The ECG in sarcoidosis – a marker of cardiac involvement? Current evidence and clinical implications

Sarcoidosis is a multisystem granulomatous disease of unknown etiology characterized by noncaseating granulomas. Cardiac involvement is often limiting patients’ prognosis. Cardiac sarcoidosis can manifest with variant cardiac arrhythmias, of which atrioventricular (AV)-block-related bradycardia and ventricular tachycardias are the most common. Although cardiac sarcoidosis remains a histopathological diagnosis, the significance of imaging modalities, especially cardiac magnetic resonance imaging is increasing rapidly but mainly remains reserved for patients with a high suspicion due to a previous arrhythmia or unknown cardiomyopathy. Thus, there is a need for screening in daily clinical practice so that possible characteristic electrocardiographic (ECG) findings may guide the way to detect the disease.We therefore evaluated the ECG as a potential tool for screening of cardiac sarcoidosis and present different electrophysiological manifestations of cardiac sarcoidosis based on a literature review.The ECG is a valuable tool for screening of cardiac involvement in patients with sarcoidosis. Several parameters have been shown to be associated with cardiac involvement in sarcoidosis such as higher-degree AV-block, QRS complex fragmentation and widening, as well as certain T wave abnormalities that may indicate cardiac involvement, of which the latter two are most promising and specific. However, prospective studies examining a large number of trials are desirable.     

Multimodality Imaging in Cardiac Sarcoidosis: Is There a Winner?

Sarcoidosis is a multisystem granulomatous disease of unknown cause that can affect the heart. Cardiac sarcoidosis may be present in as many as 25% of patients with systemic sarcoidosis, and it is frequently underdiagnosed. The early and accurate diagnosis of myocardial involvement is challenging. Advanced imaging techniques play important roles in the diagnosis and management of patients with cardiac sarcoidosis.     

Sarcoïdose cardiaque : avancées diagnostiques et thérapeutiques

Sarcoidosis is a granulomatous disorder of unknown cause characterized by non-caseating granuloma in young adults. Cardiac involvement is rare and range from 2 to 75% depending on diagnostic criteria. Cardiac involvement in sarcoidosis may be asymptomatic or may manifest as rhythm/conduction troubles or congestive heart failure. The diagnosis and treatment of cardiac sarcoidosis may be challenging. However, advances have come in recent years from the use of cardiac MRI and ¹⁸FDG-TEP scanner, as well as from the stratification of the risk of ventricular tachycardia/fibrillation. Due to the rarity of the disease, there is no reliable prospective large study to guide therapeutic strategy for cardiac sarcoidosis. Corticosteroids are probably efficacious, in particular in case of atrio-ventricular block or moderate heart failure. Immunosuppressive drugs have not been largely studied but methotrexate could be helpful. In refractory forms, TNF-α antagonists have been used with success.     

Sarcoidosis masquerading as an acute coronary syndrome

Sarcoidosis is a common multisystem granulomatous disease that affects 20 per 100,000 people in the world. Although primarily a pulmonary disease, sarcoidosis can affect multiple organ systems. Cardiac involvement of sarcoidosis is most commonly manifested as ventricular ectopy and arrhythmias; atrioventricular conduction disturbances, including complete heart block; congestive heart failure; and even may result in sudden death. Although myocardial infarction is most commonly the result of epicardial coronary artery disease, an infiltrative process such as sarcoidosis may simulate an acute coronary syndrome. Given the high prevalence of sarcoidosis, it is important to be aware of the myriad of cardiac manifestations of this infiltrative process.     

Cardiac sarcoidosis

The present report describes a patient with cardiac sarcoidosis who developed complete right bundle branch block, complete atrioventricular block and subsequent congestive heart failure. The patient demonstrated no clinical evidence of systemic sarcoidosis. Upon postmortem examination, the myocardium showed extensive noncaseating granuloma with numerous multinucleated giant cells. An initial routine microscopic examination of the lung revealed no evidence of granulomatous lesions. However, an extensive microscopic examination of the lung using serial sections demonstrated inconspicuous granulomatous lesions with giant cells. Thereby, a diagnosis of sarcoidosis was made. All other organs were free of granulomatous inflammation in spite of an extensive microscopic examination through serial sections. The present case suggests that a careful and extensive microscopic examination of the other organs may be necessary to establish a diagnosis of cardiac sarcoidosis.     

Pathology of sarcoidosis

The role of pathology in the diagnosis of sarcoidosis is identification of granulomas in tissue specimens and performance of studies to exclude known causes of granulomatous inflammation. The granulomas of sarcoidosis are nonspecific lesions that, by themselves and in the absence of an identifiable etiologic agent, are not diagnostic of sarcoidosis or any other specific disease. Among the diseases to be excluded are mycobacterial, fungal, and parasitic infections, chronic beryllium disease and other pneumoconiosis, hypersensitivity pneumonitis, and Wegener's granulomatosis. Even after extensive workup a substantial number of granulomas will remain unclassified. Not every disease that features nonnecrotizing granulomas of undetermined etiology is sarcoidosis. The granulomas of sarcoidosis may exhibit focal necrosis of minimal amount. In cases with granulomas that exhibit a greater degree of necrosis an infectious or other nonsarcoid etiology should be strongly suspected. Strict clinical, radiological, and pathological criteria must be used for diagnosis. In cases that exhibit necrotizing granulomas with more than minimal, focal necrosis, extrathoracic involvement only, and/or incompatible clinical and radiological findings, the diagnosis of sarcoidosis should be approached with great caution. The diagnosis is most secure when compatible clinical and radiological findings are supported by the demonstration of microorganism-negative, nonnecrotizing granulomas in a biopsy specimen accompanied by biopsy evidence or strong clinical evidence of multisystem involvement, and negative cultures for bacteria, mycobacteria, and fungi. A positive Kveim-Siltzbach test provides strong support for the diagnosis of sarcoidosis.     

Extrapulmonary sarcoidosis of liver and pancreas： A case report and review of literature

Sarcoidosis is a chronic multisystemic granulomatous disease of unknown origin, which can involve nearly all organs. In the case of an infrequent gastrointestinal tract involvement in systemic sarcoidosis, granulomas of the liver are most commonly described while isolated pancreatic sarcoid lesions are rarely seen. We report a case of systemic sarcoidosis with exclusive extrapulmonal involvement of the liver and the pancreas in a 71-year- old white man. The diagnosis of liver involvement was confirmed by biopsy. Pancreatic surgery was needed because preoperative evaluation could not exclude pancreatic cancer and for biliary decompression. An extensive literature review of systemic sarcoidosis, focusing on reported cases with unusual presentation of sarcoidosis in the liver and the pancreas, its diagnosis, treatment, and prognosis was made.     

The diagnosis of sarcoidosis

The diagnosis of sarcoidosis can never be assured: sarcoidosis is a diagnosis of exclusion and this cannot be accomplished with complete confidence. The diagnosis requires clinicoradiographic findings compatible with the diagnosis, histologic confirmation of granulomatous inflammation, exclusion of known causes of granulomatous disease, and evidence of disease in at least two organs. The end result of this diagnostic evaluation for sarcoidosis is neither a definitive diagnosis nor an exclusion of the diagnosis, but rather a statistical likelihood of the disease.     

The anti-arrhythmic effects of prednisone in patients with sarcoidosis

Atrial fibrillation (AF) affects 2.3 million people in the United States and is currently the most common cardiac arrhythmia. Its overall prevalence is only increasing as the population ages. The classical risk factors for developing AF include hypertension, valvular disease, ischemic cardiomyopathy, and thyroid disease. In some patients with AF, an underlying cardiovascular pathology is not identified and the etiology remains unknown. Treatment modalities for AF typically include rate control medications, antiarrhythmics and radio frequency ablation (RFA), each of which is accompanied by its own risk of complications. We report a case of symptomatic AF that was refractory to multiple antiarrhythmics and an RFA procedure which resolved with prednisone. In this case, AF was associated with cardiac sarcoidosis, a disorder that is thought to be due to granulomatous involvement of the myocardium and increased systemic inflammation.     

Value of MRI for the diagnosis of cardiac involvement in sarcoidosis

PURPOSE: The aim of the study was to assess the place of cardiac Magnetic Resonance Imaging (MRI) in patients with sarcoidosis with or without cardiac involvement. MATERIALS AND METHODS: Fifty patients with histologically-proven sarcoidosis underwent initial cardiac evaluation including MRI, ECG, holter ECG, echocardiography. Seven of them had cardiac involvement (cardiac insufficiency, auriculo-ventricular block, bundle-branch block). Fiveteen patients had a second evaluation at 10-month follow-up. MRI was classified in three stages, on the base of literature data (stage 1 "granulomatous", stage 2 "exsudative", stage 3 "fibrotic"). RESULTS: A good correlation between the type of the sarcoidosis and MRI was observed: patients with cardiac involvement had all stage 2 MRI; patients with quiescent sarcoidosis had normal or stage 3 MRI; patients without cardiac involvement had all stages on MRI. A good correlation was observed between cardiac MRI abnormalities and evolution of sarcoidosis. Patients under corticoid, with or without cardiac involvement all had regression of MRI lesions and sarcoidosis. In 2 cases, MRI was predictive of clinical cardiac involvement. CONCLUSION: Cardiac MRI is a useful non-invasive method for the early diagnosis and follow-up of cardiac sarcoidosis.     

Imaging in sarcoidosis

Sarcoidosis is a multisystemic granulomatous disease of unknown etiology that may involve virtually any organ. Pulmonary involvement predominates, but sarcoidosis can involve multiple organs, with or without concomitant lung involvement. Aberrations on chest radiographs are present in more than 90% of patients with sarcoidosis. Bilateral hilar lymphadenopathy, with or without lung parenchymal infiltrates, is typical but a wide range of chest radiographic patterns may be observed. This article discusses the characteristic chest radiographic features of sarcoidosis and the prognostic value of the radiographic staging classification as espoused by Scadding more than 4 decades ago. Thin-section high-resolution computed tomographic (HRCT) scans more clearly elucidate the intrathoracic lesions observed in sarcoidosis and may discriminate active inflammation from end-stage fibrosis. Although HRCT is not necessary to manage all cases of sarcoidosis, HRCT may be invaluable in SELECTED patients with stage II or III sarcoidosis to discriminate alveolitis (which may be amenable to therapy) from fibrosis. Additionally, radionuclide techniques may have a role in extrapulmonary sarcoidosis (particularly when central nervous system or cardiac involvement is suspected). We review the salient features and role of magnetic resonance imaging and diverse radionuclide techniques to diagnose or follow selected cases of extrapulmonary sarcoidosis.     

Sarkoidose

The most common manifestations of sarcoidosis, a systemic granulomatous disease of unknown etiology, involve the lungs but all organs may be affected. For diagnosis the typical clinical radiological picture should be supported by the histological confirmation of granuloma but this is not necessary for acute sarcoidosis (Löfgren’s syndrome). The long-term prognosis of sarcoidosis is generally good and depends on the various organ manifestations. The lungs and mediastinal lymph nodes, followed by the skin and eyes are most commonly affected but cardiac or central nervous sarcoidosis is a critical organ involvement. The cornerstone of treatment is corticosteroids but other immunosuppressants can be used to decrease the minimal required dosage of steroids. In rarely seen treatment refractory patients TNF-alpha inhibitors may be effective. Continuous monitoring by a pneumologist during the course of the disease is recommended.     

Dysfonction sinusale au cours de la sarcoïdose cardiaque: à propos d’un cas

IntroductionSarcoidosis is a multisystemic granulomatous disease of unknown cause occurring in young adults. Cardiac sarcoidosis patients are at increased risk for atrioventricular blocks and ventricular arrhythmias. Sinus node dysfunction is scarcely reported.ObservationWe report a case of cardiac sarcoidosis revealed by a sinus node dysfunction and focus on cardiac and thoracic imaging to guide diagnosis.ConclusionSinus node dysfunction may be the first manifestation of cardiac sarcoidosis. In unexplained sinus node dysfunction in young patients, advanced cardiac imaging is a key to cardiac sarcoidosis diagnostic. Early recognition of cardiac sarcoidosis enables to start immunosuppressive treatment and discuss implantable cardioverter defibrillator implantation.     

Isolated muscular sarcoidosis mimicking a tumoral lesion

We report a case of a woman with a palpable painful nodule on her left leg. MR and CT showed a lesion that could be described as a neoplasm. Excisonal biopsy revealed a noncaseating granuloma. The woman presented the nodular type of muscular isolated sarcoidosis. Further the disease involved the lungs; this confirmed the accurate diagnosis of sarcoidosis. Sarcoidosis is a chronic, multisystem granulomatous disease of unknown etiology. Muscle involvement is frequent, but often asymptomatic. There are three forms of muscular sarcoidosis: only the nodular type can be recognized by technical imaging. MR and ultrasound are the best methods to attempt the diagnosis of nodular muscular sarcoidosis; nevertheless, the lesion must have a standardized behaviour because it can mimic a malignant neoform. In this case, biopsy is the only tool to identify the disease.     

Sarcoïdose cardiaque : stratégies diagnostiques et thérapeutiques actuelles

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.     

Kardiale Sarkoidose

Sarcoidosis is a systemic granulomatous disease of unknown etiology that is histologically characterised by non-caseating granulomas. Hilar lymph nodes, lung, eyes and skin are the organs most commonly affected, although the heart, liver, spleen and bones may also be involved. Cardiac involvement is relatively rare and is associated with a poor prognosis. In addition to clinically manifest heart failure, the most common clinical manifestations are arrhythmias and sudden cardiac death. The absence of clinical symptoms does not rule out cardiac sarcoidosis, because most cases are detected as late as at necropsy. The diagnosis is based on a series of diagnostic approaches, such as electrocardiography, echocardiography, radionuclide imaging, magnetic resonance imaging, coronary angiography, endomyocardial biopsy and electrophysiological testing. However, the test results often do not allow the diagnosis to be made, and response to therapy is the only confirmation of the anticipated diagnosis. Various therapeutic modalities include treatment with immunosuppressive agents and specific cardiac medication, and implantation of a pacemaker or an implantable cardioverter defibrillator. If these methods fail to succeed, heart transplantation remains the therapy of choice.     

Sarcoidosis manifesting as cardiac sarcoidosis and massive splenomegaly

Sarcoidosis is a multisystemic granulomatous disease of unknown etiology. We report an unusual case of sarcoidosis in a woman presenting with cardiac sarcoidosis and massive splenomegaly with a familial history of cardiac sarcoidosis. Cardiac sarcoidosis was diagnosed based on electrocardiogram, echocardiogram, 18F-fluoro-2-deoxyglucose positron emission tomography (18F-FDG-PET) and skin histological findings. We performed splenectomy to rule out malignant lymphoma, and histological findings confirmed sarcoidosis. After splenectomy, we initiated prednisolone therapy. After 20 months of diagnosis, she was symptom free. Echocardiography and 18F-FDG-PET may be a key diagnostic tool and prednisolone therapy may be safe, effective, and feasible for cardiac sarcoidosis.     

Cardiac sarcoidosis responsible for localized left ventricular ectasia and refractory ventricular tachycardia. Anatomoclinical study

The authors report the case of a 63 year old woman admitted to hospital for recurrent refractory ventricular tachycardia. Echocardiography and cardiac scintigraphy showed global left ventricular function. Ventriculography confirmed the left ventricular dysfunction and also showed a localised aneurysm of the anterior left ventricular wall. Surgical resection of the aneurysm and an encircling endocardial ventriculotomy were performed but the patient died of a low output syndrome. Pathological examination of the excised tissue showed granulomatous lesions associated with fibrosis interrupting the striated myocardial bundles. The granulomata consisted in a large number of epithelioid histiocytes and very large giant cells with many nuclei. The diagnosis made retrospectively was that of cardiac sarcoidosis causing a ventricular aneurysm and global left ventricular dysfunction. The diagnosis of cardiac sarcoidosis is difficult in the absence of systemic extracardiac involvement because the clinical manifestations and complementary investigations are non specific. The diagnosis may be made by endomyocardial biopsy in 25 per cent of cases, thereby leading to specific treatment with steroids which is sometimes effective.     

Isolated renal sarcoidosis: a rare presentation of a rare disease treated with infliximab

Sarcoidosis is a multisystem disease characterized by noncaseating granulomatous reaction frequently involving the lymph nodes, lungs, liver, skin, and eyes. Acute renal failure (ARF) as an isolated manifestation of sarcoidosis is rare. We describe a case of sarcoidosis presenting as transient polyarthritis and ARF due to isolated granulomatous infiltration of the renal parenchyma. Renal biopsy showed granulomatous interstitial nephritis with noncaseating granulomas consistent with sarcoidosis. Bacterial, fungal, and mycobacterial infections were excluded. There was no evidence of extrarenal sarcoid involvement. Prednisone of 60 mg daily resulted in significant improvement in renal function. Because of recurrent flares on steroid taper and steroid toxicity, treatment with infliximab, an anti-tumor necrosis factor-α (TNF-α) antibody, was instituted and resulted in stabilization of renal function despite steroid taper. Although uncommon, renal sarcoidosis should be considered in the differential diagnosis of acute or chronic renal failure of uncertain etiology, as early diagnosis and treatment can lead to recovery of renal function and prevent interstitial fibrosis. Corticosteroids are mainstay of therapy. Steroid-dependant or refractory cases may respond to other immunosuppressants including anti-TNF-α agents.     

Nasopharyngeal sarcoidosis: a rare involvement

Sarcoidosis is a chronic, multisystemic, non-caseating granulomatous disease of unknown etiology. Nasopharyngeal involvement is very rare in sarcoidosis. The objective of this report was to evaluate a rare involvement of sarcoidosis. This report includes a case of nasopharyngeal sarcoidosis. A 51-year-old female with nasopharyngeal sarcoidosis was treated as sarcoidosis, and she was better. Nasopharyngeal involvement is very rare in sarcoidosis but it must be kept in mind.