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Cardiac involvement in the catastrophic antiphospholipid syndrome (CAPS): Lessons from the "CAPS registry"
Isaac Pons1, Alba Jeréz2, Gerard Espinosa3
1Department of Autoimmune Diseases, Reference Centre for Systemic Autoimmune Diseases (UEC/CSUR) of the Catalan and Spanish Health Systems-Member of ERNReCONNET, Hospital Clínic, Universitat de Barcelona, Barcelona, Catalonia, Spain; Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Catalonia, Spain; Department of Internal Medicine, Althaia Xarxa Assistencial Universitària de Manresa, Facultat de Medicina, Universitat de Vic-Universitat Central de Catalunya (uVic-UCC), Manresa, Catalonia, Spain.
Insights
Catastrophic antiphospholipid syndrome (CAPS) frequently involves the heart, often alongside kidney issues. This cardiac involvement, characterized by microthrombosis, does not increase mortality rates in CAPS patients.
Area of Science:
- Rheumatology
- Cardiology
- Internal Medicine
Background:
- Catastrophic Antiphospholipid Syndrome (CAPS) is a rare, severe autoimmune disease characterized by widespread thrombosis.
- Cardiac involvement is a serious complication of CAPS, but its prevalence and associated factors require further elucidation.
Purpose of the Study:
- To analyze the demographic, clinical, and laboratory characteristics of CAPS patients with cardiac involvement.
- To identify factors independently associated with cardiac involvement in CAPS.
Main Methods:
- Analysis of the "CAPS Registry" database.
- Inclusion of 749 patients with 778 CAPS events.
- Univariate and multivariate statistical analyses were performed.
Main Results:
- Over half (52%) of CAPS patients experienced cardiac involvement, with heart failure, valve disease, and myocardial infarction being most common.
- Cardiac involvement was significantly associated with increased frequency of stroke, pulmonary infarction, renal infarction, acute kidney injury, and livedo reticularis.
- Acute kidney injury was the sole independent predictor of cardiac involvement (OR 1.068, p < 0.001).
- Cardiac thrombotic microangiopathy was prevalent in autopsy/biopsy samples (83%).
Conclusions:
- Cardiac involvement is a frequent manifestation of CAPS, often linked with kidney involvement.
- Cardiac microthrombosis is a key pathological finding in affected patients.
- Importantly, cardiac involvement in CAPS is not associated with increased mortality.
Objective:
To analyze the demographic, clinical, and laboratory characteristics of catastrophic antiphospholipid syndrome (CAPS) patients with cardiac involvement, and to identify the factors associated with this cardiac involvement.
Material And Methods:
Based on the analysis of the "CAPS Registry", the demographic, clinical, and serological characteristics of patients with cardiac involvement were analyzed. Cardiac involvement was defined as heart failure, valvular disease, acute myocardial infarction, pericardial effusion, pulmonary arterial hypertension, systolic dysfunction, intracardiac thrombosis, and microvascular disease. Univariate and multivariate analysis was used for multiple comparisons.
Results:
749 patients (293 [39 %] women and mean age 38.1 ± 16.2 years) accounting for 778 CAPS events were included, of them 404 (52 %) had cardiac involvement. The main cardiac manifestations were heart failure in 185/377 (55 %), valve disease in 116/377 (31 %), and acute myocardial infarction in 104/378 (28 %). Of 58 patients with autopsy/biopsy, 48 (83 %) had cardiac thrombotic microangiopathy, Stroke (29% vs. 21 %, p = 0.012), transient cerebral vascular accident (2% vs. 1 %, p = 0.005), pulmonary infarction (26% vs. 3 %, p = 0.017), renal infarction (46% vs. 35 %, p = 0.006), acute kidney injury (70% vs. 53 %, p < 0.001), and livedo reticularis (24% vs. 17 %, p = 0.016) were significantly more frequent during CAPS events with versus without heart involvement. Multivariate analysis identified acute kidney injury (OR 1.068, IC 95 % 1.8-4.8, p < 0.001) as the only clinical characteristics that were, independently, associated with cardiac involvement in CAPS events. Cardiac involvement was not related to higher mortality.
Conclusions:
Cardiac involvement is frequent in CAPS, with association with kidney involvement, and it is not related to higher mortality. The presence of cardiac microthrombosis was demonstrated in most biopsies/autopsies performed.
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