Wrapped coronary arteries: a rare manifestation of retroperitoneal fibrosis.

Abstract

Dear Editor, We describe a young woman in whom first manifestation of idiopathic retroperitoneal fibrosis (IRPF) was coronary arterial involvement, which could not be diagnosed until typical symptoms related with urinary system involvement occurred. This is the first report in the literature demonstrating coronary arterial involvement as the first manifestation in IRPF. Clinicians should keep in mind that fibrosis and chronic inflammation associated with IRPF may involve coronary arteries and main branches of the arcus aorta, as well as the abdominal aorta, iliac arteries, before other systemic manifestations (particularly due to urinary system involvement) are established. Idiopathic retroperitoneal fibrosis is a rare collagen vascular disease of unknown etiology, characterized by the replacement of normal retroperitoneal tissue with fibrosis and/or chronic inflammation usually surrounding the abdominal aorta, iliac arteries and adjacent anatomic structures (e.g., ureters, inferior vena cava).1 Idiopathic disease most commonly occurs in individuals 40–60 years of age. The majority of studies have suggested a 2–3 : 1 male-to-female predominance,2 although this is not reported in all studies.1 Diagnosis is made following exclusion of secondary causes, including: drugs (ergot-derivatives, methysergide, bromocriptine, beta blockers, methyldopa, hydralazine, analgesics); malignancy (carcinoid, Hodgkin and non-Hodgkin lymphoma, sarcomas, colorectal, breast, prostate and bladder carcinoma); infections (tuberculosis, histoplasmosis, actinomycosis); radiation therapy; retroperitoneal hemorrhage or previous surgery.3, 4 The initial clinical features of retroperitoneal fibrosis are nonspecific and the patients are often asymptomatic until there is significant systemic involvement. Most patients have ureteral obstruction and renal impairment by the time they come to medical attention. With progression of fibrosis, many other systems, including the cardiovascular system, may be involved in the disease. Amiya et al.5 have reported a case with involvement of the aorta from aortic arch to aortic bifurcation. Maturen et al.6 have described two patients with perivascular low-attenuation soft tissue and inflammatory changes surrounding the coronary arteries in the clinical setting of idiopathic retroperitoneal fibrosis. In this case, we describe a young woman in whom first manifestation of IRPF was coronary arterial involvement, which could not be diagnosed until typical symptoms related with urinary system involvement occurred. This is the first report in the literature demonstrating coronary arterial involvement as the first manifestation in IRPF. A 30-year-old woman with no other systemic disease was referred to our hospital for further evaluation of recurrent angina. Coronary angiogram performed 2 months ago at another center due to her complaint of chest pain, revealed non-critical coronary artery disease. At the time of scheduled visit at our hospital, she also complained of severe new-onset unilateral flank pain and her serum biochemistry profile revealed impaired renal function. Examinations including anterograde pyelography and magnetic resonance imaging led to the diagnosis of retroperitoneal fibrosis (Fig. 1a,b). A comprehensive investigation for potential etiologies of retroperitoneal fibrosis was initiated. Laboratory findings of the patient are shown in Table 1. No history of drug intake related to retroperitoneal fibrosis, surgery, infection or radiotherapy was noted. Serum immunoglobulin G4 (IgG4) levels were not suggestive of IgG4-related sclerosing vasculitis. After diagnosis of IRPF at our hospital, the patient started receiving immunosuppressive therapy. During her follow-up, she had frequent unstable angina pectoris episodes. Repeated echocardiographic examinations during hospitalization revealed that ejection fraction declined from 51% to 38%. Coronary angiogram was performed following this finding and multivessel coronary artery disease was demonstrated. In order to elucidate the possible vasculitic nature of the disease, a coronary computed tomography angiogram was done. Fibrotic tissue with a maximal thickness of 5 mm, surrounding all coronary arteries and causing significant stenosis was shown (Fig. 1c,d). Patient was referred to the Department of Cardiovascular Surgery and three-vessel bypass grafting was subsequently performed. Intraoperatively, bundles of fibrosis surrounding the coronary arteries were removed and tissue samples from aorta and epicardial plaques surrounding the coronary arteries were obtained for hematoxylin and eosin (Fig. 1e), Masson's trichrome (Fig. 1f) and immunohistochemical staining. Mononuclear cell infiltration and dense fibrosis were detected in the epicardial plaque specimen. Immunohistochemical staining revealed the mononuclear cell population was composed primarily of CD2-positive (Fig. 1g), and to a lesser extent of CD20-positive cells (Fig. 1h). Rare plasma cells were determined by CD138-positive staining. Coronary arterial involvement as the first manifestation of retroperitoneal fibrosis is extremely rare. Clinicians should keep in mind that fibrosis and chronic inflammation associated with IRPF may involve coronary arteries and main branches of the arcus aorta, as well as the abdominal aorta and iliac arteries before other systemic manifestations (particularly due to urinary system involvement) are established. The authors report no relationships that could be construed as a conflict of interest.