Non-Atherosclerotic Vascular Disease in Women

Takayasu arteritis, fibromuscular dysplasia (FMD), spontaneous arterial dissection, Raynaud's phenomenon, and chilblains are vascular conditions that are associated with an increased predisposition in women and are often underdiagnosed. Takayasu arteritis has an incidence rate of 2.6 cases per million individuals per year in the USA and predominantly affects women of childbearing age. HLA-B5 genetic locus is linked with Takayasu arteritis susceptibility. Methods to determine active disease are limiting; currently utilized clinical and imaging findings and laboratory tests are of limited value for this purpose. Pregnancy poses risks for maternal and fetal complications, and these patients need additional monitoring and care before and after conception. Controlling hypertension and immunosuppression using steroids, biological and non-biological immunosuppressants, are key components of managing patients with this arteritis. FMD commonly affects middle-aged, white females. Its true prevalence is unknown. Renal and cerebrovascular beds are the most frequently involved vascular beds. Its clinical presentation varies from no symptoms to catastrophic events. Controlling vascular risk factors, periodic surveillance, and revascularization when indicated are important factors in FMD management. Spontaneous arterial dissections are less common, but are an important cause of morbidity and mortality in specific populations. Cervicocephalic dissection causes 10-20% of the strokes in young adults, and coronary artery dissection is the culprit in almost one fourth of young women presenting with acute myocardial infarction. Early diagnosis is key to improving prognosis in these patients, as the majority of patients have spontaneous resolution of the dissection with conservative management alone. Increased clinician awareness of the presentation features and angiographic findings are imperative for early diagnosis. Raynaud's phenomenon and chilblains are cold- or stress-induced cutaneous lesions, commonly involving distal extremities. Secondary causes such as connective tissue diseases and malignancies must be thoroughly excluded during evaluation of these conditions. Cold avoidance, systemic and local warming, and oral vasodilator therapy are the mainstays of therapy.