The Current Diagnostic and Treatment Landscape of Cardiac Sarcoidosis, With Vaiibhav Patel, MD

Cardiac sarcoidosis (CS) is among the hardest cardiovascular diseases to diagnose, primarily because it shares the majority of its symptoms with a long list of other conditions. Common presentations include high-grade conduction disease, left ventricular dysfunction, or ventricular arrhythmias.1

“Sarcoidosis can present very clinically silently through just imaging findings, and it could present with patients coming in really sick, with ventricular arrhythmias and potentially even cardiogenic shock,” Vaiibhav Patel, MD, a cardiologist at the Ohio State University Wexner Medical Center, told HCPLive in an exclusive interview. “There are a lot of different cardiac diseases that can present that way, so I think it’s the job of the clinician to try to rule out everything else before you come to a diagnosis of cardiac sarcoidosis.”

Cardiac Sarcoidosis Diagnosis

Current clinical guidelines recommend diagnosis through a combination of multimodality imaging and multidisciplinary collaboration – not as a method of finding indicators of the condition, but to positively rule out alternative diagnoses. CS often presents with systemic involvement, which highlights the need for a thorough organ assessment.1

The actual diagnosis process recommended involves a range of devices, including echocardiography, which may demonstrate reduced left ventricle ejection fraction or regional wall aneurysm, and ECG, which may show conduction delay, fragmented QRS complexes, and right or left bundle-branch block. However, the guidelines also warn of patients potentially presenting with normal ECG and normal echocardiography, which prompts the use of cardiac magnetic resonance and fluorine-18 fluorodeoxyglucose-positron emission tomography (PET).1

In the event of continued uncertainty, clinicians are advised to proceed to endomyocardial biopsy, polymerase chain reaction, and immunohistochemistry – these procedures are considered the gold standard for sarcoidosis. However, studies have shown that treatment should still be initiated despite a negative biopsy result if abnormalities are detected via other diagnostic methods.2

Guideline-Directed Treatment

The guidelines state that treatment should be based on a risk-benefit ratio. If the disease is symptomatic, treatment should be initiated in patients with probable or definite CS. Those with possible or low-probability CS generally do not receive treatment given the uncertainty of diagnosis and unclear benefit for treatment. Additionally, given that the primary form of treatment is immunomodulation, the guidelines acknowledge an additional potential for harm.1

Corticosteroids are first-line agents initiated at moderate to high doses and tapered according to imaging and clinical responses. Additional immunosuppression is administered in the case of incomplete response or steroid intolerance. Additionally, cardiologic management is recommended to reduce the risk of arrhythmias or heart failure. Patients at high risk may be administered an implantable cardioverter-defibrillator (ICD), although standard heart failure therapies are also assigned as indicated.2

Despite these difficulties, the horizon for CS looks promising. Novel biologics like TNF-a inhibitors are currently in testing for refractory or steroid-resistant disease, targeting inflammatory pathways central to the formation of granuloma. Additionally, new biomarkers like circulating cytokines, imaging metrics, and emerging molecule markers, are allowing for real-time monitoring and therapeutic efficacy. However, the diagnostic challenge still remains at the forefront of the treatment process.2

“In the past, we really thought of cardiac sarcoidosis as being a younger person’s disease. But I think the longer I’ve been doing this, the more I’ve been humbled by the fact that anyone can present with cardiac sarcoidosis,” Patel said. “And the caveat is that there are other things that can cause some of these presentations, and we need to rule those out first.”

Editors’ Note: Patel reports disclosures with Revamp Medical.

References

1. Cheng RK, Kittleson MM, Beavers CJ, et al. Diagnosis and management of cardiac sarcoidosis: A scientific statement from the American Heart Association. Circulation. 2024;149(21). doi:10.1161/cir.0000000000001240

2. Kancerek J, Świerczek D, Baron W, Rojek M, Lewandowski P, Wojnicz R. Unmasking Cardiac Sarcoidosis: Integrating Multimodal Imaging with Histochemical and Ultrastructural Analysis. Int J Mol Sci. 2026;27(7):2969. Published 2026 Mar 25. doi:10.3390/ijms27072969