The heart in vasculitis
Eli Miloslavsky1, Sebastian Unizony
1Rheumatology Unit, Division of Rheumatology, Allergy, and Immunology, Department of Medicine, Massachusetts General Hospital, Yawkey 2, 55 Fruit Street, Boston, MA 02114, USA.
Insights
Primary vasculitides frequently affect the heart, with specific types like Takayasu arteritis (TAK) and giant cell arteritis (GCA) posing significant risks. Early diagnosis and tailored treatments are crucial for managing cardiac complications and improving patient outcomes.
Area of Science:
- Cardiology
- Rheumatology
- Immunology
Background:
- Primary vasculitides are systemic inflammatory diseases affecting blood vessels.
- Cardiac involvement is a known but often underappreciated complication across various vasculitic syndromes.
- Specific vasculitides like Takayasu arteritis (TAK), polyarteritis nodosa (PAN), and eosinophilic granulomatosis with polyangiitis (EGPA) have higher cardiac predilection.
Purpose of the Study:
- To review the spectrum of cardiac manifestations in primary vasculitides.
- To highlight the diagnostic and prognostic significance of cardiac involvement.
- To outline current management strategies for cardiac complications in vasculitis.
Main Methods:
- Comprehensive literature review of primary vasculitides and cardiac involvement.
- Analysis of epidemiological data and clinical case series.
- Synthesis of diagnostic imaging findings and pathological evidence.
- Review of treatment guidelines and therapeutic outcomes.
Main Results:
- Pericarditis, myocarditis, coronary arteritis, aortitis, and intracavitary thrombus are key cardiac complications.
- Takayasu arteritis (TAK), polyarteritis nodosa (PAN), and eosinophilic granulomatosis with polyangiitis (EGPA) are frequently associated with cardiac issues.
- Subclinical cardiac involvement is common across multiple vasculitides, including giant cell arteritis (GCA) and granulomatosis with polyangiitis (GPA).
- Myocarditis, coronary arteritis, and valvular disease are poor prognostic indicators, often leading to heart failure.
Conclusions:
- Cardiac involvement in primary vasculitides necessitates vigilant monitoring and prompt, aggressive management.
- Treatment strategies must be individualized based on the affected cardiac structure and disease activity.
- Addressing cardiovascular risk factors and employing heart failure-targeted therapies are essential adjuncts to immunosuppressive treatment.
Abstract:
All primary vasculitides can target the heart, but this complication is more frequent in TAK, PAN, and EGPA. Although pericarditis is seen in virtually all forms of vasculitis, it rarely becomes a significant clinical problem. Myocarditis is more prevalent in EGPA and TAK, and coronary angiitis is most common in TAK, PAN, and BD. In addition, AI is a classic complication of TAK-induced aortitis, and intracavitary cardiac thrombus formation mainly affects patients with BD. Myocarditis, coronary arteritis, and valvular disease can lead to congestive heart failure and represent poor prognostic factors that require aggressive therapy. Imaging and pathology studies have found that subclinical involvement is common (e.g., TAK, PAN, EGPA, GPA, and BD). Management differs depending on the cardiac structure involved and the activity of the disease.Although pericarditis can be treated with NSAIDs, colchicine, or-low dose prednisone, myocarditis and coronary vasculitis require high doses of CS and frequently cytotoxic agents. Valvular lesions, coronary arteriopathy, and ventricular thrombosis often need surgical intervention. In the face of active disease, clinical judgment is important to help weigh the risks and benefits of delaying surgery versus operating in possibly inflamed tissues. As in other rheumatic diseases, risks factors for atherosclerosis (eg, hypertension, dyslipidemia) should be identified and corrected. Finally, heart failure–and ischemia-targeted therapies are important components of the treatment strategy when indicated.
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