It was created in December with the purpose of disseminating scientific information derived from primary and secondary research and presenting cases coming from the practice of Rheumatology in Latin America. Since its foundation, the Journal has been characterized by its plurality with subjects of all rheumatic and osteomuscular pathologies, in the form of original articles, historical articles, economic evaluations, and articles of reflection and education in Medicine. It covers an extensive area of topics ranging from the broad spectrum of the clinical aspects of rheumatology and related areas in autoimmunity both in pediatric and adult pathologies , to aspects of basic sciences. It is an academic tool for the different members of the academic and scientific community at their different levels of training, from undergraduate to post-doctoral degrees, managing to integrate all actors inter-and transdisciplinarily. It is intended for rheumatologists, general internists, specialists in related areas, and general practitioners in the country and abroad.
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It was created in December with the purpose of disseminating scientific information derived from primary and secondary research and presenting cases coming from the practice of Rheumatology in Latin America. Since its foundation, the Journal has been characterized by its plurality with subjects of all rheumatic and osteomuscular pathologies, in the form of original articles, historical articles, economic evaluations, and articles of reflection and education in Medicine.
It covers an extensive area of topics ranging from the broad spectrum of the clinical aspects of rheumatology and related areas in autoimmunity both in pediatric and adult pathologies , to aspects of basic sciences. It is an academic tool for the different members of the academic and scientific community at their different levels of training, from undergraduate to post-doctoral degrees, managing to integrate all actors inter-and transdisciplinarily.
It is intended for rheumatologists, general internists, specialists in related areas, and general practitioners in the country and abroad. It has become an important space in the work of all rheumatologists from Central and South America.
Monckeberg's sclerosis is a rare and low prevalence disease of unknown cause in which small and medium size arteries suffer calcification of the middle layer, leading to a reduction in caliber.. Clinically, the disease manifests by the appearance of arterial type ulcers on the skin of upper and lower extremities. Its diagnosis is difficult, and requires histopathological studies.
Some rheumatic conditions, such as polyarteritis nodosa and giant cell arteritis may mimic this process. Treatment of the disease is not well established.
Although the use of calcium chelators such as sodium thiosulfate has been proposed, the few available studies have failed to show significant results.. This calcification is usually circumferential and may affect the vessel either focally or diffusely.
Its pathophysiology is unclear, it is believed that the lesion will be produced by fatty degeneration of the smooth muscle cells of the middle layer, forming a mass that undergoes a hyaline degeneration and then it becomes calcified. In general, the clinical repercussion is scarce because the reduction of the lumen is minimal, unless it is overlapped with a process of atherosclerosis, where the clinical manifestations become more evident and serious.
Below is reported the case of a patient with multiple complications of Monckeberg's sclerosis, which simulates a vasculitis of medium-sized vessels, and a literature review is carried out. She entered a hospital of third level of complexity in the city of Medellin, Colombia, after 6 months of alteration of the sensitivity of the 4 extremities, Raynaud's phenomenon, weight loss and abdominal pain, with subjective fever prior to admission.
There were necrotic ulcers in the distal phalanges of the upper limbs and a holosystolic heart murmur in the aortic area.
The complement and the protein electrophoresis were normal. The corrected calcium was 9. Electromyography plus conduction velocities of the 4 extremities was performed, evidencing a chronic axonal sensorimotor polyneuropathy, denervation with increased amplitude of motor unit action potentials and neuropathic recruitment in distal muscles.
A transesophageal echocardiogram was carried out, which reported segmental contractility disorders and valve leaflets without the presence of vegetations, but with multiple atheromatous plaques in the entire aortic trajectory. It was decided to start treatment with bosentan for the management of distal vasospastic phenomena and multisystemic compromise; methylprednisolone pulses were started suspecting medial vessel vasculitis.
Biopsies of the sural nerve and adjacent muscle were performed, which were reported as normal. There was little clinical response to management, and subsequently the patient started having sudden respiratory distress ruling out PTE, but with hypotension and persistent fever, and for this reason blood cultures were carried out which evidenced Klebsiella pneumoniae bacteremia, needing to stay in the intensive care unit for vasopressor support and targeted antibiotics.
The patient continued hemodynamically unstable, with important ischemia of lower limbs, quite possibly worsened by the use of vasopressors and the hypoperfusion state, so she required finally infracondylar amputation of the left lower limb. Axial slices in which diffuse hyperdense lesions compatible with calcium deposits are visualized at the level of the abdominal aorta and its branches.
Histopathological studies of the amputated limb were performed describing, in sections of the blood vessels, smooth muscle cells of the middle layer, focally replaced by hyalinized fibrous tissue which presented concentric dystrophic calcification and osseous metaplasia associated with changes compatible with Monckeberg's disease Figs.
It can be observed a micrograph of an arterial wall with atherosclerotic calcium plaque violet, hematoxylin—eosin staining. Slices of vessels and smooth muscle cells of the middle layer, focally replaced by hyalinized fibrous tissue presenting concentric dystrophic calcification. After a prolonged stay in the intensive care unit, the patient was transferred to hospitalization, where steroid clearing was started and she died 8 days later due to ventilatory failure, for which no confirmatory clinical necropsy was performed.
Systemic vasculitis are serious and life-threatening pathologies, however, the heterogeneity of their clinical manifestations makes it difficult to establish an accurate diagnosis. It is here where becomes important a large list of etiologies that can simulate their clinical characteristics at the time of making differential diagnosis, since their treatment and prognosis will depend on it. Monckeberg's sclerosis stands among the differential diagnoses of vasculitis, however, its clinical relevance has been undervalued due to the low clinical impact attributed to it and the low reported incidence, being considered as a condition secondary to a process of deposit of inert calcium.
Despite the foregoing, recent pathophysiological studies have suggested that there are inflammatory and genetic alterations closely related with the medial vascular calcification. It has been described how the deposition of these nanocrystals is capable to induce a phenotypic plasticity of the mesenchymal cells derived from the vascular smooth muscle cells, leading them to a transdifferentiation both in vitro and in vivo , gaining osteogenic characteristics that are directly related with medial vascular calcification.
The above suggests that Monckeberg's sclerosis could be the manifestation of a vascular compromise mediated by immunological genetic and inflammatory alterations that complement a known disease such as atherosclerosis, since at the vascular level it can occur with calcification of the intima layer and this can be associated with atherosclerotic plaques, which result from the accumulation of modified lipids, proinflammatory cytokines and cell apoptosis, compromising the blood flow.
There are 2 theories to explain the described phenomenon: the first proposes the loss of expression of proteins associated with the inhibition of calcification such as: GLA, osteoprotegerin, fibrillin- I and carbonic anhydrase.
The second theory proposes that the calcification is a consequence of metabolic changes due to necrobiotic lesion of the vessel wall. It is from these data that many authors consider the process as a calciphylaxis or calcinosis that can occur concomitantly with endovascular fibrosis; however, for others, Monckeberg's sclerosis is an advanced stage of arteriosclerosis without clear evidence to define that it is a completely independent condition.
Monckeberg, but according to histopathologic studies, it seems to be equally implied. There is a broad spectrum of clinical manifestations, all derived from the vascular occlusion which is generated and that includes coronary vessels, the aorta and its branches, with special emphasis on peripheral vessels.
It is frequent to found ulcers of arterial type in the upper and lower limbs of distal predominance, so serious that sometimes may require amputation of the extremity to control the symptoms. Differential diagnoses are mainly given by vasculitis affecting these vessels, among them, polyarteritis nodosa and giant cell arteritis.
Specific measures for the treatment of Monckeberg's sclerosis are not currently available, and only are taken measures aimed to optimize the calcium metabolism, including calcium chelators such as sodium thiosulfate.
The development of calcification of the arteries is a normal process of aging; risk factors such as diabetes mellitus and chronic kidney disease are directly related to its progression. Monckeberg's sclerosis should be suspected in those patients with the risk factors described; with signs and symptoms of peripheral arterial disease or signs of critical of acute obstruction in which an atherosclerotic, embolic or autoimmune phenomenon is ruled out.
The authors declare that no experiments were performed on humans or animals for this study. The authors declare that they have followed the protocols of their work center on the publication of patient data.
The authors declare that no patient data appear in this article. The authors declare they do not have any conflict of interest. Rev Colomb Reumatol. Report of case and litera ISSN: See more Follow us:. Previous article Next article. Issue 2. Pages April - June Clinical manifestations of Monckeberg's sclerosis. Report of case and literature review. Download PDF. Corresponding author. This item has received. Article information. Show more Show less.
Monckeberg's sclerosis is a rare and low prevalence disease of unknown cause in which small and medium size arteries suffer calcification of the middle layer, leading to a reduction in caliber.
Although the use of calcium chelators such as sodium thiosulfate has been proposed, the few available studies have failed to show significant results. Monckeberg's sclerosis. Palabras clave:. Esclerosis de Monckeberg. McCullough, V. Agrawal, E. Danielewicz, G. Accelerated atherosclerotic calcification and Monckeberg's sclerosis: a continuum of advanced vascular pathology in chronic kidney disease. Clin J Am Soc Nephrol, 3 , pp. Lanzer, M. Boehm, V. Sorribas, M. Thiriet, J. Janzen, T.
Zeller, et al. Medial vascular calcification revisited: review and perspectives. Eur Heart J, 35 , pp. Santos, R. Caffaro, G. Pozzan, M. Saieg, V. Castelli Jr.. Comparative histological study of atherosclerotic lesions and microvascular changes in amputated lower limbs of diabetic and non-diabetic patients.
Arq Bras Endocrinol Metabol, 52 , pp. Couri, G. Pereira, F. BMC Cardiovasc Disord, , pp. Surg Oxf, 33 , pp. Richards, L. March, S. Best Pract Res Clin Rheumatol, 24 , pp. Son, K-H. Jung, S-Y.
Esclerosis de Monckeberg
Calcium deposits form in the middle layer of the walls of medium-sized vessels, and these vessels become calcified independently of atherosclerosis. It can easily be seen as an opaque vessel on normal X-rays and purple material on histologic slides Fig. Involvement of the uterine vessels is very rare. We would like to report the occurrence of this sclerosis in uterine vessels in an utero-vaginal prolapse.
Mönckeberg's sclerosis – is the artery the only target of calcification?
This condition occurs as an age-related degenerative process. However, it can occur in pseudoxanthoma elasticum and idiopathic arterial calcification of infancy as a pathological condition, as well. Its clinical significance and cause are not well understood and its relationship to atherosclerosis and other forms of vascular calcification are the subject of disagreement. Minor degrees of calcification of the cardiovascular system are common in elderly people,  and the prevalence of vascular calcification is increased by some diseases see Epidemiology section. Vascular calcification results from the deposition of calcium phosphate crystals hydroxyapatite as a consequence of disordered calcium phosphate regulation in the blood vessel. Hydroxyapatite is secreted in vesicles that bleb out from vascular smooth muscle cells or pericytes in the arterial wall. Numerous regulators of calcification such as osteopontin , osteoprotegerin , matrix gla protein and fetuin-A , receptor activator of NF-kappa-B , receptor activator of NF-kappa-B ligand and tumor necrosis factor TNF -related apoptosis-inducing ligand protein have been implicated in this process.