Cardiac sarcoidosis: phenotypes, diagnosis, treatment, and prognosis

Lehtonen, Jukka; Uusitalo, Valtteri; Pöyhönen, Pauli; Mäyränpää, Mikko I.; Kupari, Markku

doi:10.1093/eurheartj/ehad067

Cited by 59 publications

(58 citation statements)

References 176 publications

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“…It is characterised by a dysregulated T cell-driven immunologic response and activation of macrophages that accumulate in the affected organs. Researchers hypothesise that the pathogenesis is related to an exaggerated immune response to an environmental factor, infectious agent or antigen in a genetically susceptible individual [ 1 ] . Some clinical cases have been published reporting a correlation of sarcoidosis and COVID-19 vaccination, which may have been the trigger for our patient’s exacerbation since she was actively immunised 10 days prior to her presentation [ 2 ] .…”

Section: Discussionmentioning

confidence: 99%

Cardiac sarcoidosis presenting as sustained ventricular tachycardia

Cotrim,

Andrade,

Antunes

et al. 2024

European Journal of Case Reports in Internal Medicine

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Introduction: Sarcoidosis has many possible clinical presentations since it can affect any organ, most commonly the lungs. The hallmark of the disease consists of the formation of non-necrotising granulomas. Pathogenesis is thought to rely on the interplay of genetic, environmental and epigenetic factors. This case highlights the importance of a thorough clinical history and physical examination, and the correlation with imaging findings in the diagnostic work-up of the non-ischaemic cardiomyopathy. Case description: A 57-year-old woman was admitted due to the sudden onset of malaise, dizziness, and chest discomfort. Sustained monomorphic ventricular tachycardia was evidenced and the patient rapidly evolved with haemodynamic instability; she underwent successful electrical cardioversion. The electrocardiogram afterwards showed a high-risk electrocardiographic pattern. Invasive coronary angiography excluded obstructive epicardial coronary lesions. Physical examination revealed skin lesions on the lower limbs which raised suspicion for erythema nodosum and therefore a biopsy was performed. Transthoracic echocardiography and cardiac magnetic resonance imaging revealed features consistent with an inflammatory cardiomyopathy, and an implantable cardioverter-defibrillator was placed. The histologic examination of the cutaneous lesions showed a non-necrotising granulomatous inflammatory process. Radionuclide imaging was inconclusive. The patient underwent an endomyocardial biopsy, which confirmed the diagnosis of systemic sarcoidosis with cardiac involvement. Conclusions: Systemic sarcoidosis with cardiac involvement is a challenging diagnosis. The role of imaging techniques such as transthoracic echocardiography, cardiac magnetic resonance imaging and radionuclide imaging is essential in raising suspicion and diagnosing this pathology. Endomyocardial biopsy is the ‘gold standard’ for its diagnosis; however, it has a low diagnostic yield.

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Section: Discussionmentioning

confidence: 99%

Cardiac sarcoidosis presenting as sustained ventricular tachycardia

Cotrim,

Andrade,

Antunes

et al. 2024

European Journal of Case Reports in Internal Medicine

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“…In addition, the absence of LGE has a high negative predictive value, making it a useful screening tool for CS 18 19. Furthermore, contemporary CMR protocols including T1 and T2 mapping can provide values in detecting inflammation in CS 23. In a study comparing sarcoidosis patients to healthy controls, native T1 and T2 values in sarcoidosis subjects were found to be significantly increased, suggestive of myocardial inflammation, compared to the control group 24.…”

Section: Discussionmentioning

confidence: 99%

Great mimicker: definite isolated cardiac sarcoidosis masquerading as hypertrophic cardiomyopathy

Pham,

Abraham,

Sheikh

2023

BMJ Case Rep

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A healthy man in his 50s was hospitalised after presenting with chest pain and dyspnoea. An echocardiogram revealed asymmetrical septal hypertrophy, leading to a diagnosis of hypertrophic cardiomyopathy. Due to progressive conduction abnormalities during his hospitalisation, further evaluation was performed. Cardiac MRI revealed dense late gadolinium enhancement of the septum in the area of hypertrophy. Additionally, fluorodeoxyglucose-positron emission tomography demonstrated increased uptake within the same region, suggestive of active inflammation. Subsequent endomyocardial biopsy showed non-caseating granulomatous inflammation, consistent with cardiac sarcoidosis. Treatment with prednisone and methotrexate was initiated, and an implantable cardioverter-defibrillator was placed following thorough risk stratification. This case highlights the importance of multimodality imaging and the pursuit of a tissue diagnosis in the evaluation of cardiomyopathy.

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“…We recommend immediately initiating glucocorticoid therapy after proof of active myocardial inflammation by advanced imaging (PET or CMR) if a subsequent, immediate, and targeted biopsy is not possible ( Figure 2 and Figure 5 ) [ 10 , 11 , 47 ]. It is unknown whether asymptomatic patients should be treated as well [ 98 ]. In these cases, we opt for shared, individualized decision-making considering the extent of inflammation as well as the side effects of therapy.…”

Section: Managementmentioning

confidence: 99%

Cardiac Sarcoidosis—Diagnostic and Therapeutic Challenges

Korthals,

Bietenbeck,

Könemann

et al. 2024

JCM

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Sarcoidosis is a multisystem disorder of unknown etiology. The leading hypothesis involves an antigen-triggered dysregulated T-cell-driven immunologic response leading to non-necrotic granulomas. In cardiac sarcoidosis (CS), the inflammatory response can lead to fibrosis, culminating in clinical manifestations such as atrioventricular block and ventricular arrhythmias. Cardiac manifestations frequently present as first and isolated signs or may appear in conjunction with extracardiac manifestations. The incidence of sudden cardiac death (SCD) is high. Diagnosis remains a challenge. For a definite diagnosis, endomyocardial biopsy (EMB) is suggested. In clinical practice, compatible findings in advanced imaging using cardiovascular magnetic resonance (CMR) and/or positron emission tomography (PET) in combination with extracardiac histological proof is considered sufficient. Management revolves around the control of myocardial inflammation by employing immunosuppression. However, data regarding efficacy are merely based on observational evidence. Prevention of SCD is of particular importance and several guidelines provide recommendations regarding device therapy. In patients with manifest CS, outcome data indicate a 5-year survival of around 90% and a 10-year survival in the range of 80%. Data for patients with silent CS are conflicting; some studies suggest an overall benign course of disease while others reported contrasting observations. Future research challenges involve better understanding of the immunologic pathogenesis of the disease for a targeted therapy, improving imaging to aid early diagnosis, assessing the need for screening of asymptomatic patients and randomized trials.

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Cardiac sarcoidosis: phenotypes, diagnosis, treatment, and prognosis

Cited by 59 publications

References 176 publications

Cardiac sarcoidosis presenting as sustained ventricular tachycardia

Cardiac sarcoidosis presenting as sustained ventricular tachycardia

Great mimicker: definite isolated cardiac sarcoidosis masquerading as hypertrophic cardiomyopathy

Cardiac Sarcoidosis—Diagnostic and Therapeutic Challenges

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