Enfermedad relacionada con IgG4, enfermedad de Erdheim Chester y compromiso aórtico y/o sus ramas. Diagnóstico diferencial con otras vasculitis de grandes vasos / IgG4 related disease, Erdheim Chester disease and aortic involvement and/or its branches. Differential diagnosis with other large vessel vasculitis

La afección cardiovascular en entidades como Erdheim-Chester (EEC), una rara histiocitosis de células no Langerhans, y la enfermedad relacionada con IgG4 (ER-IgG4), una afección fibrinoinflamatoria inmunomediada, es muy variada y habitualmente asintomática hasta su progresión a daños irreversibles cuando no es sospechada. Ante la dificultad de realizar biopsias por el sitio anatómico, es fundamental valerse de características clínicas, demográficas o imagenológicas que puedan diferenciarlas de otras entidades, como las arteritis de grandes vasos. La población masculina, los mayores de 60 años con compromiso a nivel de la aorta abdominal infrarrenal o aneurismas a nivel de la aorta ascendente con o sin compromiso de otros órganos, son orientativos de ER-IgG4. En la EEC es característico el tejido blando concéntrico que recubre la aorta (aorta recubierta) y sobre todo ante la presencia de fibrosis retroperitoneal, compromiso de huesos largos, hidronefrosis, lesión renal aguda posrrenal e hipertensión arterial, existencia de tejido fibrótico perirrenal, engrosamiento de la fascia renal y tejido adiposo perirrenal (signo del riñón peludo).

Cardiovascular disease in entities such as Erdheim-Chester (ECD), a rare non-Langerhans cell histiocytosis, and IgG4-related disease (ER-IgG4), an immunemediated fibrinoinflammatory disease, is highly varied and usually asymptomatic until it progresses to irreversible damage if they are not taken into account. Given the difficulty of performing biopsies by the anatomical site, it is essential to use clinical, demographic or imaging characteristics that can differentiate them from other entities such as large vessel arteritis. Male population, over 60 years of age with involvement of the infrarenal abdominal aorta or aneurysms at the level of the ascending aorta with or without involvement of other organs, are indicative of ER-IgG4. In ECD, the concentric soft tissue covering the aorta (covered aorta) is characteristic, and especially in the presence of retroperitoneal fibrosis, involvement of long bones, hydronephrosis, post-renal acute kidney injury and arterial hypertension, the presence of perirenal fibrotic tissue, thickening of the renal fascia, perirenal adipose tissue (hairy kidney sign).

Afectación cardiovascular en una paciente con arteritis de Takayasu, a propósito de un caso / Cardiovascular involvement in a patient with Takayasu arteritis, in relation to a case

Introducción: la arteritis de Takayasu es una vasculitis sistémica que provoca alteraciones en distintos sistemas de órganos, las más características y peligrosas ocurren en el sistema cardiovascular. Objetivo: socializar las manifestaciones cardiovasculares que con mayor frecuencia se presentan en el curso de la arteritis de Takayasu. Caso clínico: se presenta el caso de una paciente femenina, de 46 años de edad, con manifestaciones clínicas que permiten llegar al diagnóstico de arteritis de Takayasu; dentro del cuadro clínico se presentan múltiples afectaciones cardiovasculares que ensombrecen el pronóstico de la paciente. Conclusiones: las manifestaciones cardiovasculares en la arteritis de Takayasu no solo forman parte de los criterios diagnósticos y de las manifestaciones clínicas de la enfermedad; sino que también forman parte de las complicaciones de la arteritis y su presencia empeora la evolución clínica de la enfermedad y complica el pronóstico del paciente(AU)

Introduction: Takayasu's arteritis is a systemic vasculitis that causes alterations in different organ systems, the most characteristic and dangerous occur in the cardiovascular system. Objective: to socialize the cardiovascular manifestations that most frequently occur in the course of Takayasu's arteritis. Clinical case: the case of a female patient, 46 years of age, with clinical manifestations that lead to the diagnosis of Takayasu arteritis; Within the clinical picture there are multiple cardiovascular affectations that overshadow the prognosis of the patient. Conclusions: the cardiovascular manifestations in Takayasu's arteritis are not only part of the diagnostic criteria and the clinical manifestations of the disease; they are also part of the complications of arteritis and their presence worsens the clinical evolution of the disease and complicates the patient's prognosis(AU)

Cardiovascular system involvement in rheumatic diseases in children / 中华实用儿科临床杂志

The common rheumatic diseases in children including rheumatic fever,juvenile idiopathic arthritis,systemic lupus erythematosus (including neonatal lupus),juvenile dermatomyositis,Kawasaki disease,anaphylactoid purpura.Rheumatic diseases as a group of unknown etiology autoimmune diseases,connective tissue and collagen fibers of different organs can be affected.Systemic inflammatory is the common prominent characteristics of this kind of disease,often resulting in multiple organ damage.Over recent decades,there has been considerable interest in the long-term outcomes of individuals with chronical inflammatory disease and an area of particular concern has been the increased prevalence of cardiovascular disease.Since sustained systemic inflammation is known to accelerate atherosclerosis,doctor should pay attention to rheumatic diseases associated cardiovascular involvements during daily clinical work.The cardiovascular involvement of common rheumatic diseases in children is summarized in this paper.

Carotid artery intima-media thickness in Behcet's disease patients without significant cardiovascular involvement

BACKGROUND/AIMS: Behcet's disease (BD) is a systemic disorder associated with a characteristic vasculitis that can involve both veins and arteries of all sizes. Endothelial activation or injury is a characteristic feature of BD. Endothelial dysfunction is widely regarded as being the initial lesion in the development of atherosclerosis. The carotid artery intima-media thickness (IMT) is a widely accepted marker of subclinical atherosclerosis. We aimed to determine the carotid IMT in BD patients with using high-resolution B-mode Doppler ultrasonography. METHODS: We studied 40 patients (24 males, mean age: 39.1+/-8.5 years) who were diagnosed by the international diagnostic criteria of Behcet's disease and 20 healthy controls (13 males, mean age: 40.2+/-5.1 years), and the two groups were matched by age and gender. No subject in either group had a history of atherosclerosis or its complications. The clinical data, including the age of onset, the duration of disease, a history of medication, the activity score and the laboratory data were analyzed. RESULTS: The carotid IMT in the BD group was significantly higher than that in the control group (0.71+/-0.22 mm vs. 0.59+/-0.09 mm, respectively, p<0.01). Cardiac and major vessel involvements were not identified in the BD group. However, minor vascular involvements were documented in 2 patients with deep vein thrombosis, in 4 patients with superficial thrombophlebitis and in 2 patients with pseudoaneurysm. The carotid IMT in the patients with posterior uveitis or retinal vasculitis was higher than that of the patients without these findings (0.85+/-0.21 mm vs. 0.64+/-0.10 mm, respectively, p=0.007), but there was no difference of the IMT according to minor vascular involvement. CONCLUSIONS: Despite that there was no significant cardiovascular involvement in the BD patients, the carotid IMT was significantly higher in the BD patients as compared with the healthy controls.

Cardiovascular involvement in Behcet's disease / 대한내과학회지

BACKGROUND: Behcet's disease (BD) is a systemic disease of unknown cause, belonging to vasculitis pathologically. There are only few reports describing the frequency and clinical features of cardiovascular involvement in BD, even though the vascular lesions involving artery and large-sized vein has been reported to be important in prognosis. We performed a retrospective study to address the clinical features of Behcet's disease involving cardiovascular system. METHODS: We studied 155 patients with BD who fulfilled the international criteria. The frequency and clinical characteristics of cardiovascular involvement were studied retrospectively. RESULTS: Among 155 patients with BD, 27 patients (17.4%) have cardiovascular involvement. When they were compared with patients without cardiovascular lesions, male proportion was significantly higher (OR 5.541, 95% CI 2.033~15.105, p=0.001) and the age at onset was younger (OR 1.059, 95% CI 1.003~1.118, p=0.037). Nine patients (5.8%) had arterial lesions which included 10 cases of arterial aneurysm and 3 cases of arterial occlusion. Arterial aneurysm occurred at aorta (3 cases) and pulmonary artery (3 cases) most frequently. Arterial occlusion occurred at coronary artery, brachial artery and digital artery. There were 4 patients with cardiac valvular lesions which included aortic regurgitation (3 cases) and tricuspid stenosis (1 case). Nineteen patients (12.3%) had venous lesions. Among them deep vein thrombosis were 25 cases, and transverse sinus thrombosis was one case. When the patients with deep vein thrombosis were divided by the site of the lesion, the cases involving deep femoral and popliteal vein were the most frequent (6 patients respectively), which were followed by iliac vein, superficial femoral vein, and superior vena cava. When the patients were divided into arterial and venous involvement groups, they showed no significant differences in clinical features. CONCLUSION: In BD patients, the frequency of cardiovascular involvement was 17.4% of which that of deep vein thrombosis was the highest. Arterial aneurysm was the most frequent among arterial lesions. The patients with cardiovascular involvement had significantly higher male proportion and younger onset age than the patients without cardiovascular lesions.