Case 22-2004: A 30-Year-Old Woman with a Pericardial Effusion

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Table 2. Causes of Pericarditis. Cause 284 The causes of pericarditis vary among populations. In a general hospitalized population, unselected cases of pericarditis are predominantly idiopathic or due to cancer (Table 2), 7 and the diagnostic yield of pericardiocentesis is low in the absence of tamponade or signs of infection. The new england journal of medicine 9-11 In contrast, among patients admitted to a university tertiary-care center with new, large, symptomatic pericardial effusions, infectious causes predominate, followed by a malignant condition and radiation-induced pericarditis. 8 noninfectious causes of pericarditis Among noninfectious causes of pericarditis, tumors are most common, with lung cancer, breast cancer, and Hodgkin’s disease heading the long list of possibilities. Given this patient’s mediastinal lymphadenopathy, it is important to consider cancer, especially lymphoma. Factors that argue against this diagnosis are the absence of a known primary tumor, the negative results of the cytologic evaluation of the pericardial fluid, and the negative results of examination of the bronchoscopic washings and the transbronchial needle–biopsy specimen. A pericardial biopsy performed at the time of the pericardiocentesis would have been useful for ruling out cancer and certain other diagnoses. Several noninfectious, inflammatory causes of pericarditis — radiation therapy, drugs, myocardial infarction, and metabolic factors — are not supported by the clinical history. Remaining are the collagen vascular diseases and sarcoidosis. Almost all of the Echocardiogram Series* Large New Effusions† percent of patients Infectious — 27 Acute idiopathic 20 — Iatrogenic 16 14 (radiation therapy) Malignant 13 23 Chronic idiopathic 9 7 Associated with preceding acute myocardial infarction 8 — Associated with uremia or dialysis 6 12 Associated with cardiovascular disease 5 12 * The data are from Sagrista-Sauleda et al. 7 and reflect findings in 322 patients. † The data are from Corey et al. 8 and reflect findings in 75 patients. n engl j med 351;3 collagen vascular diseases have been associated with pericarditis, but systemic lupus erythematosus and rheumatoid arthritis are the most common causes. The prevalence of collagen vascular diseases is increased among young women, such as this patient. Although pericardial disease is said to be the most common cause of symptomatic cardiac involvement in lupus, most pericardial effusions in patients with lupus are small and asymptomatic and are associated with a polyserositis and a pericardial friction rub on examination. In one study, pericardial effusion was seen in 19 percent of patients and tamponade in 2.5 percent, and it was the initial presentation in 1 percent. 12 www.nejm.org july 15, 2004 Mediastinal lymphadenopathy can occur in lupus, but nodular lung infiltrates are not consistent with a diagnosis of lupus. The low titer of antinuclear antibodies and the negative test for double-stranded DNA also make such a diagnosis unlikely. In one study of complications of rheumatoid arthritis, only 1.25 percent of patients with the disease had pericarditis 13 and 0.5 percent had tamponade. Furthermore, pericarditis occurs late in the course of rheumatoid arthritis. This patient had no joint symptoms, and a test for rheumatoid factor was negative. Still’s disease in adults has been associated with pericarditis, and tests for both antinuclear antibodies and rheumatoid factor are usually negative, but this patient did not have the high fever, polyarthralgias, rash, leukocytosis, abnormal aminotransferase levels, and elevated serum ferritin levels that are characteristic of that disease. Sarcoidosis can cause mediastinal lymphadenopathy and commonly affects the heart, causing arrhythmias, conduction abnormalities, heart failure, and pericardial disease. Pericardial involvement is rare, 15 but tamponade has been reported. 14 16 The level of angiotensin-converting enzyme was normal in the patient under discussion. infectious causes of pericarditis This process of elimination leads me to the infectious causes of pericarditis. Most bacterial infections of the pericardium are fulminant, result in small effusions, and are rapidly fatal. They are usually caused by common bacteria such as staphylococcus, streptococcus, Escherichia coli, salmonella, and Neisseria meningitidis, and most will be evident on Gram’s staining and will grow in culture. This patient started taking levofloxacin just before the pericardiocentesis was performed, and this drug could have interfered with growth in culture, although on staining, purulent fluid and organisms The New England Journal of Medicine Downloaded from nejm.org by LOKESH VUYYURU on February 22, 2012. For personal use only. No other uses without permission. Copyright © 2004 Massachusetts Medical Society. All rights reserved.
case records of the massachusetts general hospital would probably still be evident. The clinical course, heart.” 17 The presence of mediastinal lymphade- however, is not consistent with an acute bacterial nopathy, pulmonary lesions, and a pleural effusion process. — all on the same side — fits the clinical pattern of Viral infection is believed to account for the ma- tuberculous pericarditis. jority of idiopathic cases of pericarditis, the most As in cases of tuberculous pleuritis, the peri- common organisms being coxsackieviruses A and cardial inflammation in tuberculous pericarditis B, echovirus, adenovirus, and the human immuno- appears to occur in response to relatively few ordeficiency virus (HIV). Pericarditis may occur durganisms or to antigenic material in the pericardial ing the viral syndrome or one to three weeks later. space. Smears to test for acid-fast organisms in the The effusions are usually small but can be associat- fluid are usually negative, and cultures are positive ed with tamponade. Pain and a pericardial friction in only about half the cases. The quinolone antibi- rub are common, and the myocardium may be inotic used to treat presumed pneumonia may have volved, with unexplained arrhythmias or conduc- reduced the chance of a positive culture for M. tubertion defects. culosis. A biopsy of the pericardium at the time of the The list of other agents that can cause pericardi- pericardiocentesis might have increased the diagtis includes Rickettsia rickettsii, Chlamydia psittaci, Bornostic yield. 18 However, viral pericarditis was probrelia burgdorferi, Treponema pallidum, actinomyces, Myably presumed to be the diagnosis, and a biopsy was coplasma pneumoniae, and nocardia. Most of these deemed unnecessary. For most causes of pericar- bacteria cause acute, small pericardial effusions, dial effusion, the yield of pericardiocentesis with or and infections occur after specific exposures in en- without biopsy is low, and evidence-based indicademic areas. This patient had not recently visited tions for biopsy have not yet been established. Martha’s Vineyard, an area associated with both To confirm the diagnosis of tuberculosis and to Lyme disease and rickettsial disease. Chlamydial rule out a tumor, another specimen would be need- pericarditis can cause large effusions, but there had ed. Although a fine-needle aspiration biopsy of a been no exposure to sick birds or other likely sourc- lung nodule or a thoracoscopic biopsy of the pleura es. Cases of fungal pericarditis (histoplasmosis and might provide the necessary information for a de- coccidioidomycosis) are usually limited to areas finitive diagnosis, the most direct approach would where these infections are endemic and occur in im- be mediastinoscopy and biopsy of the mediastinal munocompromised hosts. lymph nodes. This approach would provide suffi- Exposure to infectious agents in regions where cient tissue for all the required diagnostic proce- they are endemic is always an important clue with dures for tuberculosis, tumor, and the other, less respect to infection. This patient had spent a month likely possibilities. on medical rotation in Nairobi, where tuberculosis Could this serious infection have been prevent- is endemic. Five months after her return (four ed? With increasing global travel, the risks of expo- months before the onset of her symptoms), a tubersures to infectious agents are increasing. 19 There is culin skin test was negative, but after her admission little that one can do to avoid tuberculosis in high- to this hospital, the induration in reaction to a skin risk medical settings. Tuberculin skin testing of test measured 16 mm, a finding highly predictive travelers before and two months after a visit to areas of infection with Mycobacterium tuberculosis. Both the where tuberculosis is endemic is important, and possible exposure in Kenya and the new, positive conversion to a positive result should prompt treat- reaction to a tuberculin skin test push tuberculosis ment for latent infection. 20 This patient had a neg- to the top of the list of diagnoses. ative tuberculin skin test five months after returning from Africa. Tuberculin skin-test anergy due to tuberculous pericarditis immunosuppression associated with other illness- Although tuberculous pericarditis is an uncommon es, such as infections with HIV, or drugs, such as complication in countries where tuberculosis rates corticosteroids, could explain this finding, but nei- are low, in countries where the prevalence of tuberther disease nor medication use was known to be culosis is high it is among the most common causes present. The tuberculin skin test can have technical of heart failure. In the Transkei region of South Af- problems, ranging from injections that are subcurica, for example, tuberculous pericarditis is such a taneous instead of intracutaneous to tuberculin ma- common cause of heart failure it is called “Transkei terial that is defective due to mishandling. Transient n engl j med 351;3 www.nejm.org july 15, 2004 The New England Journal of Medicine Downloaded from nejm.org by LOKESH VUYYURU on February 22, 2012. For personal use only. No other uses without permission. Copyright © 2004 Massachusetts Medical Society. All rights reserved. 285
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Case 22-2004: A 30-Year-Old Woman with a Pericardial Effusion

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