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CASE REPORT
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Case report of a lupus patient with flare and symptomatic cardiac tamponade and macrophage activation syndrome: A biochemical diagnostic perspective


 Department of Biochemistry, Armed Forces Medical College, Pune, Maharashtra, India

Date of Submission05-May-2020
Date of Decision03-Jul-2020
Date of Acceptance19-Jul-2020

Correspondence Address:
Kapil Bhatia,
Department of Biochemistry, Armed Forces Medical College, 3rd Floor, Diamond Jubilee Block, Pune - 411 040, Maharashtra
India
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/mjdrdypu.mjdrdypu_195_20

  Abstract 


Systemic lupus erythematosus (SLE) is an autoimmune disorder with multifactorial etiology involving vital organs like the heart and causing rare complications like cardiac tamponade which are indeed difficult to manage especially in a setting of macrophage activation syndrome (MAS) with flare. A case of 25-year-old female with flare having high.grade fever and breathlessness on exertion to begin with. Subsequently, she developed pericardial effusion leading to cardiac tamponade and was managed with pericardiocentesis. With the rising ferritin levels, raised triglycerides, and cytopenia, she was diagnosed as MAS, along with reactivation of tuberculosis. She was discharged on oral steroids and anti-tubercular therapy with regular follow-up. Clinical suspicion, keeping in mind, the rarest complications such as cardiac tamponade, and timely employability of biochemical tests can help in early diagnosis of MAS and can lead to early therapeutic intervention in SLE patients preventing fatal outcomes.

Keywords: Anti-tubercular therapy, anti-nuclear antibody, macrophage activation syndrome, pericardial effusion, systemic lupus erythematosus



How to cite this URL:
Singh A, Bhatia K, Misra P, Mukherjee B, Ambade VN. Case report of a lupus patient with flare and symptomatic cardiac tamponade and macrophage activation syndrome: A biochemical diagnostic perspective. Med J DY Patil Vidyapeeth [Epub ahead of print] [cited 2021 Nov 30]. Available from: https://www.mjdrdypv.org/preprintarticle.asp?id=308996




  Introduction Top


Systemic lupus erythematosus (SLE) is a heterogeneous autoimmune disorder having a variable course and prognosis.[1] It is common primarily in women in their childbearing age (20–40 years).[2] The prevalence of SLE is 3 per 100,000 in India.[3] The etiology of SLE involves multiple factors such as genetic, hormonal, immunological, and environmental. A minimum of 4 out of 11 criteria should be met to confirm the diagnosis of SLE [Table 1]. Cases with only organ involvement or presenting with some manifestations of disease are classified as having “Incomplete” or “Latent” lupus.[4] SLE usually occurs in phases which are flare, chronic, and long quiescence. Patients with long quiescence have a long remission period before having additional flare up.[5] Clearly, asymptomatic pericardial involvement with SLE occurs more frequently than the clinical pericarditis.[6]
Table 1: Criteria for systemic lupus erythematosus

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The authors present a case report of a female patient suffering from SLE, reported with symptomatic cardiac involvement, subsequently developing macrophage activation syndrome (MAS).[7]


  Case Report Top


A 25-year-old female reported following a febrile illness involving joint pains which started in knees and then progressing to small joints in hands and foot, along with erythematous annular rash on the malar areas of face, neck, back, and both forearms. History revealed tubercular lymphadenitis treated with anti-tubercular therapy (ATT). On investigating, she had rheumatoid factor (rheumatoid arthritis) positive, anti-nuclear antibody, and anti-cardiolipin antibody positive by immunofluorescence. The patient recovered with steroids and immunosuppressive drugs.

She again reported with flare of disease along with high-grade fever, loose motion, and vomiting and was managed with oral antibiotics and steroids but in view of leukopenia, the immunosuppressive drug azathioprine was stopped, and she was discharged.

After 3 weeks, she came with flare and breathlessness at rest. She underwent complete evaluation as per investigations summarized in [Table 2]. While her breathlessness was progressive, two-dimensional (2D) echocardiography revealed massive pericardial effusion for which she underwent pericardiocentesis with a pigtail catheter placed in situ. She improved clinically and her dyspnea subsided with evident parameters. During further investigations, she was found to have raised serum ferritin of 1150 ng/L, raised fibrinogen with pancytopenia, raised triglycerides from 142 to 259 mg/dL along with continuous fever, which qualifies the criteria for MAS as described in [Table 3].
Table 2: Investigations

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Table 3: Criteria for macrophage activation syndrome in systemic lupus erythematosus

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Due to continuous fever, bone marrow biopsy was done which revealed disseminated tuberculosis on the basis of granuloma and positive acid-fast bacilli. She was then started on ATT along with insulin, oral prednisolone, and hydroxychloroquine.

Later, due to persistent fever with bilateral pitting pedal edema and hypoalbuminemia, her intravenous antibiotics were resumed. All her blood parameters for renal functions were found normal except hemoglobin of 8.9 g/dL, lactate dehydrogenase of 452 IU/L, albumin of 2 g/dL, and calcium of 6.0 mg/dL. Ultrasonography of abdomen was done to rule out any hemolysis which revealed splenomegaly of around 13.6 cm. After 3 weeks of ATT, her fever subsided, and she was then discharged.


  Discussion Top


Asymptomatic cardiac involvement is common in SLE. It is challenging when a rare entity like cardiac tamponade is involved as it requires a close clinical suspicion and work up. The cardiac involvement includes various manifestations as mentioned in [Table 4].[8] In SLE the immune complex deposition leads to inflammatory response involving pericardium, myocardium, and blood vessels that causes complement activation and polymorph invasion and hence damage. In addition, the drugs such as azathioprine used for treating SLE also induce endothelial dysfunction.
Table 4: Cardiovascular manifestations in patients with systemic lupus erythematosus

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The main diagnostic intervention involves 2D echocardiography at this step. In view of cardiac tamponade, pericardiocentesis was done and some 430 mL of chylous fluid was drained with pig tail catheter in situ. Due to continuous fever and infection with underlying flare, the patient was managed with maximal tolerated dose of antibiotic along with modified immunosuppression and pulse steroids. The patient had infection fever, polyserositis, proteinuria, low C3–C4 counts, anemia, and thrombocytopenia as a form of flare activity of SLE leading to MAS.[9] Clinical and laboratory signs such as fever and pancytopenia are more common in SLE associated MAS. The more deranged laboratory parameters, the more the mortality in SLE with MAS. No clear cut guidelines have been established for SLE associated MAS. However, in our experience, combination of corticosteroids with other immunosuppressive drugs was beneficial. Early identification of MAS in SLE patients can be identified with red flags such as continuous fever, raised ferritin levels, and pancytopenia, which can improve the outcome.


  Conclusion and Clinical Significance Top


Mortality and morbidity in SLE are commonly associated with cardiac involvement and MAS. Life-threatening flare along with rare complications like cardiac tamponade is difficult to manage, hence keeping in mind early clinical signs and laboratory work up for cardiac events in SLE with MAS can prove beneficial and can improve the outcome.

Patient's consent

Taken.

Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Manson JJ, Rahman A. Systemic lupus erythematosus. Orphanet J Rare Dis 2006;1:1-6.  Back to cited text no. 1
    
2.
Maidhof W, Hilas O. Lupus: An overview of the disease and management options. P T 2012;37:240-9.  Back to cited text no. 2
    
3.
Kumar A. Indian guidelines on the management of SLE. J Indian Rheumatol Assoc 2002;10:80-96.  Back to cited text no. 3
    
4.
Swaak AJ, van de Brink H, Smeenk RJ, Manger K, Kalden JR, Tosi S, et al. Incomplete lupus erythematosus: Results of a multicentre study under the supervision of the EULAR Standing Committee on International Clinical Studies Including Therapeutic Trials (ESCISIT). Rheumatology (Oxford) 2001;40:89-94.  Back to cited text no. 4
    
5.
Györi N, Giannakou I, Chatzidionysiou K, Magder L, van Vollenhoven RF, Petri M. Disease activity patterns over time in patients with SLE: Analysis of the Hopkins Lupus Cohort. Lupus Sci Med 2017;4:e000192.  Back to cited text no. 5
    
6.
Moder KG, Miller TD, Tazelaar HD. Cardiac involvement in systemic lupus erythematosus. Mayo Clin Proc 1999;74:275-84.  Back to cited text no. 6
    
7.
Liu AC, Yang Y, Li MT, Jia Y, Chen S, Ye S, et al. Macrophage activation syndrome in systemic lupus erythematosus: A multicenter, case-control study in China. Clin Rheumatol 2018;37:93-100.  Back to cited text no. 7
    
8.
Gemal Lanzieri P, Otávio Cardoso Mocarzel L, Azêdo Montes R, Altenburg Odebrecht Curi Gismondi R, Tinoco Mesquita C, Systemic lupus erythematosus: Review of cardiovascular aspects. Int J Cardiovasc Sci 2015;28:251-61.  Back to cited text no. 8
    
9.
Dall'Ara F, Cavazzana I, Frassi M, Taraborelli M, Fredi M, Franceschini F, et al. Macrophage activation syndrome in adult systemic lupus erythematosus: Report of seven adult cases from a single Italian rheumatology center. Reumatismo 2018;70:100-5.  Back to cited text no. 9
    



 
 
    Tables

  [Table 1], [Table 2], [Table 3], [Table 4]



 

 
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