The current study includes 40 men and 32 women. This sex distribution is concordant with the slight male preponderance observed in the 1996 study (32 males: 26 females). Mean age at diagnosis in the current series was 54.7 years ± standard deviation (range, 28-84 yr), which is similar to previous findings.
Forty (56%) of the 72 patients had periaortic "fibrosis"; this involved the whole aorta in 20 cases, was limited to the thoracic aorta in 10 cases, and was limited to the abdominal aorta in 10 cases. Periaortitis was demonstrated by CT scan in 25 patients, and/or by MRI in 7 cases. Autopsy findings confirmed periadventitial, xanthogranulomatous, perivascular infiltration of the aorta in 15 cases1,12,13,18,21,22,25,31,50,60,66,80. Surgery also provided valuable data concerning periaortic infiltration in 2 cases: in the first patient reported by Serratrice et al69 a glossy aspect of the aorta was noted during aortobifemoral bypass with difficult retroperitoneal tunneling of the prosthesis due to local inflammation; in the study by Kenn et al37, the surgeon found a condensed periaortal tissue during aortic valve replacement and bypass surgery. This tissue showed remarkable histiocytic infiltration and a foamy appearance of the cytoplasm on pathologic specimen from the aortic valve and ascending aorta.
Aorta Branches and Other Arteries
Major blood vessels apart from the aorta were involved in 13 of the 66 cases described in the literature: basilar artery (n = 3)22,25,51, vertebral arteries (n = 2)22,50, carotids (n = 3)39,50,66, innominate artery (n = 1)39, anterior iliac arteries (n = 3)60,69, right renal artery (n = 3)1,18,69, left renal artery (n = 2)44,69, celiac trunk stenosis (n = 2)18,69, right popliteal artery (n = 1)49, and right medial cerebral artery (n = 1)50. However, as mentioned above, these data are certainly incomplete, and it is highly probable that other arteries were surrounded by "fibrosis." Indeed, among our 6 personal cases with aortic involvement reported here, the superior mesenteric artery (6/6), left common carotid artery (5/6), left subclavian artery (5/6), left renal artery (5/6), right renal artery (4/6), brachiocephalic trunk (4/6), pulmonary trunk (3/6), celiac trunk (3/6), and left (2/6) or right coronary artery (1/6) were surrounded by fibrosis (Table 3).
Venous involvement was reported much less frequently in the 66 cases from the literature: 2 patients had deep vein thrombosis with pulmonary embolism49,70, 2 patients had superior sagittal sinus thrombosis49,51 and 1 patient had superior vena cava obstruction77. Venous involvement was also observed in 3 of our 6 personal cases: fibrosis around the right pulmonary artery and coronary sinus was observed in Cases 2 and 3; the patient described in Case 3 had fibrosis around the superior vena cava (with a stenosis near the right atrium) and the thoracic portion of the inferior vena cava and thrombosis of the right internal jugular vein; the patient described in Case 6 had fibrosis surrounding the pulmonary trunk.
Although ECD may involve the myocardium and endocardium, pericardial involvement is by far the most frequent heart lesion4. In the current series, 32 of the 72 patients (44%) had pericardial involvement, 9 of whom also had periaortic "fibrosis." Pericardial effusion was found in 17 patients1,3,4,7,9,17,20,27,28,33,36,39,57,63,67,77 including our Case 6, leading to tamponade in 5 cases4,7,27,57,77. Detailed analysis of pericardial fluid was available in 3 patients only: clusters of mesothelial cells, foamy macrophages and eosinophils, lymphocytes and polymorphonuclear leukocytes were observed in the second patient described by Resnick et al63; mesothelial cells in their third patient63; and histiocytes and inflammatory cells in the patient reported by Gupta et al30. In most cases, other causes of pericarditis were ruled out and pericardial effusion could be attributed to ECD. Pericardial involvement was mainly identified by imaging techniques (CT scan, echocardiography) in the absence of histologic proof. In our Case 3, the pericardium was infiltrated as attested by its thickened aspect on CT scan and on MRI. Including our Case 4, histologic analysis of the pericardium revealed histiocyte infiltration in 15 patients1,3,4,7,18,20,47,51,63,66,70,71,75,77. This was discovered at autopsy in 6 cases. In 6 cases (including our Case 4), spumous histiocytes were reported in pericardial tissue4,47,66,70,75, and in only 1 case foamy histiocytes were reported to be positive for CD68, negative for CD1a, and partially positive for S-1004.
Twenty-two patients had myocardial involvement1,5,14,18,19,21,25,28,31,35,37,39,50,53,58,60,66,69,72,82 including our Cases 2 and 3. Six of these patients (including our Case 3) had a right atrial tumor5,25,28,50,58. Deep myocardial involvement was found in 1 case, as attested by myocardial thickening on echocardiography39. Endomyocardial biopsy revealed histiocyte infiltration in the first patient reported by Serratrice et al69. Epicardial involvement was seen in 4 patients14,21,26,31 as demonstrated by autopsy findings: epicardial atrial thickening14, extensive epicardial fibrosis21, subepicardial fat tissue thickening31, and microscopic foci of epicardial infiltration26.
Symptomatic heart valve diseases (3 aortic and 3 mitral regurgitations) were found in 6 patients37,39,61,69 including our Cases 3 and 4. Valve replacement was required in 3 patients, revealing histiocyte infiltration of the aortic valve in 2 patients37 including our Case 4.
Myocardial infarction was reported in 6 cases15,19,25,53,60,72, leading to death in 219,25. Mean age of the first myocardial infarction was 60.8 years (range, 33-86 yr). Coronary infiltration was noted in 5 patients: stenosis of the interventricular artery that was stented35, obstruction of the circumflex artery60, severe "2-vessel coronaropathy stage II"37, "fibrosis" around the left coronary (our Case 2), and fibrosis around both the left and right coronary arteries (our Case 3).
Nineteen patients had heart failure, leading to death in 11% of cases (8/72). In 2 cases, heart failure could be attributed to dilated cardiomyopathy39,74. In 1 case, it was due to "constrictive and restrictive" cardiomyopathy with paroxysmal atrial flutter77, and in 1 case to "decreased systolic function of the myocardium"17.
In our literature review, blood pressure was surprisingly not reported in 42 cases (58%). Twenty-two patients had systemic hypertension. Six of these patients had renovascular hypertension related to perirenal artery stenosis1,18,44,69, as described in our Cases 1 and 2. However, in reports it was not always possible to correlate the presence of hypertension with imaging of renal arteries.
We found no data regarding lipid profile for 42 of the 72 patients in this series. For 22 patients, cholesterol and triglyceride profiles were within the normal range; 5 had hypertriglyceridemia (including our Cases 1 and 2); and 3 had hypercholesterolemia.
Association of ECD Cardiovascular Involvement With Other Clinical Features
Other organs were frequently infiltrated in our 72 ECD patients: 32 (44%) displayed exophthalmos, 28 (39%) had "xanthelasma" or "xanthoma-like lesions," 25 (35%) had neurologic involvement, and 25 (35%) had diabetes insipidus. Among the 58 patients with all forms of ECD included in the 1996 study, we found that cardiovascular involvement was significantly associated with exophthalmos, neurologic involvement, and xanthelasma (p = 0.006, 0.0153, and 0.03 according to the Fisher exact test, respectively). There was a trend toward an association between cardiovascular involvement and diabetes insipidus (p = 0.06).
Follow-Up With Special Focus on Cardiovascular Involvement
Data concerning follow-up were available for 58 of the 72 (81%) patients: 35 of these 58 (60%) patients died, which is consistent with our prior findings, that is, 22 deaths (59%) out of 37 available patients in the 1996 report (21 deaths being included in both series), confirming the severe prognosis of ECD. Cardiovascular complications were responsible for death in 11 of these 35 patients (31%): 7 died of heart failure3,13,18,19,69,71,72. Of these 7 patients, 2 died of right-sided heart failure secondary to extensive bilateral pulmonary fibrosis3,71; 1 died of tamponade27; 2 died of myocardial infarction19,25; 1 died following occlusion of the abdominal aorta due to severe atherosclerosis22; and 1 died of colic ischemia following surgery60. The other 24 patients seemed to die from infiltration of other organs and/or treatment-related complications, that is, death was not directly related to cardiovascular involvement.
Data were available for 34 of the 35 patients who died; mean survival time was 19.2 months after diagnosis (range, 0-120 mo). Considering all 55 patients for whom follow-up duration was known (35 deaths, 14 patients reported alive with variable follow-up periods, and our 6 cases), the mean follow-up time was 28.9 months (range, 0-300 mo), which is shorter than the mean follow-up reported in the 1996 series (32 mo).
Although cardiovascular involvement was reported in the first case of ECD14, its importance has been largely overlooked. Improvements in radiologic imaging techniques have clearly increased the likelihood of detecting cardiovascular involvement in recent case reports. We found cardiovascular infiltration in about 40% of the 178 cases of ECD reported in literature. This is probably an underestimation as data regarding cardiovascular involvement were often incomplete, emphasizing the lack of recognition of this manifestation of ECD.
Periaortic "fibrosis" is the most frequent cardiovascular involvement in ECD. We found 40 cases of ECD with periaortic fibrosis (including our 6 cases), 20 of which had a "coated aorta" aspect. When the whole aorta is infiltrated, this "coated aorta" aspect can give the impression of a lead pipe39,69. Analysis of samples obtained during autopsy or surgery has shown that this "fibrosis" is due to periaortic infiltration by histiocytes37,69. Fibrosis is mainly located in the adventitia1,13,22,23,60. Nevertheless, as seen on the CT scans of Cases 1 and 3, the intima can appear irregular, suggesting an intimal tropism of histiocytes. When faced with this type of periaortic infiltration, physicians should consider the diagnosis of ECD. Indeed, this particular aspect seems to be specific to ECD, as it has not been reported in other forms of histiocytosis particularly, Langerhans cell histiocytosis.
Complete radiologic data for our personal cases reported here showed that the perivascular infiltration frequently spread around all aortic collaterals. This feature of ECD has been mentioned rarely in other cases, and probably has been overlooked3,66. Modern imaging techniques highlight the general perivascular tropism of ECD "fibrosis," which is clearly not limited to the aorta wall. This finding supports the hypothesis that the xanthogranulomatous process is diffused from the aorta to all aortic collaterals. The significant association found between cardiovascular involvement and extraskeletal manifestations of ECD is consistent with this hypothesis. Nevertheless, 2 of our personal cases displayed "isolated" infiltration of an aortic collateral in the absence of adjacent periaortic "fibrosis": Case 4 with abdominal periaortic "fibrosis" and distal infiltration around the left renal artery, sparing the ostium; and Case 6 with 2 distinct involved sites, the superior mesenteric artery and the aortic arch.
These findings stress the clinical consequences of cardiovascular involvement in ECD. Infiltration of arteries can lead to ischemic manifestations like myocardial infarction25, mesenteric angina18,69, or cerebral ischemia22,50,66. Renal artery stenosis caused hypertension in 6 patients1,18,44,69 including our Cases 1 and 2. Angioplasty and stenting normalized blood pressure in 4 of these cases, but follow-up data remain limited. Arterial infiltration was not always associated with clinical symptoms. Indeed, although all our personal cases had regular infiltration surrounding mesenteric superior artery, only Case 5 had stenosis and mesenteric angina. Similarly, neither of our 2 personal cases with pericoronary infiltration were symptomatic. Heart infiltration by ECD may affect the pericardium, myocardium, valves, and/or coronary arteries. Although frequent, pericardial infiltration with pericardial effusion sometimes leading to tamponade was recognized only recently as a feature of ECD. Clinicians should be aware of the possible occurrence of right atrial tumors, given that the usual means of diagnosing ECD does not involve heart biopsy. Heart involvement was found to be responsible for one-quarter of all deaths in our series.
Due to the major clinical implications of heart abnormalities and the fact that they were identified in 30 patients by echocardiography, we suggest that echocardiography should be performed systematically when evaluating patients with ECD, especially those with vascular involvement. The following echographic signs should be sought: presence of pericardial effusion or of a thickened pericardium, a thickened myocardium and the presence of a right atrial infiltration, the existence of mitral or aortic regurgitation, and infiltration of the aortic arch and descending aorta (the latter is most easily seen on transesophageal echocardiography). Although most of the cases with echocardiographic data reported in the literature focused on pericardial involvement, no data on cardiac hypertrophy or ventricular dilation were available (emphasizing the unrecognized status of these abnormalities). However, Cases 1 and 2 from the present series had mild septal hypertrophy, and Case 5 had mild concentric hypertrophy. None of our cases had ventricular dilation.
Venous manifestations were less frequent (7 cases, including personal Cases 2 and 3). However, unlike arterial involvement, it was generally due to a thrombotic complication of a venous compression by the xanthomatous process rather than to direct infiltration of the vessel wall.
ECD should be added to the list of diseases that physicians consider when faced with 1 of the above-mentioned clinical manifestations or radiologic aspects. However, most of these symptoms are common and unspecific when considered alone. As we found that cardiovascular manifestations of ECD are significantly associated with exophthalmos, neurologic involvement, and xanthelasma, the presence of any of these 3 should prompt clinicians to evoke the diagnosis of ECD.
Parietal aortic wall thickening with diffusion to the main aortic branches can be observed in Takayasu arteritis, which mainly affects young women. However, the radiologic findings of Takayasu arteritis and ECD are different (Table 4). The entire wall, that is, adventitia, media, and intima, is affected in Takayasu arteritis, whereas the adventitial and periadventitial periaortic spaces but not the wall itself are affected in ECD patients. Radiologic abnormalities can also be used to distinguish ECD from mediastinal and retroperitoneal fibrosis. Typically, retroperitoneal fibrosis is not circumferential and infiltrates the anterior and the lateral sides of the aorta, sparing the posterior side. Retroperitoneal fibrosis, but not ECD, may involve the inferior vena cava (which may be stenosed or occluded) or the pelvic ureters. Extravascular images observed in ECD and not in retroperitoneal fibrosis, such as bilateral infiltration of the perirenal space ("hairy kidneys" appearance), can be useful for differential diagnosis.
It is important to note that the concomitant presence of an acute inflammatory response is not helpful for the differential diagnosis because our 6 patients all exhibited elevated levels of acute-phase protein despite receiving high-dose steroid treatment. Strikingly, inflammation resolved after alpha interferon therapy. Although CRP levels decreased, periaortic infiltration remained largely unchanged. Numerous treatments for ECD have been proposed, including steroids78, multiple chemotherapy drugs65,69,70,73,78, alpha interferon therapy78, biphosphonate52, and radiation therapy48. It is difficult to evaluate these treatments, however, as most of them have been tested only in a small number of patients or in combination with other drugs. Furthermore, the follow-up period in most of these reports is too short to make it possible to assess their real long-term efficacy.
In conclusion, cardiovascular involvement of ECD is much more frequent than previously thought. Clinical manifestations, such as heart failure, valvular dysfunction, renovascular hypertension, and pericarditis possibly leading to tamponade, are encountered with variable frequencies. Cardiovascular involvement accounts for a significant proportion of the deaths associated with ECD, and thus should be sought systematically.
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