DIAGNOSIS

The diagnosis of SLE was established based on the American College of Rheumatology (ACR) criteria, fulfilling 4 of the 11 criteria, including serositis, evidenced by pericarditis; kidney disorder with significant proteinuria less than 500 mg/24 hours; leukopenia; and positive anti ds-DNA and positive ANA. Moreover, the diagnosis was supported by the Systemic Lupus Collaborating Clinics (SLICC) criteria, by fulfilling at least 4 criteria.

After pericardiocentesis, hydroxychloroquine and intravenous high-dose corticosteroids were administered with relief of symptoms. This therapy was followed by oral prednisone.

The patient returned to the emergency department for fatigue and body aches 4 months after her initial presentation, with no evidence of pericardiac tamponade recurrence on echocardiogram.


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DISCUSSION

Systemic lupus erythematosus is a multisystem autoimmune disease with a worldwide prevalence that affects nearly all organs of the body. Serosal involvement is common in SLE and has been consistently included in the ACR classification criteria of SLE in 1982, reviewed in 1997, and later in the SLICC 2012 classification criteria.2

Pericarditis is the most common cardiac manifestation of lupus occurring in 9% to 54% of patients with SLE. Cardiac tamponade is rare with an incidence of less than 2.5%, with fewer than 50 cases reported.1 The low incidence of tamponade in SLE may, in part, be due to the widespread use of steroids and nonsteroidal anti-inflammatory drugs, which are quite effective in reducing pericardial inflammation.3 A better understanding of SLE may help reduce the frequency of diagnostic and intervention delays observed in such a life-threatening manifestation.

In clinical practice, measuring ANA, complement levels, or immune complexes in pericardial fluid is not recommended as it may reflect similar tests in peripheral blood. The pericardial fluid may contain ANA, phagocytic cells containing nuclei (lupus erythematosus cells), low complement levels, and immune complexes similar to those seen in SLE-related pleural effusions. If the glucose concentration is normal, and the protein level is variable, it may be low with a transudate effusion and elevated with an exudate effusion. There is typically a predominance of mononuclear cells; scarring may be seen as a primary finding in healed disease. Purulent pericarditis may occur typically in an immunosuppressed patient.4

Interestingly, a low C4 level at presentation may be predictive of the development of tamponade physiology,5 which was the case in this patient. Our patient presented with nonspecific symptoms of shortness of breath, fever, and upper abdominal pain. Physical examination did not show specific signs of tamponade. However, upon further investigation to rule out occult infection due to fever, tachycardia and leukopenia, the finding of large pericardial fluid was striking. As a result, echocardiography was done in a timely manner. Her symptoms improved with pericardiocentesis and high-dose corticosteroids and hydroxychloroquine.

In a retrospective review study of 325 cases by Castier et al,6 a very good response was observed with pericardiocentesis associated with high doses of corticosteroids only. In clinical and laboratory follow-ups performed over a period of 3 years, neither recurrence of the pericardial effusion nor evolution to constriction occurred. Accordingly, cardiac tamponade has benign evolution when properly treated.

Recurrent cardiac tamponade has been reported in a retrospective study with 409 patients with SLE. Goswami et al1 conducted repeated pericardiocentesis in 3 patients, with 1 of 24 patients with cardiac tamponade needing surgical intervention. In this study,1 the immunosuppressives used were oral corticosteroids with monthly intravenous cyclophosphamide and intravenous methylprednisolone with monthly cyclophosphamide that alleviated the need for surgery including pericardial window.

CONCLUSION

Cardiac tamponade as an initial presentation of SLE is rarely reported in literature; this manifestation could be life threatening if not diagnosed and treated in a timely fashion. Appropriate history, physical examination, readily available investigations, including electrocardiogram and chest radiography, with high index of suspicion can lead to the diagnosis. Physicians must consider rheumatologic diseases as a differential diagnosis in pericardial diseases, including pericardial effusion and cardiac tamponade.

References

  1. Goswami RP, Sircar G,  Ghosh A, P Ghosh. Cardiac tamponade in systemic lupus erythematosus. QJM: Int J Med; 2018;111(2):83-87.
  2. Petri M, Orbai A‐M, Alarcon GS, et al. Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum. 2012;64:2677-2686.
  3. Lee IH, Yang SC, Kim TH, et al. Cardiac tamponade as an initial manifestation of systemic lupus erythematosus–single case report. J Korean Med Sci. 1997;12(1):75-77.
  4. Costenbader KH. Non-coronary cardiac manifestations of systemic lupus erythematosus in adults. UpToDate. Updated November 30, 2020. Accessed January 17, 2021. https://www.uptodate.com/contents/non-coronary-cardiac-manifestations-of-systemic-lupus-erythematosus-in-adults
  5. Rosenbaum E, Krebs E, Cohen M, Tiliakos A, Derk CT. The spectrum of clinical manifestations, outcome and treatment of pericardial tamponade in patients with systemic lupus erythematosus: a retrospective study and literature review. Lupus. 2009;18(7):608-12. doi:10.1177/0961203308100659
  6. Castier MB, Albuquerque EMN, Menezes MEFCC, Klumb E, Albanesi Filho FM. Cardiac tamponade in systemic lupus erythematosus. Report of four cases. Arq Bras Cardiol. 2000;75(5):446-448. doi:10.1590/s0066-782×2000001100008