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Controversies in the Treatment of Cardiac Sarcoidosis
Ogugua Ndili Obi1, Elyse E Lower2, Robert P Baughman2
1Division of Pulmonary Critical Care and Sleep Medicine, Brody School of Medicine, East Carolina University, Greenville, North Carolina, United States.
Insights
Managing cardiac sarcoidosis (CS) with immunosuppressive therapy (IST) presents challenges in treatment initiation and drug selection. Evidence is limited, with corticosteroids as first-line, but early steroid-sparing agents may improve outcomes.
Area of Science:
- Cardiology
- Immunology
- Pulmonology
Background:
- Cardiac sarcoidosis (CS) management is complex, lacking clear guidelines for treatment initiation and drug choice.
- Corticosteroids are the primary immunosuppressive therapy (IST) for CS.
- Limited high-quality evidence, such as randomized controlled trials, exists to guide CS treatment strategies.
Purpose of the Study:
- To review the challenges and current understanding of immunosuppressive therapy (IST) for cardiac sarcoidosis (CS).
- To discuss the role of corticosteroids and steroid-sparing agents in CS management.
- To address the controversial use of anti-tumor necrosis factor (anti-TNF) agents in CS.
Main Methods:
- Review of European Respiratory Society (ERS) clinical practice guidelines for sarcoidosis treatment.
- Analysis of existing data on the efficacy of corticosteroids and early steroid-sparing agents.
- Discussion of the controversial role of anti-tumor necrosis factor (anti-TNF) agents in refractory CS.
Main Results:
- ERS guidelines strongly recommend IST for CS patients with cardiac abnormalities at risk of adverse outcomes.
- Corticosteroids are first-line, but early initiation of steroid-sparing agents may be beneficial.
- The use of anti-tumor necrosis factor (anti-TNF) agents in CS remains controversial.
Conclusions:
- Effective management of cardiac sarcoidosis requires careful consideration of IST, balancing benefits and risks.
- Further research is needed to establish optimal treatment protocols, including the role of advanced therapies like anti-TNF agents.
- Personalized treatment strategies are crucial for patients with cardiac sarcoidosis.
Abstract:
There are many challenging aspects of the management of cardiac sarcoidosis (CS) with corticosteroids and other immunosuppressive therapy (IST). First, it is not always clear who will benefit from therapy or when to initiate treatment. Secondly, there are no randomized controlled trials or large prospective studies to guide what medications to use, at what doses, and for how long. The European Respiratory Society (ERS) clinical practice guidelines on the treatment of sarcoidosis makes a strong recommendation for the use of immuno-suppressive therapy in CS patients with functional cardiac abnormalities, including heart blocks, dysrhythmias, or cardiomyopathy where patients are considered at-risk of adverse outcomes. Corticosteroids are the first line immunosuppressive therapy in CS however, early initiation of second-line steroid sparing medications has been advocated and there is data to suggest that concomitant initiation of therapy may be more beneficial. The use of anti-tumor necrosis factor (anti-TNF) agents (including infliximab and adalimumab) considered beneficial third-line anti-sarcoidosis treatment agents in other severe refractory manifestations of disease remains controversial.
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