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Cardiac Involvement in Cryoglobulinemia: Clinical Characteristics, Radiological Features, and Outcomes
Hongxiao Han1,2, Kaini Shen2, Yubo Guo3
1Department of Internal Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China.
Insights
Cardiac involvement in cryoglobulinemia (CG) is rare but serious. Early B-cell targeted therapy shows initial promise for CG heart disease, but relapses are common, necessitating prompt intervention.
Area of Science:
- Cardiology
- Rheumatology
- Hematology
Background:
- Cardiac involvement in cryoglobulinemia (CG) is a rare but potentially fatal complication.
- The clinical spectrum and management of cardiac involvement in CG remain poorly understood.
Purpose of the Study:
- To analyze the clinical characteristics, radiological features, and management outcomes of cardiac involvement in patients with CG.
- To highlight the importance of considering cardiac involvement in CG patients with unexplained cardiac symptoms.
Main Methods:
- Retrospective analysis of 11 patients with cardiac involvement among 885 CG patients.
- Review of clinical data, echocardiography, cardiac MRI, and treatment regimens.
- Assessment of treatment response, relapse rates, and survival outcomes during a median follow-up of 57 months.
Main Results:
- 1.2% of CG patients (11/885) had cardiac involvement, presenting with symptoms like dyspnea and chest tightness.
- Elevated N-terminal pro-B-type natriuretic peptide and diverse echocardiographic findings including left heart enlargement and reduced ejection fraction were common.
- B-cell targeted therapies (rituximab, bortezomib) led to initial cardiac improvement in all treated patients, but 50% relapsed, and 20% died within 4 years.
Conclusions:
- Cardiac involvement in CG presents with varied cardiac abnormalities and requires consideration in CG patients with unexplained cardiac symptoms.
- B-cell targeted therapy is effective for initial management, but high relapse rates underscore the need for early and sustained intervention.
- Proactive management strategies are crucial to mitigate the significant morbidity and mortality associated with cardiac cryoglobulinemia.
Abstract:
Background: Cardiac involvement in cryoglobulinemia (CG) is rare but potentially fatal, and its clinical spectrum remains poorly characterized. Methods: This retrospective study enrolled 11 patients with cardiac involvement among 885 patients with CG at Peking Union Medical College Hospital between January 2015 and March 2026. We analyzed its clinical characteristics, radiological features and management. Results: Among 885 CG patients, 11 (1.2%; 4 type I, 7 type II) had cardiac involvement. Cardiac symptoms included dyspnea (n = 6), chest tightness (n = 4), edema (n = 3), and orthopnea (n = 1). All patients had elevated N-terminal pro-B-type natriuretic peptide (median 29,799 pg/mL). Echocardiography, performed in all 11 patients, revealed left heart enlargement (n = 9), reduced left ventricular ejection fraction (n = 7), myocardial disease (n = 6), pericardial effusion (n = 4), and pulmonary hypertension (n = 3). Cardiac magnetic resonance in 5 of 11 patients showed non-ischemic late gadolinium enhancement in two cases. For first-line therapy, 6 of 11 patients received rituximab-based regimens, 3 of 11 received bortezomib-based regimens, and 1 of 11 received antiviral therapy with corticosteroids; 1 patient declined treatment. All 10 treated patients achieved initial cardiac improvement, with 5 relapsing and 2 dying during a median follow-up of 57 months (range 9-130 months). The estimated 4-year overall and progression-free survival rates were 77.9% (95% CI: 0.546-1.000) and 50.0% (95% CI: 0.269-0.929), respectively. Conclusions: Cardiac involvement in CG is rare and associated with diverse structural and functional abnormalities. Cardiac involvement should be considered in CG patients presenting with unexplained cardiac manifestations after excluding alternative causes. B-cell-targeted therapy induced an initial response, but relapse is common. Early intervention is essential given the substantial relapse burden and potential for severe morbidity.
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An X-ray, or radiograph, is a non-invasive method that uses ionizing radiation to take images of internal structures. It is mainly used in cardiac imaging to examine the heart, lungs, and major blood vessels, aiming to identify abnormalities in the heart's size, shape, and position, such as heart failure, congenital defects, and vascular...