The author refers to a case described in 1761 by G.B. Morgagni, in which there were all the clinico-pathological features of aspecific aorto-arteritis, as it was pointed out in this century by some Japanese authors. Morgagni's case was a woman 40 years old, whose radial pulses were never perceived for many years before her death; the necroscopic examination showed severe aortic alterations, characterized by ectasias, aneurysms and stenosis, with subclavian obstruction at the origin. The radial arteries, on the contrary, were found unaffected. This case, as well as another reported by Savory in 1856, demonstrates that an obstructive disease of the aorta and its branches had been already noted and described long before in the European Countries in spite of the rarity of this sickness.
Of ten patients with Takayasu's disease (TD), all women, hospitalized in our Service in the last 5 years, seven were more than 38 years old. In these subjects the mean age at diagnosis was 41.2 years. These findings confirm that, in Italy as in other Western countries, including the United States, the diagnosis of TD is usually made later than in Asia and Latin America. This circumstance is probably related to actual later onset of the disease. In most of our cases both the anamnestic data and the angiographic findings demonstrated an evolution of the disease with further involvement of other arteries. Several arterial biopsies consistently showed, within a diffuse sclerosis, more or less extensive inflammatory aggregates characterized by a lymphoplasmacellular infiltration often associated with giant cells. Such a pattern testifies to the persistence of an active arteritis even a long time after the onset of the disease - "persistent active arteritis" instead of "residual arteritis." Pointing out such an evolution of the TD in every stage, our clinicopathologic study emphasizes the importance of a careful followup of the patients; it also advises continuous treatment with corticosteroid drugs, sometimes associated with immunosuppressors, to attain a reduction of the inflammatory process.
After three months of corticosteroid treatment, a sixty-nine-year-old man, suffering from temporal arteritis, developed a Kaposi's sarcoma (KS) initially located on the left ankle and subsequently spread over both feet and hands. Laboratory data showed a deficiency of both humoral and cellular immunity and constant positivity in the tests for cytomegalovirus. The onset of KS during corticosteroid treatment of temporal arteritis is an extremely rare occurrence, this case being only the second one reported in the literature. In our case the development of this neoplasm can be related to an immunodeficiency that led to a deficit in the immunological surveillance, along with an activation of oncogenic viruses.
The clinical and angiographic features of 12 patients showing a significant reduction in the diameters of the distal aorta and the common iliac arteries are described. All the patients were women aged between 23 and 63 years (average age: 49 years), heavy smokers in the majority. An associated aortic arch syndrome was observed in 5 patients. Type IV dyslipidemia was found in 5. In 10 patients the erythrocyte sedimentation rate was increased. Claudication of the lower limbs, without dystrophic phenomena, was the most frequent symptom. Intraoperative biopsies of the distal aorta at the level of the aortic bifurcation were performed in 9 patients who underwent vascular surgery. In all the specimens an extensive fibrosis of the arterial wall was noted, but diffuse or focal lymphoplasmacellular infiltrates were also detected in 7 of them, with giant cells in 2 of the 7. The profile of small aorta and iliac arteries has recently been identified as an autonomous morbid process due to a developmental deficit with superimposition of atherosclerotic phenomena ("hypoplastic aorto-iliac syndrome" — HAIS). The clinical data and the histopathologic findings suggest, however, that several cases of HAIS can claim an acquired rather than a congenital origin. An arteritic process, causing an extensive fibrosis of the arterial wall, might be able to give rise to a noteworthy reduction in the aortic diameter. Among the possible inflammatory causes, Takayasu's disease (aspecifc aortoarteritis) seems to play an important role. The denomination HAIS seems, however, to be too restrictive. It should be replaced by "small aorto-iliac syndrome" (SAIS), which is noncommittal as regards the pathogenesis and applicable in any case.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.