Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2017:2017:2908185.
doi: 10.1155/2017/2908185. Epub 2017 Jan 29.

Antiproteinase 3 Positive Eosinophilic Granulomatosis with Polyangiitis Presenting with Heart Failure and Intraventricular Thrombosis

Dan Zhu  1 , Yiming Luo  2 , Xiangyuan Liu  3 , Lingyun Zu  1
Affiliations
Case Reports

Antiproteinase 3 Positive Eosinophilic Granulomatosis with Polyangiitis Presenting with Heart Failure and Intraventricular Thrombosis

Dan Zhu et al. Case Rep Rheumatol. 2017.

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis commonly with cardiac complications. We describe a case of anti-PR3 ANCA-positive EGPA complicated by congestive heart failure and intraventricular thrombosis. Interestingly, the thrombus was resolved rapidly with steroid and cyclophosphamide in the setting of interrupted anticoagulation. To the best of our knowledge, we report the first case of anti-PR3 positive EGPA with extensive cardiac involvement. Our patient had overlapping features with previously studied ANCA-positive and ANCA-negative EGPA cases. We also hypothesize that the thrombogenic potential of eosinophils may play a central role in thrombogenesis in EGPA and aggressive immunosuppressive therapy remains the cornerstone of treatment, and the addition of anticoagulation therapy in the setting of thrombus formation and also very high risk of bleeding needs to be considered cautiously.

PubMed Disclaimer

Conflict of interest statement

The authors declare that there is no conflict of interests regarding the publication of this paper.

Figures

Figure 1
Figure 1
Chest CT. It shows bilateral patchy ground-glass opacities and subpleural nodules.
Figure 2
Figure 2
Transthoracic Echocardiogram (TTE). A left ventricular apical intramural thrombus can be visualized.
See this image and copyright information in PMC

References

    1. Watts R., Lane S., Hanslik T., et al. Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Annals of the Rheumatic Diseases. 2007;66(2):222–227. doi: 10.1136/ard.2006.054593. - DOI - PMC - PubMed
    1. Masi A. T., Hunder G. G., Lie J. T., et al. The American college of rheumatology 1990 criteria for the classification of churg-strauss syndrome (allergic granulomatosis and angiitis) Arthritis and Rheumatism. 1990;33(8):1094–1100. - PubMed
    1. Mahr A., Moosig F., Neumann T., et al. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): evolutions in classification, etiopathogenesis, assessment and management. Current Opinion in Rheumatology. 2014;26(1):16–23. doi: 10.1097/bor.0000000000000015. - DOI - PubMed
    1. Comarmond C., Pagnoux C., Khellaf M., et al. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): clinical characteristics and long-term followup of the 383 patients enrolled in the French Vasculitis Study Group cohort. Arthritis and Rheumatism. 2013;65(1):270–281. doi: 10.1002/art.37721. - DOI - PubMed
    1. Cui Z., Zhao M.-H., Segelmark M., Hellmark T. Natural autoantibodies to myeloperoxidase, proteinase 3, and the glomerular basement membrane are present in normal individuals. Kidney International. 2010;78(6):590–597. doi: 10.1038/ki.2010.198. - DOI - PubMed

Publication types

LinkOut - more resources