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Case Reports
. 2018 Feb;9(1):68-71.
doi: 10.14740/cr654w. Epub 2018 Feb 11.

Massive Hemorrhagic Pericardial Effusion With Cardiac Tamponade as Initial Manifestation of Mixed Connective Tissue Disease

Ashraf Abugroun  1 , Osama Hallak  1 , Fatima Ahmed  1 , Safwan Gaznabi  1
Affiliations
Case Reports

Massive Hemorrhagic Pericardial Effusion With Cardiac Tamponade as Initial Manifestation of Mixed Connective Tissue Disease

Ashraf Abugroun et al. Cardiol Res. 2018 Feb.

Abstract

Mixed connective tissue disease (MCTD) is a distinct entity of connective tissue disorders characterized by overlapping clinical features of various autoimmune diseases along with the presence of antibodies to ribonucleoprotein (anti-RNP). The prevalence of cardiac involvement in MCTD varies from 13% to 65% and accounts for approximately 20% of MCTD related mortality. In this case, we describe an elderly female patient with multiple complaints without a clear etiology on presentation. Echocardiogram revealed severe rapidly accumulating pericardial effusion causing tamponade necessitating pericardial window. Laboratory investigations showed positive ribonucleoprotein antibodies. Biopsy of pericardial tissue revealed fibrinous pericarditis. While pericarditis is commonly associated with MCTD, pericardial tamponade on the other hand is rarely described. This case highlights a very rare complication of the disease. Early recognition, prompt treatment, and regular follow-up with serial echo are essential for treatment.

Keywords: Cardiac tamponade; Mixed connective tissue disease; Pericardial window; Ribonucleoprotein antibodies.

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Figures

Figure 1
Figure 1
EKG shows sinus rhythm with occasional premature ventricular complexes and low voltage QRS.
Figure 2
Figure 2
Transthoracic echocardiography, subcostal four chamber view showing diastolic collapse of the RV.
Figure 3
Figure 3
CT scan of the chest shows pigtail catheter coursing within the pericardium and around the anterolateral heart border with tip terminating posterior to the left atrium. Mild to moderate residual pericardial effusion along the inferior heart border. Bilateral pleural effusions, small to moderate on the left and small on the right. Moderate to severe emphysematous changes.
Figure 4
Figure 4
Pericardial tissue biopsy showing reactive mesothelial lining correlated with pericarditis.
See this image and copyright information in PMC

References

    1. Sharp GC, Irvin WS, Tan EM, Gould RG, Holman HR. Mixed connective tissue disease—an apparently distinct rheumatic disease syndrome associated with a specific antibody to an extractable nuclear antigen (ENA) Am J Med. 1972;52(2):148–159. doi: 10.1016/0002-9343(72)90064-2. - DOI - PubMed
    1. Gunnarsson R, Andreassen AK, Molberg O, Lexberg AS, Time K, Dhainaut AS, Bertelsen LT. et al. Prevalence of pulmonary hypertension in an unselected, mixed connective tissue disease cohort: results of a nationwide, Norwegian cross-sectional multicentre study and review of current literature. Rheumatology (Oxford) 2013;52(7):1208–1213. doi: 10.1093/rheumatology/kes430. - DOI - PubMed
    1. Venables PJ. Mixed connective tissue disease. Lupus. 2006;15(3):132–137. doi: 10.1191/0961203306lu2283rr. - DOI - PubMed
    1. Aringer M, Steiner G, Smolen JS. Does mixed connective tissue disease exist? Yes. Rheum Dis Clin North Am. 2005;31(3):411–420, v. doi: 10.1016/j.rdc.2005.04.007. - DOI - PubMed
    1. Greidinger EL, Hoffman RW. Autoantibodies in the pathogenesis of mixed connective tissue disease. Rheum Dis Clin North Am. 2005;31(3):437–450. doi: 10.1016/j.rdc.2005.04.004. vi. - DOI - PubMed

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