Rare case of cardiac sarcoidosis seen with pericardial involvement
Report underscores importance of advanced imaging, biopsy for diagnosis
Involvement of the pericardium, a sac-like membrane that surrounds and protects the heart, is an uncommon manifestation of cardiac sarcoidosis that doctors should consider when there’s evidence of heart inflammation, a case report suggests about a woman who reported chest pain during a visit to a clinic.
“This case highlights the importance of considering pericardial involvement in sarcoidosis,” wrote the researchers, who said “advanced imaging and timely biopsy are crucial for accurate diagnosis.” The report, “A rare case of pericardial sarcoidosis presenting as chest pain,” was published in Clinical Case Reports.
In sarcoidosis, small clumps of inflammatory cells called granulomas build up in tissues. Cardiac sarcoidosis, where the granulomas affect the heart, doesn’t cause any overt symptoms in patients; only around 5% of sarcoidosis patients have heart-related symptoms. Still, it’s a serious condition and, at its worst, can lead to sudden cardiac arrest due to abnormal heart rhythms, making diagnosing and promptly treating it critical.
Cardiac sarcoidosis most often affects the heart’s pumping muscle, called the myocardium. In around 12% of people with cardiac sarcoidosis, the pericardium is involved. When pericardial sarcoidosis occurs, it’s often due to the spread of disease from the myocardium, with its mostly likely manifestation being pericardial effusion, or fluid buildup.
Pericardial sarcoidosis can be particularly difficult to diagnose and distinguish from other conditions, given how rare it is and how variable it can be.
Chest pain leads to pericardial sarcoidosis diagnosis
Here, researchers write about a a 52-year-old African American woman who complained of chest pain at the hospital and was found to have pericardial sarcoidosis.
The woman said she’d been having left-side chest pain for seven months, along with progressive shortness of breath, weight loss of 50 pounds, fatigue, and neck swelling. She was found to have elevated blood levels of angiotensin-converting enzyme and interleukin-2 receptor, both sarcoidosis biomarkers.
Electrical studies of her heart revealed some abnormalities in heart function. She also showed signs of fluid buildup in the pericardium and around the lungs, and had swelling of several lymph nodes, which made the doctors consider lymphoma, a type of blood cancer. Testing didn’t show signs of cancer, however, but a lymph node biopsy did show the presence of granulomas, and examining cells in the myocardium showed noncancerous immune cells, along with evidence of granuloma-associated inflammation, supporting cardiac sarcoidosis.
The woman was started on prednisone, a steroid used for sarcoidosis, but her symptoms weren’t well controlled after two months. Her doctors added methotrexate, a potent immunosuppressant, to her regimen. Six months later, her chest pain declined, imaging showed no signs of active cardiac inflammation, and her heart function was largely normal. Still, sarcoidosis outside the heart, including in her spleen and parts of her chest, were still present. A scan taken eight months after treatment showed her pericardial effusion had resolved, but her lymph nodes remained swollen.
An exact treatment regimen for cardiac sarcoidosis may depend on a number of factors, said the researchers. “The specific regimen is chosen based on experience, patient preference, and co-morbidities,” they said, adding the woman’s case “underscores the need for heightened clinical awareness and a multidisciplinary approach in managing this … aspect” of cardiac sarcoidosis, which remains “underdiagnosed.”
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