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 Table of Contents  
Year : 2017  |  Volume : 12  |  Issue : 3  |  Page : 175-176

Cardiac tamponade as the sole presenting manifestation of systemic lupus erythematosus in a 3-year-old girl

Department of Pediatrics, Sri Ramachandra University, Chennai, Tamil Nadu, India

Date of Web Publication25-Apr-2017

Correspondence Address:
Mahesh Janarthanan
Department of Pediatrics, Sri Ramachandra University, Chennai - 600 116, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/injr.injr_25_17

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Keywords: Cardiac tamponade, systemic lupus erythematosus, young child

How to cite this article:
Janarthanan M, Kalaiarasan N, Rajakumar P S, Jebaraj R. Cardiac tamponade as the sole presenting manifestation of systemic lupus erythematosus in a 3-year-old girl. Indian J Rheumatol 2017;12:175-6

How to cite this URL:
Janarthanan M, Kalaiarasan N, Rajakumar P S, Jebaraj R. Cardiac tamponade as the sole presenting manifestation of systemic lupus erythematosus in a 3-year-old girl. Indian J Rheumatol [serial online] 2017 [cited 2022 Jan 24];12:175-6. Available from:

A 3-year-old female, previously well, 1st born child of nonconsanguineous parents and no family history of autoimmune diseases presented to our hospital with complaints of periorbital puffiness for 45 days. She was afebrile but tachycardic, pulse rate (PR-140/min) and tachypneic, respiratory rate (RR-35/min). Her central and peripheral pulses were well felt. Her blood pressure was high at 140/90 mmHg (>99th centile). Periorbital and facial puffiness, and bilateral minimal pitting pedal edema were also present. Cardiac examination revealed muffled heart sounds, raised jugular venous pressure, and pulsus paradoxus. There was diminished air entry over the bilateral lung bases. Musculoskeletal and genitourinary system examination was normal.

Routine blood tests including complete blood count, erythrocyte sedimentation rate, liver function test, and renal function test were normal. Her serum lactic dehydrogenase level was high at 2757 (105–333 IU/L). Chest X-ray showed cardiomegaly and pleural effusion bilaterally. Computed tomography chest showed moderate bilateral pleural effusion and massive pericardial effusion. Cardiac monitoring showed pulsus alternans and echocardiogram revealed massive pericardial effusion with the right ventricular wall collapse during systole [Figure 1]. Ultrasonography abdomen reported moderate ascites. Workup for infectious etiology including Mantoux, GeneXpert of lymph node, gastric aspirate for acid-fast bacteria, cultures of blood, urine, bone marrow, pericardial, and pleural fluid) were negative. Lymph node and bone marrow biopsy showed reactive cells. Urine protein/creatinine and 24 h urine protein were slightly elevated, but renal biopsy was normal. Pleural and pericardial fluid showed no malignant cells. Autoimmune workup revealed a low C3-44 (88–206 mg/dl) and low C4-8.7 (13–75 mg/dl), positive direct Coombs test (DCT), anti-nuclear antibody (ANA) 3+ positive by IF (1 in 100 dilution) nucleus granular pattern suggestive of autoantibodies against U1-nRNP/Sm antigen. Lupus anticoagulant and antiphospholipid antibodies both IgG and IgM were negative. Extractable nuclear antigen (ENA profile) was negative.
Figure 1: Echocardiogram showing massive pericardial effusion with the right ventricular wall collapse during systole, pulse oximeter shows alternating small and large electrocardiogram complexes revealing pulsus alternans

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With features of cardiac tamponade (serositis), low C3 C4 levels, positive DCT and ANA the patient fulfilled 4/17 of SLICC 2012 classification criteria for systemic lupus erythematosus (SLE). Hypertension was treated with nifedipine and furosemide. With pericardiocentesis and pulse corticosteroids therapy the child improved dramatically. Hydroxychloroquine and azathioprine were added to therapy. At follow-up, her pericardial effusion had resolved, and we were able to wean her steroids completely. The patient continued on azathioprine and hydroxychloroquine therapy at 1 year after presentation.

  Discussion Top

SLE is a chronic autoimmune multisystem disorder. The actual data regarding the incidence of childhood SLE in India is not known. Estimated incidence around the globe is 0.3–6 per 100,000 children.[1] Pediatric SLE is rare before 5 years of age. Compared to adults, children tend to have more severe disease at onset.[2],[3] Etiology includes genetic, environmental, and hormonal causes. Cardiac involvement with cardiac tamponade is unusual as an initial sole manifestation of the disease in children.

The unusual features, in this case, were the young age of the child at onset of disease and cardiac tamponade being the only presenting manifestation of SLE at this age. Childhood SLE accounts for about 20% among SLE patients; this occurs more often in the second decade of life.[2],[3] The onset of the disease under 5 years of age is very rare. In children, the disease tends to be more severe at presentation.[2],[3] Fever, hematologic manifestations, oral ulcers, nephritis, and neurological manifestations are common presenting clinical features.[2],[3] Cardiac tamponade as the initial manifestation of SLE has been reported in adults, in children in the second decade of life and very rarely under 5 years of age.[4] To the best of our knowledge, this patient is the youngest to present with cardiac tamponade as the only presenting manifestation and to also fulfill SLICC 2012 classification criteria for SLE.[4] Although these criteria are essentially for classification and not for the diagnostic purpose, the presence of at least 4 of these criteria gives more credence to the diagnosis. A renal biopsy was performed as there were hypertension and some proteinuria initially. However, the renal biopsy showed normal histopathology and immunofluorescence was negative.

Young children with complement deficiencies such as C1q and C2 can present with SLE-like disease. However, the most common manifestations in these children are rashes, glomerulonephritis followed by cerebral involvement.[5] Common nonsurgical causes of cardiac tamponade in young children include tuberculosis, other bacterial infections, malignancy, and rheumatic fever.[6] Once these conditions have been ruled out connective tissue disorders should be considered and investigated for. Urgent pericardiocentesis and intravenous high-dose steroids maybe lifesaving in this situation.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Kamphuis S, Silverman ED. Prevalence and burden of pediatric-onset systemic lupus erythematosus. Nat Rev Rheumatol 2010;6:538-46.  Back to cited text no. 1
Ambrose N, Morgan TA, Galloway J, Ionnoau Y, Beresford MW, Isenberg DA; UK JSLE Study Group. Differences in disease phenotype and severity in SLE across age groups. Lupus 2016;25:1542-50.  Back to cited text no. 2
Brunner HI, Gladman DD, Ibañez D, Urowitz MD, Silverman ED. Difference in disease features between childhood-onset and adult-onset systemic lupus erythematosus. Arthritis Rheum 2008;58:556-62.  Back to cited text no. 3
Maharaj SS, Chang SM. Cardiac tamponade as the initial presentation of systemic lupus erythematosus: A case report and review of the literature. Pediatr Rheumatol Online J 2015;13:9.  Back to cited text no. 4
Pickering MC, Walport MJ. Links between complement abnormalities and systemic lupus erythematosus. Rheumatology (Oxford) 2000;39:133-41.  Back to cited text no. 5
Bagri NK, Yadav DK, Agarwal S, Aier T, Gupta V. Pericardial effusion in children: Experience from tertiary care center in Northern India. Indian Pediatr 2014;51:211-3.  Back to cited text no. 6


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