The primary vascular dysregulation syndrome: implications for eye diseases

Abstract

Vascular dysregulation refers to the regulation of blood flow that is not adapted to the needs of the respective tissue. We distinguish primary vascular dysregulation (PVD, formerly called vasospastic syndrome) and secondary vascular dysregulation (SVD). Subjects with PVD tend to have cold extremities, low blood pressure, reduced feeling of thirst, altered drug sensitivity, increased pain sensitivity, prolonged sleep onset time, altered gene expression in the lymphocytes, signs of oxidative stress, slightly increased endothelin-1 plasma level, low body mass index and often diffuse and fluctuating visual field defects. Coldness, emotional or mechanical stress and starving can provoke symptoms. Virtually all organs, particularly the eye, can be involved. In subjects with PVD, retinal vessels are stiffer and more irregular, and both neurovascular coupling and autoregulation capacity are reduced while retinal venous pressure is often increased. Subjects with PVD have increased risk for normal-tension glaucoma, optic nerve compartment syndrome, central serous choroidopathy, Susac syndrome, retinal artery and vein occlusions and anterior ischaemic neuropathy without atherosclerosis. Further characteristics are their weaker blood–brain and blood-retinal barriers and the higher prevalence of optic disc haemorrhages and activated astrocytes. Subjects with PVD tend to suffer more often from tinnitus, muscle cramps, migraine with aura and silent myocardial ischaemic and are at greater risk for altitude sickness. While the main cause of vascular dysregulation is vascular endotheliopathy, dysfunction of the autonomic nervous system is also involved. In contrast, SVD occurs in the context of other diseases such as multiple sclerosis, retrobulbar neuritis, rheumatoid arthritis, fibromyalgia and giant cell arteritis. Taking into consideration the high prevalence of PVD in the population and potentially linked pathologies, in the current article, the authors provide recommendations on how to effectively promote the field in order to create innovative diagnostic tools to predict the pathology and develop more efficient treatment approaches tailored to the person.

Endothelial dysfunction in cardiovascular disease and Flammer syndrome—similarities and differences

“…The regulators of the size of retinal vessels are, thus, the vascular endothelial cells. An effect from systemic ET-1 is likely if the blood-brain barrier is disrupted [66,116]. The blood-brain barrier in the ONH is incomplete [117].…”

“…Autonomic dysfunction is suggested by the observation of cold extremities (hands, feet, cornea) exacerbated by cold or emotional upset [97,116]. Cold acra have been found in FS similar to Raynaud's phenomenon (CREST) or even Raynaud's disease, yet less pronounced [98].…”

Breast cancer and Flammer syndrome: any symptoms in common for prediction, prevention and personalised medical approach?

“…On the other hand, the above-listed symptoms are characteristic for the Flammer Syndrome (FS) phenotype extensively described in 2014 by the dedicated research group [3]. FS is described for both healthy individuals in suboptimal health condition and severely diseased patients [4,5]. Consequently, our particular interest to FS in the context of BC research and management may be formulated as follows:…”

Postmenopausal breast cancer: European challenge and innovative concepts

“…FS phenotype [11] is described for both healthy individuals in sub-optimal health condition [12] and severely diseased patients [13]. FS is particularly relevant for females [11].…”

“…FS prevalence evaluated by individual symptoms Figure 1 summarises the prevalence of individual Flammer syndrome symptoms (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15) in two groups of comparison-the BPostmenopausal breast cancer^patients versus the reference group of BBC-free^individuals. Higher prevalence in postmenopausal breast cancer (red) has been demonstrated for altogether 11 symptoms.…”