Impact of cardiac magnetic resonance imaging for assessment of Churg-Strauss syndrome: a cross-sectional study in 20 patients.

OBJECTIVE: To examine the diagnostic contributions of cardiac magnetic resonance imaging (CMRI) with delayed-enhancement (DE) in patients with Churg-Strauss syndrome (CSS). METHODS: We consecutively recruited 14 men and 6 women (mean age: 50+/-14 years) with CSS (mean disease duration: 4.5+/-3.6 years) and investigated them independently of the presence/absence of cardiac manifestations. Cardiac manifestations included heart failure in 6 patients, angina pectoris in 1, isolated ECG abnormality in 1, and isolated echocardiography and ECG abnormalities in 1. T1-weighted sequences were recorded after gadolinium injection to study myocardial DE. RESULTS: CMRI abnormalities were found in 13/20 patients, including all 9 patients with myocardial manifestations, and 4 of the 11 asymptomatic patients. DE was centromyocardial in 6 patients, subepicardial in 4, and subendocardial in 3. Most enhanced lesions were in the anteroseptal or lateral walls. Patients with myocardial symptoms and DE had higher transmyocardial wall DE scores (mean: 9.4 vs. 3.7, respectively; p=0.01) and lower left ventricular ejection fractions (mean: 42% vs. 59%; p=0.001) than asymptomatic patients with DE. CONCLUSION: CMRI with DE enabled the detection of myocardial involvement in CSS patients with or without clinical symptoms. The clinical relevance of CMRI abnormalities in patients without clinical, echocardiographic and ECG signs of cardiac involvement remains unknown and needs to be evaluated in future studies. It seems premature to intensify treatment or to prescribe systematically steroids and cytotoxic agents based on the presence of isolated CMRI anomalies.

[Cushing's syndrome and adrenal adenoma. Two surprising associations]. / Maladie de Cushing et adénome cortico-surrénalien. Deux associations étonnantes.

INTRODUCTION: Cushing's syndrome has a very low incidence (1-10 cases/million/year), and familial cases are even more rare. We report on two situations involving different causes of Cushing's syndrome. CASES: In the first case, we describe the case of a patient with an adrenal adenoma 20 years before the occurrence of Cushing's disease related to the pineal gland. In the second case, two members of the same family were diagnosed almost simultaneously with adrenal cortical adenoma (mother) and Cushing's disease (daughter). DISCUSSION: These cases lead us to consider the known causes of familial Cushing's syndrome, which were not found here.

Association of peripheral multifocal choroiditis with sarcoidosis: a study of thirty-seven patients.

OBJECTIVE: To assess the clinical spectrum of peripheral multifocal choroiditis (PMC) and its association with sarcoidosis. METHODS: Thirty-seven patients examined between November 1997 and November 2001 who met all diagnostic criteria for PMC were included in this retrospective study. Patients were assessed for the following signs of sarcoidosis: typical changes on chest radiography or computed tomography; predominantly CD4 lymphocytosis in bronchoalveolar lavage fluid; elevated serum angiotensin-converting enzyme levels; elevated gallium uptake; and noncaseating granuloma on biopsy. RESULTS: Most of the patients were female (30 of 37; 81%) and white (30 of 37; 81%). Mean +/- SD age at onset was 57.5 +/- 18.7 years. Seven (19%) of the 37 patients had biopsy-proven sarcoidosis and 18 patients (49%) with presumed sarcoidosis met at least 2 of the above-mentioned criteria for sarcoidosis but had normal biopsy results. Twelve patients (32%) had an indeterminate diagnosis. Patients with presumed sarcoidosis did not differ from those with proven sarcoidosis as regards the above-mentioned criteria, except for noncaseating granuloma, implying that more than two-thirds of patients (predominantly whites) had underlying sarcoidosis. Most patients with positive gallium scintigraphy had increased mediastinal uptake, as described in sarcoidosis. Patients with underlying sarcoidosis had more severe visual impairment due to cystoid macular edema (CME). Weekly methotrexate (0.3 mg/kg) seemed to control CME. CONCLUSION: White patients with PMC should be considered to have sarcoidosis. The identification of sarcoidosis in patients with severe ocular disease can help with therapeutic choices.

Hepatofugal portal flow in advanced liver cirrhosis with spontaneous portosystemic shunts: effects on parenchymal hepatic enhancement at dual-phase helical CT.

BACKGROUND: We investigated whether spontaneous hepatofugal portal flow in advanced cirrhosis affects hepatic enhancement on dual-phase helical computed tomography. METHODS: Fifteen patients with hepatofugal portal flow on Doppler sonography and angiography (group 1) and 15 age- and sex-matched patients with hepatopetal portal flow (control; group 2) underwent dual-phase helical computed tomography. Vascular and liver attenuation values were measured on unenhanced scans and scans obtained during hepatic arterial (HAP; 25 s) and portal venous (PVP; 70 s) phases. RESULTS: Portal vein enhancement was lower during PVP in group 1 than in group 2 ( p < 0.0001). Liver enhancement was higher during HAP and lower and delayed during PVP in group 1 versus group 2 ( p < 0.0001). CONCLUSION: In hepatofugal portal flow, liver enhancement is increased during HAP and compensating for decreased and delayed liver enhancement during PVP, resulting in potential decreased hypervascular tumor conspicuity.

Peliosis hepatis: triphasic helical CT and dynamic MRI findings.

We report the triphasic helical computed tomographic (CT) and dynamic magnetic resonance imaging (MRI) findings in a unique case of biopsy-proven peliosis of the liver. Several reports have described the CT and MRI findings of this entity without specific appearance. This report discusses the usefulness of dynamic helical CT and MRI for the early diagnosis of peliosis.

[Non-invasive imaging of atherosclerosis by MRI and ultrasonography]. / Imagerie non invasive de l'athérosclérose en IRM et échographie.

The concept of the unstable, so-called "high risk" or vulnerable atheromatous plaque has been accepted for several years and its biological, mechanical and cellular features (collagen top, lipid core with macrophages, metalloproteinases...) have been determined. However, the means of investigating these lesions remain limited. A description of their composition has become essential as the prognosis of coronary, aortic and carotid artery disease is no longer evaluated only by morphometric data (degree of stenosis). MRI with T1 or T2 contrast sequences, density of spin, chemical shift, diffusion, magnetisation transfer or spectroscopy provides a non-invasive tool for their study. This method, based on the detection of magnetic atomic nuclei (such as proton H1, carbon 13, sodium 23, potassium 49) enables tissue characterisation by means of the variations in chemical environment and their consequences on the degree of magnetisation of the nuclei studied. High frequency ultrasound could also be used to this end by measuring the coefficients of attenuation and back-scattering. These methods, used clinically and in different animal models, should improve our understanding of the physiopathology, facilitate diagnosis and improve the prognostic accuracy in patients with atherosclerosis.