Surgery usually required for rare heart issue in IgG4-related disease
Study: Condition affects pericardium, a protective sac that surrounds organ
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In rare cases where IgG4-related disease (IgG4-RD) affects the pericardium, surgery to remove all or part of this sac surrounding the heart is usually necessary to control the disease, according to a study of 5 patients in the U.S.
“Medical therapy alone is often inadequate, and [surgery] remains the definitive treatment,” researchers wrote. They noted that, even after surgery, “long-term multidisciplinary follow-up is required to monitor for systemic [body-wide] disease and treatment-related complications.”
The study, “IgG4-Related Pericarditis: Multimodality Imaging Diagnosis and Surgical Outcomes in a Case Series,” was published in JACC: Case Reports.
Pericardium normally helps protect the heart from damage
The pericardium is a sac of fibrous tissue that surrounds the heart. Normally, it helps protect the heart from damage and provides lubrication, helping ensure the heart beats smoothly and efficiently. But if the pericardium becomes inflamed, a condition called pericarditis, it can interfere with the heart’s ability to beat correctly.
IgG4-RD is a rare inflammatory disorder marked by the formation of abnormal clumps of immune cells, mostly those producing the antibody IgG4, in tissues. This leads to long-lasting inflammation and scarring (fibrosis).
The disease can affect virtually any tissue in the body, with symptoms depending on the tissue and organs affected. In rare cases, IgG4-RD may affect the pericardium, presenting as “recurrent or constrictive pericarditis and [being] frequently [resistant] to conventional anti-inflammatory therapy,” the researchers wrote.
Constrictive pericarditis occurs when the pericardium becomes scarred or thickened, restricting the heart’s ability to fill properly.
Biospy ultimately required to diagnose IgG4-RD pericarditis
In this study, a team of scientists in Ohio described the cases of five men with constrictive pericarditis due to IgG4-RD who were treated at their center.
All men displayed signs and symptoms indicative of constrictive pericarditis, including shortness of breath, swelling, and/or chest pain. However, their doctors generally didn’t consider IgG4-RD as a likely cause, and most patients were initially believed to have pericarditis of unknown or viral cause.
“This reflects the nonspecific clinical presentation of IgG4-RD pericarditis, which frequently overlaps with more common [causes],” the team wrote.
The scientists highlighted that imaging tests were key for accurate evaluation, with biopsy (tissue sample analysis) of the pericardium ultimately required to definitively diagnose IgG4-RD pericarditis.
“Diagnosis requires a high index of suspicion” and “relies on multimodality imaging, invasive [heart tests], and pericardial biopsy with IgG4 [testing],” the researchers wrote.
IgG4-RD pericarditis is a rare but important cause of constrictive pericarditis. Standard anti-inflammatory therapy is often ineffective; pericardiectomy is frequently required and can be curative.
All but one man received medications aimed at controlling their condition, including glucocorticoids, other types of anti-inflammatory drugs, immunosuppressive treatments, and colchicine (a therapy used to treat certain heart infections). However, medication was ineffective at controlling IgG4-RD in the pericardium.
“Medical therapy with nonsteroidal anti-inflammatory drugs, colchicine, and [glucocorticoids], as well as [immunosuppressants] … was trialed in most patients but failed to provide a durable benefit,” the researchers wrote. “This finding underscores the limited efficacy of pharmacologic therapy once advanced fibrotic pericardial disease is established.”
Ultimately, all five men underwent pericardiectomy, a surgical procedure where all or part of the pericardium is removed. This was effective for relieving symptoms of constrictive pericarditis in all cases, though two men continued to require low-dose glucocorticoids to manage other manifestations of IgG4-RD.
One man experienced side effects of glucocorticoids, including diabetes, “emphasizing the need for careful long-term monitoring,” the researchers wrote.
These findings highlight that “IgG4-RD pericarditis is a rare but important cause of constrictive pericarditis,” the team wrote, adding that “standard anti-inflammatory therapy is often ineffective; pericardiectomy is frequently required and can be curative.”
“Long-term multidisciplinary follow-up is required to monitor for [body-wide] disease and treatment-related complications,” the researchers concluded.
