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(Circulation. 2008;118:e511-e512.)
© 2008 American Heart Association, Inc.

Images in Cardiovascular Medicine

Pericarditis Heralding Erdheim-Chester Disease

Augusto Vaglio, MD; Domenico Corradi, MD; Roberta Maestri, PhD; Sergio Callegari, MD; Carlo Buzio, MD; Carlo Salvarani, MD

From the Department of Clinical Medicine, Nephrology and Health Science (A.V., C.B.), and Department of Pathology and Laboratory Medicine, Section of Pathology (D.C., R.M.), University of Parma, Parma, Italy; Division of Cardiology, Fidenza Hospital (S.C.), Fidenza, Italy; and Rheumatology, Arcispedale Santa Maria Nuova (C.S.), Reggio Emilia, Italy.

Correspondence to Dr Augusto Vaglio, Dipartimento di Clinica Medica, Nefrologia e Scienze della Prevenzione, Università degli Studi di Parma, Via Gramsci 14, 43100 Parma, Italy. E-mail augusto.vaglio{at}virgilio.it

A 47-year-old man with a history of gynecomastia due to prolactin-releasing pituitary microadenoma was admitted to the hospital because of persistent chest pain and fatigue. Chest x-ray showed pronounced enlargement of the cardiac profile (Figure 1), and echocardiography demonstrated a marked pericardial effusion (Figure 2). Pericardiocentesis allowed the drainage of 1800 mL of pericardial fluid whose biochemical analysis was consistent with an exudate (total protein and lactate dehydrogenase fluid-to-serum ratios of 0.9 and 2.6, respectively); cytological examination showed the presence of mesothelial cells, histiocytes, and lymphocytes.

View larger version (157K): [in this window] [in a new window]   Figure 1. Chest x-ray performed at the time of admission to hospital showing marked enlargement of the cardiac profile.

View larger version (75K): [in this window] [in a new window]   Figure 2. Transthoracic echocardiographic image, obtained on admission to hospital, showing large circumferential pericardial effusion (apical 4-chamber view). PE indicates pericardial effusion; LV, left ventricle; RV, right ventricle; LA, left atrium; and RA, right atrium.

Whole-body computed tomography disclosed the presence of a homogeneous mass of soft-tissue density coating the thoracic (Figure 3) and abdominal aorta (Figure 4) and also surrounding both kidneys (Figure 4). Computed tomography–guided biopsy of the perirenal masses was performed, and histology showed a diffusely fibrotic tissue with a population of infiltrating histiocytes, which stained positive for CD68 (Figure 5) and negative for S100 and CD1a (data not shown). These findings were consistent with Erdheim-Chester disease (ECD), a diagnosis that was also supported by the presence of osteosclerotic lesions involving the tibias.

View larger version (65K): [in this window] [in a new window]   Figure 3. Contrast-enhanced computed tomography scan of the chest showing the presence of muscle-isodense tissue surrounding the thoracic aorta, particularly the ascending portion (arrow).

View larger version (102K): [in this window] [in a new window]   Figure 4. Contrast-enhanced computed tomography scan of the abdomen showing muscle-isodense tissue encircling the abdominal aorta (arrow) and also surrounding both kidneys (arrowheads).

View larger version (88K): [in this window] [in a new window]   Figure 5. A, Low-power view of the needle biopsy specimen at the boundary between the renal cortical parenchyma (asterisk) and the perirenal retroperitoneal soft tissues. The latter are diffusely fibrotic and infiltrated by cells frequently organized in clusters (arrows). Hematoxylin-Eosin stain; original magnification x4; scale bar is 400 µm. B, The infiltrating elements (arrows) are mononuclear, show either polygonal or elongated shape, and diffusely permeate the collagen fibers (reddish bands). The mild inflammatory background is mainly composed of small lymphocytes (arrowheads). Hematoxylin-Eosin stain; original magnification x20; scale bar is 100 µm. C, Immunohistochemically, the mononuclear cells show strong cytoplasmic granular positivity for the CD68 antigen (brown color), thus indicating a histiocyte phenotype. Counterstained with Mayer hematoxylin, original magnification x20; scale bar is 100 µm.

ECD is a rare form of non-Langerhans cell histiocytosis whose hallmark is tissue infiltration by CD68⁺ CD1a^– foamy non-Langerhans histiocytes and mononuclear cells, along with pronounced fibrosis.¹ The mechanisms leading to accumulation of histiocytes in ECD lesions seem to be chemokine-mediated, although clonal histiocyte proliferation has also been demonstrated in some cases.²

ECD has a broad spectrum of clinical manifestations and may range from a localized pauci-symptomatic form to a disseminated and life-threatening disease; it typically affects the long bones, the hypothalamic-pituitary axis, the skin, the lungs, and the retroperitoneum. Cardiovascular involvement is considered to be an overlooked feature of ECD, although diffuse periaortic fibrosis (also known as "coated aorta"),³ pericardial infiltration with pericardial effusion, myocardial infiltration, atrial pseudotumour, and aortic and mitral valve disease are not uncommon clinical findings. A careful evaluation of cardiovascular involvement in ECD patients is warranted because it accounts for a considerable proportion of ECD-related deaths.⁴

	Acknowledgment

 
We gratefully acknowledge Dr Ariberto Corradi for performing the perirenal tissue biopsy.

Disclosures

None.

	References

Top References

 
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2. Stoppacciaro A, Ferrarini M, Salmaggi C, Colarossi C, Praderio L, Tresoldi M, Beretta AA, Sabbadini MG. Immunohistochemical evidence of a cytokine and chemokine network in three patients with Erdheim-Chester disease. Arthritis Rheum. 2006; 54: 4018–4022.[CrossRef][Medline] [Order article via Infotrieve]

3. Serratrice J, Granel B, De Roux C, Pellissier JF, Swiader L, Bartoli JM, Disdier P, Weiller PJ. "Coated aorta": a new sign of Erdheim-Chester disease. J Rheumatol. 2000; 27: 1550–1553.[Medline] [Order article via Infotrieve]

4. Haroche J, Amoura Z, Dion E, Wechsler B, Costedoat-Chalumeau N, Cacoub P, Isnard R, Généreau T, Wechsler J, Weber N, Graef C, Cluzel P, Grenier P, Piette JC. Cardiovascular involvement, an overlooked feature of Erdheim-Chester disease: report of 6 new cases and a literature review. Medicine (Baltimore). 2004; 83: 371–392.[CrossRef][Medline] [Order article via Infotrieve]

This Article Extract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Alert me to new issues of the journal Download to citation manager Request Permissions Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Vaglio, A. Articles by Salvarani, C. Search for Related Content PubMed Articles by Vaglio, A. Articles by Salvarani, C. Related Collections Pericardial disease CT and MRI Computerized tomography and Magnetic Resonance Imaging Vasculitis Brain Arteries