cardiovascular-agents and Sarcoidosis

Cardiac sarcoidosis usually occurs in the context of systemic disease; however, isolated cardiac involvement can occur in up to 25% of cases and tends to be clinically silent. When symptoms are present, they are often nonspecific and occasionally fatal, representing a diagnostic challenge. A high index of clinical suspicion and the integration of appropriate imaging, laboratory, and pathologic findings is always required. Treatment aims to control the systemic inflammatory condition while preventing further cardiac damage. However, even with adequate diagnosis and treatment strategies, prognosis remains poor. We describe the case of a patient who presented with cardiac symptoms, whose initial examination was unrevealing. Diagnosis was made retrospectively based on later systemic manifestations that revealed characteristic sarcoidosis findings.

Topics: Cardiomyopathies; Cardiovascular Agents; Drug Therapy, Combination; Female; Humans; Immunosuppressive Agents; Losartan; Methotrexate; Metoprolol; Middle Aged; Prednisone; Sarcoidosis

Cardiac involvement is undeniably one of the most challenging issues in sarcoidosis. Although autopsy studies reveal heart lesions in 20 to 30% of sarcoid patients, fewer than 5% suffer from clinical disease. Cardiac sarcoidosis (CS) has a predilection for the myocardium, but the pericardium and endocardium may also be affected. CS manifestations are various and most frequently include the following: (1) aberrations of atrioventricular or intraventricular conduction, either silent or symptomatic; (2) ventricular arrhythmias; (3) subacute congestive heart failure; and (4) sudden death. CS must be detected in all sarcoid patients by means of detailed medical history, physical examination, and resting electrocardiogram (ECG) at first evaluation and during follow-up. In patients with suspected CS, further investigations are aimed at evaluating diagnosis and cardiac consequences. Unfortunately, no gold standard exists that would allow CS diagnosis with a level of confidence. Endomyocardial biopsy is an invasive procedure that lacks sensitivity. Patients need, at a minimum, specialized cardiologic advice, echocardiography, and 24-hour ambulatory ECG. Other diagnostic tools include thallium, technetium, and gallium scintigraphy, and more recently, 18F-fluorodeoxyglucose positron emission tomography and cardiac magnetic resonance (CMR). The respective role of these new imaging tools in the diagnostic approach remains to be defined. CMR has the advantage of not exposing patients to radiation, but it is not feasible in those with cardiac devices. In Western countries, heart involvement accounts for 13 to 25% of sarcoidosis-related deaths, and it is the leading cause of mortality in Japan. The main prognostic indicators are New York Heart Association functional class, left ventricular enlargement, and sustained ventricular tachycardia. Treatment is based on systemic corticosteroids with an initial dose between 30 mg/day and 1 mg/kg/day (which is usually maintained for at least 24 months), specific cardiologic agents, and the placement of a pacemaker or implantable cardiac defibrillator in case of an atrioventricular block or severe intractable ventricular arrhythmias. Cardiac transplantation is exceptionally required.

Topics: Biopsy; Cardiovascular Agents; Echocardiography; Electrocardiography, Ambulatory; Glucocorticoids; Heart Diseases; Heart Transplantation; Humans; Magnetic Resonance Imaging; Positron-Emission Tomography; Sarcoidosis

A cardiac localization is one of the most severe manifestations of sarcoidosis and may cause sudden death (ventricular tachycardia or atrial ventricular block III) or restrictive cardiomyopathy. Lesions are most frequently observed in the interventricular septum and the free left wall. Granulomatous infiltation can provoke nonspecific clinical, electric and echocardiographic signs, which, associated with regressive dipyridamol uptake on tomoscintigraphy, are suggestive of cardiac sarcoidosis. The diagnosis of cardiac sarcoidosis is based on the presence of systemic sarcoidosis, histological evidence of granuloma and the lack of another cause of cardiomyopathy. Corticosteroid therapy is indicated, associated with specific cardiologic treatments.

Topics: Anti-Inflammatory Agents; Biopsy; Cardiomyopathies; Cardiomyopathy, Restrictive; Cardiovascular Agents; Death, Sudden, Cardiac; Dipyridamole; Echocardiography; Electrocardiography; Heart Block; Humans; Immunosuppressive Agents; Magnetic Resonance Imaging; Sarcoidosis; Steroids; Tachycardia, Ventricular; Vasodilator Agents

Topics: Adrenal Cortex Hormones; Biopsy; Cardiomyopathies; Cardiovascular Agents; Catheter Ablation; Combined Modality Therapy; Cyclophosphamide; Defibrillators, Implantable; Electrocardiography; Heart Septum; Humans; Hypertrophy, Right Ventricular; Lymph Nodes; Magnetic Resonance Imaging; Male; Middle Aged; Sarcoidosis; Sarcoidosis, Pulmonary; Syncope; Tachycardia, Ventricular; Ultrasonography

This retrospective study concerned 18 female and 23 male patients with cardiac sarcoidosis (CS). The average age at CS diagnosis was 38 years. CS was observed in white (73% of cases) and in black or Caribbean patients (27% of cases). All patients had extracardiac histologic proof of sarcoid tissue. In 63% of cases, the CS arose during the follow-up of systemic sarcoidosis. Systemic sarcoidosis was not specific except for a high frequency of neurosarcoidosis. Revealing cardiac signs were clinical in 63% of cases and electrical in 22%. In most patients these signs were associated with an abnormal echocardiography (77%) and/or a defect on thallium-201 or sestamibi imaging (75%). Thirty-nine patients received steroid therapy (initial dose mostly equal to 1 mg/kg per day), associated in 13 cases with another immunosuppressive treatment. In 26% of cases the immunosuppressive treatment was associated with a specific cardiac treatment. In the long-term follow-up (average follow-up, 58 mo), 87% of the cases showed an improvement, and 54% were cured from a clinical and laboratory point of view (electrocardiogram, 24-hour monitoring, echocardiography, radionuclide imaging). There was no sudden death. Two patients worsened, which can be explained in 1 case by very late treatment and in the other case by lack of treatment, except for a pacemaker. Our experience leads us to treat CS with corticosteroids as soon as possible and to use another immunosuppressive treatment where there is an insufficient therapeutic response or where there are contraindications to corticosteroids.

Topics: Adolescent; Adult; Aged; Alkaline Phosphatase; Biopsy; Black People; Blood Cell Count; Blood Sedimentation; Cardiomyopathies; Cardiovascular Agents; Echocardiography; Electrocardiography; Female; Humans; Hypercalcemia; Immunosuppressive Agents; Male; Middle Aged; Peptidyl-Dipeptidase A; Retrospective Studies; Sarcoidosis; Treatment Outcome; White People

Long-term (3-25 years) responses to treatment are available for 1820 patients with respiratory sarcoidosis. 65.7% of them were females and 34.3% males primarily at the age of 21-50 (92.7%). Sarcoidosis of the intrathoracic lymph nodes (ITLN), sarcoidosis of the lungs and ITLN, diffuse forms of pulmonary sarcoidosis, generalized sarcoidosis were diagnosed in 64, 28.2, 4.6, 2.6% of patients, respectively. All the patients received combined pathogenetic treatment (hormones, antioxidants, antihypoxants, immunomodulators, angio- and hepatoprotectors) which proved highly long-acting in stage I-II (a complete response rate 84.3% and 82.7%, respectively, residual changes remained in 1/5 of the patients). Long-term recurrences occurred in 3.5% of patients who had marked residual changes in the lungs. The disease took a chronic course in 8.7%, progression was recorded in 9.3, lethal outcome in 0.7% of the cases.

Topics: Adjuvants, Immunologic; Adolescent; Adult; Aged; Antioxidants; Cardiovascular Agents; Combined Modality Therapy; Female; Follow-Up Studies; Hormones; Humans; Lung Diseases; Lymphatic Diseases; Male; Middle Aged; Prognosis; Radiography; Sarcoidosis; Time Factors