ABC | Volume 111, Nº6, December 2018

Brief Communication Costa et al Erdheim-Chester’s Disease Arq Bras Cardiol. 2018; 111(6):852-855 Figure 2 – A) Thoracic and abdominal aorta in 3D reconstruction. B) Aorta seen in the sagittal view, showing diffuse thickening of the entire wall with a narrowing area in the infra-renal aorta. C) Thoracic aorta with parietal thickening and luminal reduction in the origin of left subclavian artery. D) Thickening at the origin of the renal arteries, without significant obstruction characterization. Discussion DEC is a rare disease, which is difficult to diagnose. The symptoms are varied and not present in all patients. The main complaint is bone pain and there may be fever, night sweats, adynamia, and weight loss, among other symptoms. 7 These symptoms are not pathognomonic but are useful for assessing treatment response. The interaction of the Cardiology and Hematology services in this scenario allows the adequate diagnosis and management of the cardiovascular system involvement. The definitive diagnosis is attained through the histological analysis of biopsy samples of affected tissues showing granulomatous infiltration, with CD68 expression, but with negative CD1a staining. Treatment is based on the administration of interferon- α and Vemurafenib, Cladribine and AnA-kinase may be used as the second treatment line, aiming to achieve disease control. 6 Cardiac involvement in ECD has a worse prognosis andmost of the time, it is asymptomatic. Approximately 75% of patients with ECD have some cardiovascular impairment and 60% will be diagnosed with ECD 7 based on cardiovascular findings, such as in cases 1 and 4 reported above. The cardiologist’s knowledge about this disease allows an early diagnosis in these situations. The most characteristic cardiovascular finding of ECD is aortic involvement, 5 as seen in case 4, and the most common cardiac lesion is found in the pericardium as pericardial effusion, rarely being associated with cardiac tamponade. The myocardium, endocardium and valvular apparatus may also be involved. Left ventricular dysfunction, as seen in patient 3, is less frequently observed, but it has also been previously described. 7,8 Aortic infiltration by ECD is visualized on CT scans as a “coated aorta.” This phenomenon results from periaortic infiltration by histiocytes, predominantly in the adventitial layer. 5 The periaortic fibrosis degree varies from patient to patient, as well as the affected segment. It can occur symmetrically, circumferentially and limited to a specific segment of the aorta or throughout the vessel. Perivascular infiltration in vessels adjacent to the aorta can also occur in the brachiocephalic trunk, left carotid artery, left subclavian artery, coronary arteries, pulmonary trunk, celiac trunk, superior mesenteric artery, and renal arteries. 7 The clinical presentation depends on which artery is involved and its degree of stenosis. Cerebral ischemia may occur due to carotid involvement, as identified in case 4, and myocardial infarction due to coronary involvement. Renal artery involvement occurs in approximately 20% of cases 7,8 and may result in stenosis of these vessels and renovascular hypertension. Treatment is performed through angioplasty and stenting. Pericardial infiltration can manifest as pericardial thickening with or without fibrosis, and symptoms vary according to the degree of disease severity. Myocardial involvement occurs sequentially to the pericardial involvement and manifests as myocardial hypertrophy, easily diagnosed by the echocardiogram. Thickening can be found in the ventricles, atria, coronary sulci 7 and interatrial septum. 9 Most patients have atrial involvement, often as a pseudotumor, affecting mainly the atrial posterior wall, often projecting into the atrium. Another observed lesion is the infiltration of the right atrioventricular sulcus, where the tissue usually surrounds or infiltrates the right coronary artery. 10 Haroche et al., 11 retrospectively analyzed 37 patients with ECD using CT and CMR: 70% had abnormal cardiac imaging, of which 49% had abnormal infiltration of the right cavities, including 30% with pseudotumor infiltration in the RA, as demonstrated in cases 1 and 2, and 19% with infiltration of the atrioventricular sulcus. Lipomatous hypertrophy of the interatrial septum (LHIS) is a differential diagnosis that should be considered in some cases, since the TTE often describes the alterations as lipomatous infiltrations. All patients with LHIS show uptake at the 18-FDG PET-CT; however, with smaller mean SUVs 854