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 Table of Contents  
Year : 2021  |  Volume : 8  |  Issue : 1  |  Page : 35-38

Systemic autoimmune diseases presenting as acute myocarditis in pediatric emergency: Two case reports

1 Department of Pediatrics, Nil Ratan Sircar Medical College and Hospital, Kolkata, West Bengal, India
2 Department of Pediatrics, Medical College and Hospital, Kolkata, West Bengal, India
3 Department of Pediatrics, North Bengal Medical College and Hospital, Siliguri, West Bengal, India

Date of Submission30-Jul-2020
Date of Decision17-Sep-2020
Date of Acceptance13-Oct-2020
Date of Web Publication08-Jan-2021

Correspondence Address:
Dr. Sayantan Mondal
Talas Co-Operative P-4 C.I.T Scheme-143, Sankar Bose Road Extn., Kolkata - 700 027, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/JPCC.JPCC_119_20

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Children seldom present to the emergency with cardiac manifestations, some of which eventually are diagnosed as manifestations of rheumatological diseases such as systemic lupus erythematosus or arthropathies. While cardiac involvement is known in these cases, their presentation solely as a cardiologic event initially is not widely known. The following two case reports depict how cardiological emergencies ultimately evolve as systemic autoimmune diseases with atypical involvement such as myocarditis with heart failure and are salvaged with appropriate management.

Keywords: Cardiac emergencies, myocarditis, systemic juvenile idiopathic arthritis, systemic lupus erythematosus

How to cite this article:
Mondal S, Das R, Samanta M, Nandi M. Systemic autoimmune diseases presenting as acute myocarditis in pediatric emergency: Two case reports. J Pediatr Crit Care 2021;8:35-8

How to cite this URL:
Mondal S, Das R, Samanta M, Nandi M. Systemic autoimmune diseases presenting as acute myocarditis in pediatric emergency: Two case reports. J Pediatr Crit Care [serial online] 2021 [cited 2021 Jan 26];8:35-8. Available from: http://www.jpcc.org.in/text.asp?2021/8/1/35/306479

  Introduction Top

Childhood rheumatic diseases being heterogeneous, multisystemic in nature, and termed systemic autoimmune diseases have varied clinical manifestations which represent diagnostic and therapeutic challenges such as Systemic lupus erythematosus (SLE) and Systemic juvenile idiopathic arthritis (sJIA). Cardiac complications of lupus have been described since the early 20th century and include pericarditis, endocarditis, coronary artery disease, and rarely myocarditis.[1] The cardiac involvement in sJIA was first described by Still in 1897 with symptomatic events such as pericarditis, perimyocarditis, and heart failure.[2] As manifestation with myocarditis is less known in these disorders and it has a relevant negative burden on prognosis, the following case reports depict this atypical presentation, evolution, and adequate management bringing out a favorable outcome.

  Case Report 1 Top

A young boy of 6-years presented with a history of fever and respiratory distress of increasing severity for about a week, referred as a case of pleural effusion with intercostal chest drain in situ [Figure 1]. He also had an antecedent history of cough for the past 3 months with the loss of weight, though no contact with tuberculosis. Examination revealed tachycardia, tachypnea, bilateral basal crepitations, decreased breath sounds, and gallop rhythm without any murmur. There was tender hepatomegaly (5 cm) without ascites or splenomegaly. Initial investigations revealed anemia (Hb-5 g/dl) with leukocytosis (22,400/cu. mm.) and high erythrocyte sedimentation rate (ESR) (65 mm/1st h), while other laboratory parameters were normal. The pleural fluid analysis was reported to have high protein (5 gm/dl) and cell-count 3500/cu. mm (predominantly lymphocytic and increased mesothelial cells). Electrocardiogram showed ST-T changes and a bedside echocardiography suggested pericarditis with poor biventricular systolic function (ejection fraction [EF]-43%) with mild pericardial effusion. The child was managed in a pediatric intensive care unit (PICU) initially with antitubercular drugs, backed by the above reports alongside management of heart failure.
Figure 1: Chest X-ray of case 1 showing bilateral pleural effusion and left-sided implantable cardioverter-defibrillator in situ

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Although there was the initial response, subsequently, there was an effusion on the right side of the chest requiring intercostal drain. A repeat echocardiography showed further accumulation of pericardial fluid requiring pericardiocentesis. Meanwhile, cartridge-based nucleic acid amplification test reported negative and symptoms of persistent heart failure, recurrent pleural and pericardial effusion, pallor, and chest infiltrates on high-resolution computed tomography prompted for immunological testing and the child was started with pulse methylprednisolone therapy for 5 days. This time the child improved gradually by day 9 of admission and eventually antinuclear antibody report came out to be positive in 1: 320 dilution with low C3 (59 mg/dl), a positive dsDNA, corroborating the diagnosis of SLE with suspected myopericarditis. Here, creatine phosphokinase-MB (CPK-MB) was high though troponin levels were normal. He was continued on oral corticosteroids and kidney biopsy later revealed Class II B lupus nephritis. The child is now doing well on regular follow-up.

  Case Report 2 Top

An eight-year-old girl child was admitted with a history of worsening respiratory distress with palpitations for the past 15 days worsening on the supine position. She had a history of painful restricted neck movements since 4 years of age with painful swelling of large and small joints of bilateral upper and lower extremities along with fever requiring hospital admissions, provisionally diagnosed as sJIA [Figure 2]. The child was noncompliant and lost to follow-up suffering from recurrent similar episodes. This time she had tachycardia, tachypnea, and low volume irregular pulses with hypotension and pallor with generalized lymphadenopathy on admission. There were bilateral basal crepitations with hyperdynamic apex, hepatosplenomegaly, engorged jugular venous pressure, and gross musculoskeletal abnormality with signs of inflammation in small and large joints of the extremities. Investigations revealed anemia (Hb-8.1 gm/dl), high ESR (85 mm in 1st h), and average C-reactive protein (20 mg/dl) with cardiomegaly in chest-X-ray. CPK-MB levels reached 2500 IU/L. Electrocardiogram features were suggestive of type II heart block with ST-T changes in chest leads (V2–V7) with Qtc = 480 ms [Figure 3]. Echocardiography corroborated with heart failure showing left ventricular dysfunction with a left ventricular ejection fraction (LVEF) of 35%. The child was managed in PICU with inotropes and supportive treatment. X-ray of the neck and hand revealed osteopenia around joints, bony fusions with a reduction in joint spaces, and hence this case was diagnosed to be sJIA with cardiac decompensation possibly due to myocarditis. Loading dose of methylprednisolone was initiated along with naproxen and methotrexate as anti-inflammatory therapy. Repeat echocardiography and electrocardiogram showed reversal of abnormalities with LVEF 50% after 5 days of definitive management and now doing well on follow-up.
Figure 2: Bilateral lower extremities in case 2 showing joint changes

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Figure 3: Electrocardiogram tracing at presentation showing rhythm abnormalities in case 2

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  Discussion Top

Various case reports and studies in recent times display evidence that systemic auto immune diseases such as SLE and sJIA, though unusual, may frequently present with cardiological exemplifications.[3],[4] Of almost 30% of children who develops cardiac complications in SLE,[5] the clinical diagnosis of myocarditis happens only in a few by mostly autopsy studies which thereby states the subclinical nature of lupus myocarditis,[6] not overlooking the fatality though where there are reports of death within a week of presentation.[7],[8] While in sJIA, myocarditis with or without pericarditis produces more expressive manifestations such as congestive heart failure and arrhythmias, and even death, as portrayed vividly in recent literature.[2],[9]

Juvenile rheumatologic conditions such as SLE records around 5%–30% cardiac involvement,[4] with a disease course more fulminant when compared with adults. Specifically in SLE though renal involvement tends to be significantly more common, cardiological involvement is still a notch higher (prevalence of myo/pericarditis 10.8% in 5–17 years, 6.2% in 18–44 years, and 5.3% in 45 years and above age groups) than adults, making evaluation all the more relevant.[10]

Elevation of cardiac enzymes specifically troponins, are associated significantly with the presence of myocarditis, though it has average sensitivity[11] and even low Trop T/I may be associated with myocarditis in autoimmune diseases. The time frame for assessment becomes important for the elevated results, done at a later date in our case, yet elevated CK-MB here was rather unusual, may be explained by the systemic nature of the disease itself.[12] Endomyocardial biopsy is a gold standard for the diagnosis of myocarditis,[13] but carries a high risk of mortality in sick children, hence was not undertaken here. In such a scenario, the presence of global hypokinesia with a low LVEF on echocardiography strongly points toward the diagnosis as found in these cases.

Recently, cardiac involvement in systemic autoimmune diseases can be assessed by cardiac magnetic resonance (CMR) imaging with T1 and T2 weighted images for tissue characterization.[14] Late gadolinium enhancement CMR helps in categorization and prognostication by Lake Louis classification,[6] but was out of scope in our set up.

Myocarditis in sJIA was due to disease activity, clinically evident by serositis, raised inflammatory parameters, and corroborated by improvement with immunosuppressants. Treatment with nonsteroidal anti-inflammatory drugs, disease-modifying antirheumatic drugs such as methotrexate and glucocorticoids have been recommended by the American college of rheumatology guidelines, yielding beneficial results even in our case.[15] Anakinra and tocilizumab (interleukin-1 and 6 receptor antagonists, respectively) are now being described as new modalities of treatment in some case reports.[16]

Immunosuppressive drugs and decongestants remain the treatment of choice till now, alongside supportive medical therapy and intensive care facilities due to lacking evidence for treatment of lupus myocarditis.[17] Intravenous immunoglobulins play a controversial role, indicated mostly in viral myocarditis, while in lupus myocarditis, immunosuppression in the form of pulse corticosteroid therapy, cyclophosphamide, mycophenolate mofetil, and even rituximab has been used effectively and documented in various studies and case reports, as similar to the present scenarios showing marked improvement.[4],[18],[19],[20]

  Conclusion Top

Above two cases highlight that systemic autoimmune diseases like SLE and sJIA can present with features of acute myocarditis with heart failure as initial manifestations in the emergency department, requiring urgent intensive care management. Myocarditis associated with multiorgan involvement can be a pointer toward SIDs in such cases.


The authors are thankful to Dr. T.K. Sinha Mahapatra, Head of the Department of Pediatrics, NRSMCH for his constant support.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

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Svantesson H. Bjorkhem G, Elborgh R. Cardiac involvement in juvenile rheumatoid arthritis. Acta Paediatr Stand 1983;72:345-50.  Back to cited text no. 2
Baquero G, Banchs JE, Naccarelli GV, Gonzalez M, Wolbrette DL. Cardiogenic shock as the initial presentation of systemic lupus erythematosus: A case report and review of the literature. Congest Heart Fail 2012;18:337-41.  Back to cited text no. 3
Mondal R, Sarkar S, Ghosal A, Sabui T. Congestive heart failure: An uncommon presentation of systemic onset juvenile idiopathic arthritis (SOJIA). Indian J Pediatr 2013;80:67-9.  Back to cited text no. 4
Cassidy JT, Petty RE, Laxer RM, Lindsay CB, Wedderburn LC. Systemic lupus erythematosus, systemic juvenile onset idiopathic arthritis. In: Textbook of Pediatric Rheumatology. 7th ed. Philadelphia: Elsevier; 2016. p. 205-15, 285-316.  Back to cited text no. 5
Wijetunga M, Rockson S. Myocarditis in systemic lupus erythematosus. Am J Med 2002;113:419-23.  Back to cited text no. 6
Acharya S, Pal P, Giri PP, Saha A. Myocarditis with cardiac failure: Initial manifestation of systemic lupus erythematosus. J Clin Diagn Res 2019;13:SD01-2.  Back to cited text no. 7
Gupta A, Singh S, Minz RW, Radotra BD, Ahluwalia J, Grover A. Lupus myocarditis in children. Ann Rheum Dis 2004;63:745-6.  Back to cited text no. 8
Goldenberg J, Ferraz MB, Pessoa AP, Fonseca AS, Carvalho AC, Hilario MO, et al. Symptomatic cardiac involvement in juvenile rheumatoid arthritis. Int J Cardiol 1992;34:57-62.  Back to cited text no. 9
Chang JC, Xiao R, Mercer-Rosa L, Knight AM, Weiss PF. Child-onset systemic lupus erythematosus is associated with a higher incidence of myopericardial manifestations compared to adult-onset disease. Lupus 2018;27:2146-54.  Back to cited text no. 10
Smith SC, Ladenson JH, Mason JW, Jaffe AS. Elevations of cardiac troponin I associated with myocarditis. Experimental and clinical correlates. Circulation 1997;95:163-8.  Back to cited text no. 11
Lauer B, Niederau C, Kühl U, Schannwell M, Pauschinger M, Strauer E, et al. Cardiac troponin T in patients with clinically suspected myocarditis. Journal of the American College of Cardiology 1997;30: 1354-9. https://doi.org/10.1016/S0735-1097(97)00317-3.  Back to cited text no. 12
Tunuguntla H, Jeewa A, Denfield SW. Acute myocarditis and pericarditis in children. Pediatr Rev 2019;40:14-25.  Back to cited text no. 13
Lagan J, Schmitt M, Miller CA. Clinical applications of multi-parametric CMR in myocarditis and systemic inflammatoy diseases. Int J Cardiovascular Imaging 2018;34:35-54.  Back to cited text no. 14
Sarah R, Angeles-Han ST, Timothy B, Lovell D, Cuello CA, Becker ML, et al. American college of rheumatology/arthritis foundation guideline for the treatment of juvenile idiopathic arthritis: Therapeutic approaches for non-systemic polyarthritis, sacroiliitis, and enthesitis. Arthritis Care Res 2019;71:717-34.  Back to cited text no. 15
Sriharsh G, Shenoi S. Update on the management of systemic juvenile idiopathic arthritis and role of IL-1 and IL-6 inhibition. Adolesc Health Med Ther 2017;8:125-35.  Back to cited text no. 16
Ansari A, Larson PH, Bates HD. Cardiovascular manifestations of systemic lupus erythematosus: Current perspective. Prog Cardiovasc Dis 1985;27:421-34.  Back to cited text no. 17
Aggarwal P, Singh S, Suri D, Rawat A, Narula N, ManojKumar R. Rituximab in childhood lupus myocarditis. Rheumatol Int 2012;32:1843-4.  Back to cited text no. 18
Klein-Gitelman M, Sundel R, Te Pas E. Systemic lupus erythematosus (SLE) in children: Treatment, complications, and prognosis. UpToDate. Available from: https://www.uptodate.com/contents/systemic-lupus-erythematosus-sle-in-children-treatment-complications-andprognosis. [Last updated on 2019 Sep 09].  Back to cited text no. 19
Allan CK, Fulton DR. Treatment and prognosis of myocarditis in children.[Internet] UpToDate. Available from: https://www.uptodate.com/contents/treatment-and-prognosis-of-myocarditis-in-children. [Last updated on 2019 Sep 07].  Back to cited text no. 20


  [Figure 1], [Figure 2], [Figure 3]


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