Mortality relating to heart disease has continued to decrease over the past several years, making the management of patient morbidity increasingly important. This trend coincides with an increased emphasis on managing patients with pericarditis, a disease defined as inflammation of the pericardial sac and that is often characterized by significant morbidity. Most patients with acute pericarditis have a benign course and a good prognosis. However, others will develop complicated pericarditis, which is characterized by incessant, recurrent, chronic, or constrictive disease. “Many of these patients will experience severe debilitation because of this chronic disease,” says Allan L. Klein, MD.
A Helpful Review
In a review published in the Journal of the American College of Cardiology, Dr. Klein and colleagues addressed several important aspects of improving the diagnosis and management of patients with complicated pericarditis. “There has been a renaissance in our understanding of complicated pericarditis with data from recent clinical trials and Pericardial Centers of Excellence that is stirring the pot on how we manage this disease,” Dr. Klein says. “We can now better characterize complicated pericarditis using multimodality imaging, which in turn can help us improve our treatment strategies.”
The review by Dr. Klein and colleagues focused on identifying patients at risk for complicated disease after acute pericarditis and which patients with the disease could benefit from multimodality imaging. The review also examined the pathological progression of pericarditis and how auto-inflammatory pericarditis is distinct from autoimmune pericarditis. In addition, the article looked at the established treatments for pericarditis as well as emerging therapies for patients with complicated disease.
Identifying Those at Risk
According to Dr. Klein, long-term outcomes are generally favorable for patients with acute pericarditis when they are treated. “Most patients who receive appropriate anti-inflammatory medications will have their symptoms resolve within days to weeks,” he says. “However, other patients will experience adverse events relating to their initial presentation or will be debilitated by recurrent attacks.” The key is to stratify patient risk according to their likelihood of developing complicated pericarditis later in their course of care. These risks can be categorized as patient- or treatment-related (Table).
Studies indicate that early high-dose corticosteroids, a lack of colchicine, an elevated high-sensitivity C-reactive protein, and undertreatment with NSAIDs are associated with the development of complicated pericarditis. Identifying the causes of complicated disease is paramount, according to Dr. Klein. “In select cases, cardiovascular MRI may help clinicians in their assessment of pericardial inflammation and constriction,” he says. “To appropriately select patients with complicated pericarditis for multimodality imaging, it’s important to have an understanding of the sequences used to evaluate the pericardium and how these images correlate with the progressive pathology of pericarditis. The stage and severity of pericardial inflammation is better assessed with cardiac MRI and advanced echocardiography.”
Auto-Inflammatory Therapies & Autoimmune Pericarditis
The review by Dr. Klein and colleagues notes that, given phenotypic similarities between recurrent idiopathic pericarditis and periodic fever syndromes, disorders of the inflammasome may contribute to relapsing attacks. Therapies that target the inflammasome, such as anakinra, may lead to more durable remission and resolution. Recent studies looking at the role of the inflammasome in complicated pericardial disease highlight important distinctions with regard to inappropriate auto-inflammation as compared with those with predominant autoimmune disease.
The mainstay of medical treatment for recurrent idiopathic pericarditis has been use of anti-inflammatory therapies. The goals of these agents are to control symptoms during a flare and to decrease risks for recurrence. “It’s important to emphasize efforts to reduce recurrence,” says Dr. Klein. “Many patients with recurrent idiopathic pericarditis have a chronic disease. They may take anti-inflammatories for months or years, but then experience random relapses or have attacks when their medications are tapered. For these patients, clinicians need to understand the safety and efficacy of the therapies they prescribe.” He adds that potential use of disease-modifying therapies and biologics should be investigated.
Among patients with complicated pericarditis, a better understanding of the causes and pathological progression of pericardial inflammation is needed. “We need research on the role of multimodality imaging to guide appropriate care,” Dr. Klein says. “In addition, randomized control trials are needed to assess and compare novel therapies—like biologic drugs—and surgical options for complicated pericarditis. Furthermore, studies on genetic mutations that increase susceptibility to the disease are also warranted.”
Dr. Klein adds that optimizing the care of complicated pericarditis is important not only for cardiologists but also for clinicians in emergency medicine, internal medicine, rheumatology, and other disease states. “We have made significant strides in caring for this patient group and complicated pericarditis patients can now be referred to Centers of Excellence, which are located in several regions of the United States, including the Cleveland Clinic,” he says. “The next step is to further our knowledge on management of the disease and to conduct multicenter trials and registries that collect data on the effects of novel treatments and other strategies so that we can improve patient outcomes.”
Cremer PC, Kumar A, Kontzias A, et al. Complicated pericarditis: understanding risk factors and pathophysiology to inform imaging and treatment. J Am Coll Cardiol. 2016;68:2311-2328. Available at: http://www.sciencedirect.com/science/article/pii/S0735109716353207.
Dr. Klein answers questions about the correct treatment of recurrent pericarditis on a YouTube video that is available at https://www.youtube.com/watch?v=zkR4BVd-pTg.
Klein AL, Abbara S, Agler DA, et al. American Society of Echocardiography clinical recommendations for multimodality cardiovascular imaging of patients with pericardial disease: endorsed by the Society for Cardiovascular Magnetic Resonance and Society of Cardiovascular Computed Tomography. J Am Soc Echocardiogr. 2013;26:965-1012.
Imazio M, Spodick DH, Brucato A, et al. Controversial issues in the management of pericardial diseases. Circulation. 2010;121:916-928.
Imazio M, Gaita F, LeWinter M. Evaluation and treatment of pericarditis: a systematic review. JAMA. 2015;314:1498-1506.
Imazio M, Gaita F. Diagnosis and treatment of pericarditis. Heart. 2015;101:1159-1168.