Clinical Pearls & Morning Reports
Published May 29, 2019
Ninety percent of patients diagnosed with systemic lupus erythematosus are female. The disease is more severe and hematologic abnormalities and serositis are more common in men than in women. Read the latest Case Records of the Massachusetts General Hospital here.
Q: What is the most common etiology of pericarditis in the United States?
A: In the United States, approximately 80% of cases of pericarditis are idiopathic, 5 to 10% are due to cancer, 2 to 7% are due to autoimmune or pericardial-injury syndromes, 4% are due to tuberculosis, and less than 1% are due to a purulent bacterial infection. However, in developing countries, the epidemiology of pericardial disease is quite different, such that 70% of cases are attributed to tuberculosis.
Q: What are some of the rheumatic diseases that are commonly associated with pericardial involvement?
A: The rheumatic diseases that are most commonly associated with pericardial involvement include systemic lupus erythematosus, rheumatoid arthritis, adult-onset Still’s disease, and systemic sclerosis.
A: Pericarditis is the most common cardiac manifestation of lupus. Symptomatic pericarditis may develop in 25% of patients and is often accompanied by pleuritis; subclinical pericarditis is even more common. Tamponade, however, is rare, occurring in 1 to 2.5% of patients with lupus. In patients with systemic lupus erythematosus, tamponade has been associated with hemolytic anemia, renal disease, hypocomplementemia, and female sex. The pericardial fluid is typically a neutrophilic, inflammatory exudate.
A: The diagnosis of systemic lupus erythematosus is based on clinical and laboratory features. There are no formal diagnostic criteria. However, classification criteria, including the American College of Rheumatology (ACR) criteria and the Systemic Lupus International Collaborating Clinics (SLICC) criteria, are helpful in considering the diagnosis. Patients who have mild manifestations of lupus, such as skin and joint involvement, can be treated with hydroxychloroquine and low-dose prednisone or nonsteroidal antiinflammatory drugs. Patients who have clinical manifestations, such as skin, joint, hematologic, or non–life-threatening serositis, are treated with hydroxychloroquine and moderate-dose prednisone and are often also treated with another immunosuppressive agent, such as methotrexate, azathioprine, mycophenolate mofetil, or belimumab. Patients who have severe, organ-threatening disease are often treated with high-dose glucocorticoids and also with a glucocorticoid-sparing agent, such as cyclophosphamide, mycophenolate mofetil, or azathioprine. In the absence of a contraindication, all patients with systemic lupus erythematosus would be treated with hydroxychloroquine, given its numerous benefits, including increased survival, decreased disease damage, and fewer flares, as well as improved renal function, fewer thrombotic complications, and potentially improved glycemic status and lipid profiles.