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What cardiologists should know about cardiac sarcoidosis in 2022?
Sylvain Lemay1, Laurie Marchand, Mario Sénéchal
1Department of Cardiology, Institut Universitaire de Cardiologie et de Pneumologie de Québec, Laval University, Quebec City, Quebec, Canada.
Purpose Of Review:
Cardiac sarcoidosis (CS) is a potentially fatal condition when unrecognized or not treated adequately. The purpose of this review is to provide new strategies to increase clinical recognition of CS and to present an updated overview of the immunosuppressive treatments using most recent data published in the last 18 months.
Recent Findings:
CS is an increasingly recognized pathology, and its diagnostic is made 20 times more often in the last two decades. Recent studies have shown that imaging alone usually lacks specificity to distinguish CS from other inflammatory cardiomyopathies. However, imaging can be used to increase significantly diagnostic yield of extracardiac and cardiac biopsy. Recent reviews have also demonstrated that nearly 25% of patients will be refractory to standard treatment with prednisone and that combined treatment with a corticosteroid-sparing agent is often necessary for a period that remains undetermined.
Summary:
CS is a complex pathology that should always require a biopsy attempt to have a histological proven diagnosis before starting immunosuppressive therapy consisting of corticosteroids with or without a corticosteroid-sparing agent.
Insights
Cardiac sarcoidosis (CS) requires timely diagnosis and treatment. New strategies improve recognition, while updated immunosuppressive therapies, including corticosteroid-sparing agents, are crucial for refractory cases.
Area of Science:
- Cardiology
- Immunology
- Pathology
Background:
- Cardiac sarcoidosis (CS) is a serious condition with increasing diagnoses.
- Imaging lacks specificity but aids biopsy in CS diagnosis.
- A significant portion of patients are refractory to standard prednisone treatment.
Purpose of the Study:
- To present novel strategies for enhancing clinical recognition of CS.
- To offer an updated review of immunosuppressive treatments for CS.
- To incorporate the latest data from the past 18 months.
Main Methods:
- Review of recent literature on CS diagnosis and treatment.
- Analysis of imaging's role in diagnostic yield.
- Evaluation of current immunosuppressive regimens and outcomes.
Main Results:
- CS diagnostic rates have risen significantly.
- Imaging supports biopsy but lacks standalone specificity.
- Approximately 25% of CS patients do not respond to prednisone alone.
- Combined therapies with corticosteroid-sparing agents are often necessary.
Conclusions:
- Histological confirmation via biopsy is essential before initiating immunosuppressive therapy for CS.
- Corticosteroids, potentially with corticosteroid-sparing agents, form the cornerstone of CS treatment.
- Long-term treatment duration for CS remains to be determined.
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