Clinical Significance of Myocardial Magnetic Resonance Abnormalities in Patients with Sarcoidosis: A 1-Year Follow-up Study

doi:10.1378/chest.122.6.1895

Chest

Volume 122, Issue 6, December 2002, Pages 1895-1901

https://doi.org/10.1378/chest.122.6.1895 Get rights and content

Purpose

To assess the follow-up of patients with sarcoidosis and myocardial MRI abnormalities.

Materials and methods

Twelve patients with histologically proven sarcoidosis and highly suspected cardiac involvement underwent initial and 12-month follow-up cardiac assessment including cardiac MRI (T2-weighted, functional gradient echo, and T1-weighted gadolinium-diethylenetriamine penta-acetic acid-enhanced sequences). MRI abnormalities and clinical and MRI progression were scored by two observers.

Results

Six patients receiving corticosteroid therapy (including three patients with clinical cardiac involvement) were scored as having cleared or improved at MRI follow-up, while others were seen to have worsened or remained stable. The stability, improvement, or clearing of MRI findings were correlated with clinically stable, improved or cleared sarcoidosis, while a worsening at MRI follow-up was correlated with a worsening of sarcoidosis and, in one patient, was predictive of clinical cardiac involvement.

Conclusion

Cardiac MRI is a useful noninvasive method for the early diagnosis and follow-up of cardiac sarcoidosis.

Section snippets

Study Population

Between October 1999 and September 2000, 20 patients with histologically proven, active sarcoidosis were referred for cardiac assessment. A diagnosis of sarcoidosis was confirmed if the clinical presentation and chest radiographic findings were supported by histologic evidence of noncaseous epithelioid granulomas by bronchial or salivary gland biopsy, and once the possibility of infection, environmental factors or medical treatment causing the granuloma had been eliminated. Other data collected

Clinical Data at Inclusion

The clinical findings concerning the 12 patients included in the study are summarized in Table 1. Eight patients had intrathoracic disease and the involvement of other organs. Two patients (patients 4 and 5) exhibited isolated intrathoracic involvement. One patient presented with Löfgren syndrome (patient 10), and one patient with myositis and intrathoracic involvement (patient 11). Sarcoidosis had been diagnosed only 1 year previously in seven patients, while disease duration ranged from 5 to

Discussion

The pathologic features of cardiac sarcoidosis include patchy infiltration of the myocardium with three successive histologic stages: edema, granulomatous infiltration, and fibrosis leading to postinflammatory scarring.² MRI provides high-resolution information for both morphology and function, rendering MRI an effective method for the assessment of myocardial diseases. The ability of MRI to diagnose sarcoidal heart disease has been emphasized in several case reports,⁷^,⁸^,⁹^,¹⁰^,¹¹^,¹² allowing

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La sarcoïdose est une maladie systémique granulomateuse, caractérisée par une atteinte médiastinopulmonaire, chez la majorité des patients et, plus rarement, par des atteintes extrapulmonaires, comme des atteintes oculaires, cutanées, des glandes exocrines, ganglionnaires et articulaires. Les atteintes neurologiques et cardiaques sont plus rares, mais font toute la gravité de la maladie. Les atteintes cardiaques concernent 5 à 20 % des patients, selon les séries, et touchent même plus de 25 % des patients dans les séries autopsiques. Cette revue vise à faire le point sur l’intérêt diagnostique des différentes techniques d’imagerie dans la sarcoïdose cardiaque et également détailler la prise en charge « interniste » et cardiologique de ces patients qui requièrent une prise en charge pluridisciplinaire.
Sarcoidosis is a systemic granulomatous disease characterized by pulmonary involvement in most patients and more rarely by extrapulmonary involvement such as ocular, skin, salivary, lymph nodes and joints damages. Neurological and cardiac involvements are uncommon but are associated with increased morbidity and mortality. Cardiac sarcoidosis affects 5 to 20% of patients depending on the studies and autopsy studies even report cardiac involvement in 25% of sarcoidosis patients. This review aims to summarise main data on the diagnostic value of the different imaging techniques in cardiac sarcoidosis and to also detail the management of these patients who require a multidisciplinary approach.
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La sarcoïdose est une granulomatose systémique caractérisée par la formation de granulomes épithélioïdes et gigantocellulaires sans nécrose caséeuse. Pour retenir son diagnostic, il faut faire la preuve d’une granulomatose systémique impliquant au moins deux organes ; en pratique, réunir un faisceau d’arguments cliniques, paracliniques et anatomopathologiques. Elle touche de façon prépondérante les femmes avec deux pics d’incidence : 25–29 ans et 65–69 ans. Les organes les plus fréquemment touchés sont le système lymphatique médiastinal, les poumons, la peau et les yeux. Une atteinte ophtalmologique est présente dans 20 à 50 % des cas. La présentation oculaire caractéristique est celle d’une uvéite granulomateuse, associée à des vascularites rétiniennes veineuses et des lésions de choroïdite multifocale périphérique. Cette présentation ophtalmologique, bien que très évocatrice, n’est pas toujours associée à une atteinte systémique. Le diagnostic de sarcoïdose oculaire reste alors présumé en l’absence de preuve histologique. Des algorithmes combinant signes ophtalmologiques et extraophtalmologiques ont été proposés en cas d’uvéites isolées. Ils permettent d’établir le diagnostic de sarcoïdose oculaire avec des niveaux de probabilité différents. L’absence de granulome au niveau systémique lors d’atteintes oculaires reste la principale hypothèse pour expliquer ces difficultés diagnostiques. Le traitement est bien codifié dans la mesure où l’uvéite de la sarcoïdose est le plus souvent corticosensible. En cas d’uvéite corticodépendante, l’immunosuppresseur de première intention reste le méthotrexate. Le recours aux anti-tumor necrosis factor-alpha est une alternative intéressante chez des patients dont la sarcoïdose oculaire est réfractaire aux immunosuppresseurs conventionnels.
Sarcoidosis is a systemic granulomatosis characterized by the formation of epithelioid and giant cell granulomas without caseous necrosis. To make the diagnosis, it is necessary to prove systemic granulomatosis involving at least two organs; but in practice, a combination of clinical, paraclinical and histologic findings is used. It affects predominantly women with a bimodal age distribution: 25–29 years and 65–69 years. The most commonly affected organs are the mediastinal lymphatic system, lungs, skin and eyes. Ophthalmological involvement is present in 20 to 50% of cases. The typical ocular presentation is that of granulomatous uveitis associated with venous retinal vasculitis and lesions of peripheral multifocal choroiditis. This ophthalmological presentation, although very evocative, is not always associated with systemic disease. The diagnosis of ocular sarcoidosis is then presumed in the absence of histological evidence. Algorithms combining ophthalmological and systemic signs have been proposed in cases of isolated uveitis. They make it possible to establish the diagnosis of ocular sarcoidosis with various levels of probability. The absence of significant granulomas on a systemic level during primary ocular involvement remains the main hypothesis to explain these diagnostic difficulties. Treatment is well described, as the uveitis of sarcoidosis is most often steroid responsive. In the case of corticosteroid-dependent uveitis, the first-line immunosuppressant remains methotrexate. The use of anti-tumor necrosis factor-alpha is an interesting alternative in patients whose ocular sarcoidosis is refractory to conventional immunosuppressants.

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Chest

Clinical Investigations: TechniquesClinical Significance of Myocardial Magnetic Resonance Abnormalities in Patients with Sarcoidosis: A 1-Year Follow-up Study

Purpose

Materials and methods

Results

Conclusion

Section snippets

Study Population

Clinical Data at Inclusion

Discussion

Am J Med

Am J Med

Am Heart J

Clin Chest Med

Chest

Clin Radiol

Clin Radiol

MAGMA

Am Heart J

Clinical Investigations: Techniques
Clinical Significance of Myocardial Magnetic Resonance Abnormalities in Patients with Sarcoidosis: A 1-Year Follow-up Study