PubMed · 7567772
The source did not provide an abstract. Follow the original record for more information.
Explore connections, maps & timelines
E Cooke. 1995. Reflex sympathetic dystrophy.. https://pubmed.ncbi.nlm.nih.gov/7567772/
Cite the original work for its findings. Save a collection to share your selection of sources.
KEEP EXPLORING
Vasculitis, one of the clinical features shared by connective tissue diseases, should be considered when signs and symptoms are observed that may result from tissue ischaemia due to damaged vessels. The lesions seem to result from specific and non-specific immunopathogenic mechanisms targeted at the vascular endothelium. Because of the therapeutic implications it is the physician's responsibility to document its presence and the extent of organ involvement. Prompt institution of immunosuppressive drugs may be lifesaving. On the other hand there are some forms of vasculitis accompanying connective tissue disease which are entirely benign. Patients with infarctions of extremities and progressive functional disturbances of the central nervous system or internal organs because of vasculitis should be treated with high dosages of corticosteroids in combination with cytostatic drugs. Remissions are frequently obtained within three to six months of initiation of treatment and can be maintained with a less aggressive treatment regimen.
Dysfunction of vascular tone control is mainly caused by the derangement of endothelium and of the peripheral nervous system. Studies have suggested changes in the nervous system at either the peripheral level (loss of sensory motor nerves, increased alpha 2-receptor activity, and so on) or the central level (impaired thermoregulation), always linked to the profound abnormality of endothelial function. This dual involvement consistently affects the blood flow, which can be measured with several tools (laser Doppler velocimetry, digital blood pressure response, and so on). Studies have analyzed serologic and instrumental predictors of a possible development of a connective tissue disease in Raynaud's phenomenon. A new wave of studies addressed the role of reperfusion injury and oxygen radicals in provoking chromosomal breakage and generating an immune response through fragmentation of autoantigens in the presence of metals. Antioxidants have been proposed as efficacious treatment of Raynaud's phenomenon. The continued study of regeneration of vessels (therapeutic angiogenesis) may open a new avenue for the treatment of Raynaud's phenomenon and the loss of angiogenesis as in diffuse scleroderma.
The etiology and pathogenesis of intracranial aneurysms are clearly multifactorial, with genetic factors playing an increasingly recognized role. Intracranial aneurysms have been associated with numerous heritable connective tissue disorders, which account for at least 5% of cases. Of these disorders, the most important are Ehlers-Danlos syndrome Type IV, Marfan's syndrome, neurofibromatosis Type 1, and autosomal dominant polycystic kidney disease; the association with intracranial aneurysms, however, has been firmly established only for polycystic kidney disease. Familial intracranial aneurysms are not rare but account for 7 to 20% of patients with aneurysmal subarachnoid hemorrhage and are generally not associated with any of the known heritable connective tissue disorders. First-degree relatives of patients with aneurysmal subarachnoid hemorrhage are at an approximately fourfold increased risk of suffering ruptured intracranial aneurysms, compared to the general population. Various possible modes of inheritance have been identified in families with intracranial aneurysms, suggesting genetic heterogeneity. Although the benefits have never been quantified, screening for asymptomatic intracranial aneurysms should be considered in families with two or more affected members. The yield of such a screening program may approximate 10%. Although it is unlikely that there is a single gene with major effect, much effort is currently being directed at locating intracranial aneurysm genes.