Cardiomyopathy in Celiac Disease: A Systematic Review
Stefan Milutinovic1, Predrag Jancic2, Adam Adam3
1Internal Medicine Residency Program at Lee Health, Florida State University College of Medicine, Tallahassee, FL 32301, USA.
Insights
Celiac cardiomyopathy (CCM) is a serious condition linked to celiac disease (CD). Early diagnosis and treatment with a gluten-free diet can improve outcomes, highlighting the need for increased clinical awareness.
Area of Science:
- Cardiology
- Gastroenterology
- Pediatrics
Background:
- Celiac cardiomyopathy (CCM) is a serious condition associated with celiac disease (CD), affecting both adults and children.
- Existing data on the causal link between CD and cardiomyopathy (CMP) remain inconsistent.
- This study aims to synthesize current literature to establish a more evidence-based relationship between CD and CMP.
Purpose of the Study:
- To review and synthesize existing literature on celiac cardiomyopathy (CCM).
- To investigate the relationship between celiac disease (CD) and cardiomyopathy (CMP).
- To provide evidence-based insights into the causal link and clinical management of CCM.
Main Methods:
- Systematic review adhering to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines.
- Searched Medline, Embase, and Scopus databases from inception until September 2023.
- Identified and analyzed 1187 original articles, focusing on 28 confirmed CCM cases.
Main Results:
- Twenty-eight CCM patients (19 adults, 9 pediatric) were identified, with mean age 27.4 years.
- Common comorbidities included anemia (75%) and pulmonary hemosiderosis (20%).
- CCM diagnosis often preceded or coincided with CD diagnosis (35% and 48% respectively), with an average diagnostic delay of 16 months. Cardiovascular improvement was observed in 60.7% of patients after 11 months of treatment. Pediatric mortality was 33.3%, while adult mortality was 5.3%.
Conclusions:
- Clinicians should consider CD screening in CMP patients, especially those with anemia.
- Increased awareness and prompt diagnosis of CCM are crucial for reducing morbidity and mortality.
- Gluten-free diet combined with guideline-directed medical therapy can improve clinical outcomes in CCM patients.
Abstract:
(1) Background: Cardiomyopathy in celiac disease or celiac cardiomyopathy (CCM) is a serious and potentially life-threatening disease that can occur in both adults and children. However, data supporting the causal relationship between celiac disease (CD) and cardiomyopathy (CMP) are still inconsistent. The aim of this study was to review and synthesize data from the literature on this topic and potentially reveal a more evidence-based causal relationship. (2) Methods: The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were used to search Medline, Embase, and Scopus databases from database inception until September 2023. A total of 1187 original articles were identified. (3) Results: We identified 28 CCM patients (19 adult and 9 pediatric) with a mean age of 27.4 ± 18.01 years. Adult patients with CCM were predominantly male (84.2%) while pediatric patients were predominantly female (75%). The most common comorbidities associated with CCM were anemia (75%) and pulmonary hemosiderosis (20%). In 35% of patients, CCM occurred before the diagnosis of CD, while in 48% of patients, CCM and CD were diagnosed at the same time. Diagnosis of CD preceded diagnosis of CCM in only 18% of patients. Diagnosis of CCM is often delayed with an average, from the onset of symptoms to diagnosis, of 16 months. All patients were treated with a gluten-free diet in addition to guideline-directed medical therapy. At 11-month follow-up, cardiovascular improvement was seen in 60.7% of patients. Pediatric mortality was 33.3%, while adult mortality was 5.3%. (4) Conclusions: Clinicians should be aware of the possible association between CD and CMP, and we recommend CD work-up in all patients with CMP who have concomitant anemia. While we identified only 28 cases in the literature, many cases might go unreported due to a lack of awareness regarding CCM. A high degree of clinical suspicion and a prompt diagnosis of CCM are essential to minimizing the risks of morbidity and mortality, as the combination of a gluten-free diet and guideline-directed medical therapy can improve clinical outcomes.
