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Incessant pericardial effusion in a 9 year old male responding to infliximab

Published online by Cambridge University Press:  01 August 2009

Rowan F. Walsh and
Devyani Chowdhury
Rowan F. Walsh*
Affiliation:
Department of Pediatrics, Division of Pediatric Cardiology, Schneider Children’s Hospital, New Hyde Park, New York, United States of America
Devyani Chowdhury
Affiliation:
Department of Pediatrics, Division of Pediatric Cardiology, Schneider Children’s Hospital, New Hyde Park, New York, United States of America
*
Correspondence to: Rowan Walsh, MD, Department of Pediatrics, Division of Pediatric Cardiology, The Mount Sinai Kravis Children’s Hospital, One Gustave L. Levy Place, Box 1201, New York, NY 10029-6574, United States of America. Tel: 718 601 2404; Fax: 718 601 2404; E-mail: rowando@eircom.net
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Abstract

Recurrent idiopathic pericardial effusion can be a challenging medical problem. Multiple medical interventions may yield minimal improvement. We describe a patient with an incessant pericardial effusion that responded to infliximab. The use of infliximab should be considered in the management of recurrent pericardial effusions resistant to other treatment modalities.

Keywords

Pericarditistumor necrosis factor alphasteroids

Information

Type
Brief Reports
Information
Cardiology in the Young , Volume 19 , Issue 4 , August 2009, pp. 413 - 415
Copyright
Copyright © Cambridge University Press 2009

We report our use of infliximab to treat a recurrent pericardial effusion in a 9 year old male. Traditionally, attempts at management of such problematic and challenging cases have been undertaken using anti-inflammatory medications, steroids, and colchicine. While temporary relief of symptoms and collection of pericardial fluid may be achieved with these therapies, the problem often recurs. Given our satisfactory results using infliximab, we suggest this agent should be considered in those suffering recurrent idiopathic pericardial effusions when other modes of therapy have failed.

Case report

A 9 year old male presented to the emergency room with a history of chest pain and dyspnoea lasting 1 day. The symptoms became more severe when he was supine. There was no history of trauma or reactive disease of the airways. The past medical history included an episode of serum sickness in response to cephalosporins and penicillin. The family history was significant for Hashimoto’s thyroiditis and diabetes mellitus. The electrocardiogram showed normal sinus rhythm, normal intervals, and diffuse elevation of the ST segments. The chest X-ray was normal. The echocardiogram demonstrated normal cardiac anatomy and normal ventricular function. A pericardial effusion of moderate size was noted. Having made the diagnosis of pericarditis, we started the patient on a course of indomethacin, giving 25 milligrams three times daily, for 1 month. His symptoms gradually improved, and the pericardial effusion resolved. After a period of 4 months following his initial presentation, he again developed symptoms of chest pain, along with pains in the joints of his right foot and ankle. He rapidly developed again a pericardial effusion of moderate size. He was treated with intravenous solumedrol at 1 milligram per kilogram per dose every 12 hours for 4 days. Extensive investigation of his rheumatologic and immunologic systems proved negative. He was discharged from the hospital on a combination of prednisone, at 30 milligrams twice daily, and indomethacin 25 millgrams three times daily. The steroids were weaned over a period of 2 months, and he was started on colchicine at 0.6 milligrams twice daily.

The patient again presented with chest pain and orthopnoea. He was re-admitted and treated with solumedrol. Upon discharge he was again started on indomethacin and his colchicine was continued. Despite treatment with steroids, and the adjunctive therapies of indomethacin and colchicines, his symptoms of pericarditis persisted, as did the development of pericardial fluid. Because of the side effects of steroids, specifically a Cushingoid appearance and osteoporosis, we started the patient on calcium supplementation and Vitamin D. An extensive laboratory work-up did not reveal any infectious or rheumatologic cause for his disease.

Because of the inability to wean his steroids in the face of recurrent pericardial effusion, and the development of the inevitable side effects of steroids, we decided to treat his recurrent pericardial effusion with intra-pericardial steroids. Thus, 11 months following his initial presentation, we drained his effusion. Following complete drainage, we instilled triamcinolone at 300 milligrams per square metre into the pericardial space. The intra-pericardial steroid was left in place for 24 hours and then drained. Viral polymerase chain reaction of the pericardial effusion was unremarkable. The patient was subsequently discharged home. Following this procedure, after 2 months, the patient once again developed a small pericardial effusion. Stress doses of steroid were commenced, at 5 milligrams per day for 3 days. Following recurrence of the pericardial effusion, infliximab at 5 milligrams per kilogram per dose was administered once per week for three weeks. Following this round of therapy, he had no recurrence of pericardial effusion. His other therapies were gradually weaned. Calcium supplementation and Vitamin D were continued for osteoporosis. We administered 2 further doses of infliximab 6 months following the initial course because of recurrence of symptoms of pericarditis and evidence of a small pericardial effusion. He did not require any further courses of infliximab, and he now continues to do well after follow-up of 4 years.

Discussion

In many instances, the cause of pericardial effusion can be determined, but in some cases the cause is not apparent despite a thorough diagnostic evaluation. In a recent study investigating the aetiology of pericardial effusions, one-third were idiopathic.Reference Khun, Peters, Marx and Breitbart1 Recurrent idiopathic pericarditis continues to be a difficult problem and a therapeutic challenge in all age groups and populations.Reference Sagrista-Sauleda, Angel, Permanyer-Miralda and Soler-Soler2 Despite extensive evaluations, the aetiology often remains elusive. While the treatment of patients with acute pericarditis is mainly to alleviate symptoms of chest pain, the management of chronic pericardial effusions is to prevent long-term complications, such as constrictive pericarditis and the possibility of cardiac tamponade. Chronic pericardial effusions following surgical intervention for congenital cardiac disease is a well recognized post-operative morbidity. Recurrent pericardial effusion may be a harbinger of more sinister pathologies such as neoplasm. Idiopathic recurrent pericardial effusion is also well described.

The current modalities for treatment of chronic and recurrent pericardial effusion include anti-inflammatory medications, colchicines, and parenteral or intracardiac steroids. The failure of one or all of these modalities is not uncommon, and can result in significant morbidity, as well as frustration on the part of the provider of care. In our patient, the incessant pericardial effusion responded to infliximab. The efficacy of this agent is well established for rheumatological and gastroenterological disease, and the treatment was tolerated well by our patient. Those beginning such treatment should be made cognizant of the potential risks of serious opportunistic infection, demyelinating disorders, congestive cardiac failure, and lymphoma.

The agent is a genetically constructed monoclonal antibody that binds the soluble subunit and the membrane-bound precursor of tumour necrosis factor alpha. It inhibits a broad range of the biologic activities of the factor, presumably by blocking its interaction with its receptors. Its pathophysiologic activities include a pro-inflammatory effect, potential activation of macrophages, as well as a role in influencing innate and adaptive immunity.Reference Kolias, Douni, Kassiotis and Kontoyiannis3 The aetiology of the pericarditis in our patient remained obscure. We surmise that an overzealous immune system may have produced an exaggerated elevation in tumour necrosis factor alpha in response to an undiagnosed subclinical viral infection manifesting as recurrent pericarditis. Elevated baseline levels of the factor have been demonstrated in children developing effusions and oedema after cardiac surgery.Reference Bocsi, Hambsch, Osmancik, Schneider, Valet and Tarnok4 The levels of the factor, however, were not measured in our patient. It remains possible that the recurrent pericardial effusions may be the sole manifestation of an autoimmune disease, or a systemic disease such as inflammatory bowel disease. Given the response of our patient to the treatment, follow-up will include a detailed review of these systems.

To our knowledge, our patient is the first such case reported. In the absence of specific contraindications, infliximab should therefore be considered part of the armamentarium for the medical management of recurrent pericardial effusion that has not responded to other treatments.

References

1.Khun, B, Peters, J, Marx, G, Breitbart, R. Etiology, management and outcome of pediatric pericardial effusions. Pediatr Cardiol 2008; 29: 90–94.CrossRefGoogle Scholar
2.Sagrista-Sauleda, J, Angel, J, Permanyer-Miralda, G, Soler-Soler, J. Long-term follow-up of idiopathic chronic pericardial effusion. N Engl J Med 1999; 341: 2054–2059.CrossRefGoogle ScholarPubMed
3.Kolias, G, Douni, E, Kassiotis, G, Kontoyiannis, D. On the role of tumor necrosis factor and receptors in models of multiorgan failure, rheumatoid arthritis, multiple sclerosis and inflammatory bowel disease. Immunol Rev 1999; 169: 175–194.CrossRefGoogle Scholar
4.Bocsi, J, Hambsch, J, Osmancik, P, Schneider, P, Valet, G, Tarnok, A. Preoperative prediction of pediatric patients with effusions and edema following cardiopulmonary bypass surgery by serological and routine laboratory data. Crit Care 2002; 6: 226–233.CrossRefGoogle ScholarPubMed