Home Print this page Email this page Small font size Default font size Increase font size
Users Online: 4245
Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Subscribe Contacts Login 


 
 Table of Contents 
CASE REPORT
Year : 2020  |  Volume : 9  |  Issue : 8  |  Page : 4441-4443  

Lupus myocarditis-A rare case


Department of Medicine, Tata Main Hospital, Jamshedpur, Jharkhand, India

Date of Submission28-Apr-2020
Date of Decision11-Jun-2020
Date of Acceptance01-Jul-2020
Date of Web Publication25-Aug-2020

Correspondence Address:
Dr. Bijaya Mohanty
AC11 K, L, 11th Phase, Adarsh Nagar, Sonari, Jamshedpur - 831 011, Jharkhand
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jfmpc.jfmpc_716_20

Rights and Permissions
  Abstract 


Myocarditis in patients of systemic lupus erythematosus is extremely rare and is potentially life threatening. This may be the first presentation of the disease. Here, we report a patient who presented with features of heart failure and was later diagnosed to have SLE.

Keywords: Cardiogenic shock, myocarditis, systemic lupus erythematosous


How to cite this article:
Mohanty B, Sunder A. Lupus myocarditis-A rare case. J Family Med Prim Care 2020;9:4441-3

How to cite this URL:
Mohanty B, Sunder A. Lupus myocarditis-A rare case. J Family Med Prim Care [serial online] 2020 [cited 2020 Sep 28];9:4441-3. Available from: http://www.jfmpc.com/text.asp?2020/9/8/4441/293074




  Introduction Top


Lupus myocarditis is a potentially life-threatening condition, clinically found in 3–9% of SLE patients.[1] However, the incidence is as high as 57% (post mortem analyses), suggesting a high prevalence of subclinical disease.[2] Patients may present with complains of dyspnea, orthopnea, chest pain, pedal edema, fever, diaphoresis, paroxysmal nocturnal dyspnea, nausea, vomiting, or palpitations. Myocarditis may progress to arrhythmias, conduction disturbances, dilated cardiomyopathy, and heart failure which need to be identified and treated early.[3] The pathophysiology of myocardial dysfunction in SLE is usually multifactorial. The prime factors involved are immunological injury and ischemia. Here, we are reporting a case who presented with features of heart failure and was initially thought to be due to diabetes and hypertension per se but was later diagnosed to have lupus myocarditis. She improved with steroids, immunosuppressive agents, and other supportive care.


  Case Summary Top


A 40-year-old female was admitted with complaints of shortness of breath at rest for three days. She was diagnosed with type-2 diabetes mellitus, hypertension, and hypothyroidism and was on regular medication. She also had history of pain involving large joints along with low-grade intermittent fever.

Upon examination she was febrile and dyspnoeic. Pulse was 110/min, regular, and blood pressure was 90/50 mmHg. The jugular venous pressure was raised. There was mild pallor and bilateral pedal edema. There was no cyanosis, clubbing, icterus, or lymphadenopathy. Examination of chest revealed bilateral basal creptitions. Cardiovascular system examination revealed normal heart sounds. There was no pericardial rub, murmur, or gallop. Liver was enlarged and tender. Examination of the musculosleletal system showed Z-shaped thumbs, swan neck deformities of the distal interphalangeal joints, and reduced flexion at the metacarpophalangeal (MCP) joints [Figure 1]. X-ray of the hands confirmed nonerosive disease suggestive of Jaccoud arthropathy [Figure 2]. She also had oral ulcers.
Figure 1: Hand deformity

Click here to view
Figure 2: X-ray of hands showing jaccouds arthropathy

Click here to view


Upon investigation, there was normocytic normochromic anemia with a high ESR of 60 mm. The CRP was high (17.38) with slightly raised creatinine and urine ACR was 486.71 mg/g. While in hospital, she deteriorated rapidly developing significant dyspnea and hypoxemia, hypotension, azotemia, and decreased urine output. She was shifted to the intensive care unit and started on noninvasive ventilation and needed double inotropic support.

ECG showed sinus tachycardia [Figure 3], chest X-ray revealed cardiomegaly, and bilateral pleural effusion [Figure 4]. Echocardiogram showed generalized wall hypokinesia with an ejection fraction of 36%, mild mitral and tricuspid regurgitation along with minimal pericardial effusion.
Figure 3: ECG-sinus tachycardia

Click here to view
Figure 4: Chest X-Ray- cardiomegaly

Click here to view


On further workup, ANA & DsDNA were positive. She was diagnosed with systemic lupus erythematosous. She was treated with intravenous methylprednisolone followed by oral prednisolone, hydroxychloroquine and mycophenolate mofetil. She improved and was shifted out of intensive care unit. Finally, she was discharged on mycophenolate mofetil, hydroxychloroquine, oral diabetic agents and deflazacort. She is on regular follow up and a repeat echo after three months showed normal ejection fraction (70%).


  Discussion Top


Systemic lupus erythematosous patients can present with features of myocarditis, pericarditis, endocarditis, valvular lesions, and conduction abnormalities. Cardiovascular involvement in SLE might be associated with disease severity and activity.[4] Cardiac involvement as an initial manifestation of SLE is rare but when present it is associated with significant morbidity and mortality in 50% of cases.[5]

Endomyocardial biopsy is one of the modality for diagnosis of myocarditis but is less sensitive due to the focal nature of the disease. The histopathology report usually shows areas of myocardial fibrosis with sparse interstitial mononuclear cell infiltrates. There may be areas of occasional myocyte necrosis with immune complex deposition. The diagnosis is usually based on clinical and imaging studies. In our case, the diagnosis of SLE was made based on the basis of SLICC criteria. Our patient had features of serositis (pleural and pericardial effusions), positive ANA and anti-dsDNA. Jaccoud arthropathy (JA) with the involvement of the metacarpophalangeal (MCP), proximal interphalangeal joints and wrists was a clue in our case.

Primary lupus myocarditis is uncommon. There are few reports of heart failure and acute myocarditis as the initial manifestation of SLE.[5],[6],[7],[8],[9],[10],[11] Borenstein et al. found only five cases of myocarditis in 140 patients with SLE.[12] Badui et al. have reported 14% incidence in a prospective study.[13] Cheng et al. reported one such case for the first time who presented with severe LV dysfunction without other manifestations of SLE.[14] Tanwani et al. reported a series of patients of myocarditis in 2018.[15] Immune-complex deposition with complement activation is the primary pathogenetic mechanism in myocardial lesions.

Treatment with high-dose corticosteroids has been shown to improve left ventricular function in lupus myocarditis.[16],[17] Other drugs used in treatment are azathioprine, cyclophosphamide, rituximab and intravenous immunoglobulins. Sandrasegaran et al. reported a patient of SLE presenting with myocarditis who responded dramatically to steroid therapy.[18] Two case reports were published by Shamma Ahmed et al. where it was a challenge to diagnose lupus myocarditis.[19]

There were challenges and difficulties in the diagnosis of lupus myocarditis mainly due to the presence of other factors potentially responsible for myocardial damage, such as anemia, hypertension, diabetes, and hypothyroidism. Initially, she had a slow response to therapy oriented with diuretics, intravenous vasodilators, and noninvasive mechanical ventilation but later on showed good response after the addition of intravenous methylprednisolone. Therefore, it is essential for the family physicians to identify and treat these cases early.

We followed up the case and with steroids and mycophenolate mofetil. Her left ventricular function improved dramatically to normal. Though myocarditis is a rare initial presentation of SLE, its timely diagnosis is of paramount importance to prevent morbidity and mortality. The mainstays of treatment are corticosteroids, immunosuppressive agents, and other supportive care.


  Conclusion Top


The purpose of reporting this case is to emphasize the importance of systemic lupus as a differential diagnosis in cases who present with features of myocarditis along with comorbid conditions. A high degree of clinical suspicion, early diagnosis, and supportive care in combination with immunosuppressants may lead to favorable outcomes.

Institutional ethical permission

Taken

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patients have given their consent for 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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Miner JJ, Kim AH. Cardiac manifestations of systemic lupus erythematosus. Rheum Dis Clin North Am 2014;40:51-60.  Back to cited text no. 1
    
2.
Wijetunga M, Rockson S. Myocarditis in systemic lupus erythematosus. Am J Med 2002;113:419-23.  Back to cited text no. 2
    
3.
Doherty NE, Siegel RJ. Cardiovascular manifestations of systemic lupus erythematosus. Am Heart J 1985;110:1257-65.  Back to cited text no. 3
    
4.
Doria A, Iaccarino L, Sarzi-Puttini P, Atzeni F, Turriel M, Petri M. Cardiovascular involvement in systemic lupus erythematosus. Lupus 2005;14:683-6.  Back to cited text no. 4
    
5.
Nossent J, Cikes N, Kiss E, Marchesoni A Nassono V, Mosca M, et al. Current causes of death in systemic lupus erythematosus in Europe, 2000-2004: Relation to disease activity and damage accrual. Lupus 2007;16:309-17.  Back to cited text no. 5
    
6.
Woo SL, Hwang GS, Kang SJ, Park JS, Park SJ, Lee YS, et al. Lupus myocarditis presenting as acute congestive heart failure: A case report. J Korean Med Sci 2009;24:176-8.  Back to cited text no. 6
    
7.
Law WG, Thong BY, Lian TY, Kong KO, Chng HH. Acute lupus myocarditis: Clinical features andoutcome of an oriental case series. Lupus 2005;14:827-31.  Back to cited text no. 7
    
8.
Van der Laan-Baalbergen NE, Mollema SA, Kritikos H, Schoe A, Huizinga TW, Bax JJ, et al. Heart failure as presenting manifestation of cardiac involvement in systemic lupus erythematosus. Neth J Med 2009;67:295-301.  Back to cited text no. 8
    
9.
Sandrasegaran K, Clarke CW, Nagendran V. Subclinical systemic lupus erythematosus presenting with acute myocarditis. Postgrad Med J 1992;68:475-8.  Back to cited text no. 9
    
10.
Sayin T, Kýnýklý G, Döven O, Karaoǧuz R. Systemic lupus erythematous mimics heart failure. Int J Cardiol 2001;78:95-96.  Back to cited text no. 10
    
11.
Usalan C, Buyukhatipoglu H, Tiryaki O. Systemic lupus erythematosus complicated by dilated cardiomyopathy and severe heart failure. Clin Rheumatol 2007;26:125-7.  Back to cited text no. 11
    
12.
Borenstain DG, Fye WB, Arnett FC, Stevens MB. The myocarditis of systemic lupus erythematosus: Association with myositis. Ann Intern Med 1978;89:619-24.  Back to cited text no. 12
    
13.
Badui E, Garcia-Rubi D, Robles E, Jimenez J, Juan L, Deleze M, et al. Cardiovascular manifestations in systemic erythematosus : Prospective study of 100 patients. Angiology 1985;36:431-40.  Back to cited text no. 13
    
14.
Cheng SM, Chang DM, Lee WH, Ding YA. Acute myocarditis as an initial manifestation of Systemic Lupus Erythematosus: A case report. Chung Hua I Msuch Tsa Chih-Chinese Med J 1996;58:205-8.  Back to cited text no. 14
    
15.
Tanwani J, Tselios K, Gladman DD, Su J, Urowitz MB. Lupus myocarditis: A single center experience and a comparative analysis of observational cohort studies. Lupus 2018;27:1296-302.  Back to cited text no. 15
    
16.
Zhang L, Zhu YL, Li MT, Gao N, You X, Wu QJ, et al. Lupus myocarditis: A case-control study from China. Chin Med J 2015;128:2588-94.  Back to cited text no. 16
[PUBMED]  [Full text]  
17.
Barnado A, Kamen DL. Myocarditis successfully treated with intravenous immunoglobulin in a patient with systemic lupus erythematous and myositis. Am J Med Sci 2014;347:256-7.  Back to cited text no. 17
    
18.
Abraham H, Kuzhively J, Rizvi S. Heart failure-a rare initial manifestation of systemic lupus erythematosus. MOJ Clin Med Case Rep 2016;5:214-5.  Back to cited text no. 18
    
19.
Al-Nokhatha SA, Khogali HI, Al Shehhi MA, Jassim IT. Myocarditis as a lupus challenge: Two case reports. J Med Case Rep 2019;13:343.  Back to cited text no. 19
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

Top
   
 
  Search
 
Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

 
  In this article
   Abstract
  Introduction
  Case Summary
  Discussion
  Conclusion
   References
   Article Figures

 Article Access Statistics
    Viewed91    
    Printed2    
    Emailed0    
    PDF Downloaded20    
    Comments [Add]    

Recommend this journal