized as subadventital or perimedial fibroplasia. A previously unrecognized form of perimedial dysplasia, characterized by dense accumulations of elastic tissue about the periphery of the media, is reported as a distinct pathologic entity. Hormonal influences, traction-stretch stresses, and peculiarities in distribution of vasa vasorum in vessels affected by dysplastic processes may be prerequisite to the evolution of arterial fibrodysplasia.
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