Background/Purpose: Despite improved therapeutic strategies, patients with systemic vasculitis (SV) continue to experience serious morbidity and mortality from persistent low grade disease activity and permanent damage, particularly cardiovascular diseases. Nonetheless, the outcome of cerebrovascular disease (CVD) in patients with SV during the first- ever stroke remains unclear when compared to patient with other causes for their first-ever stroke. Purpose: To examine the clinical outcome related to CVD in SV, and determine whether disability and mortality are related to the disease itself, or associated comorbidities.

Methods: Disability and mortality were examined in 24 SV patients (large vessel vasculitis n = 8, medium vessel vasculitis n = 10, and small vessel vasculitis n= 6; with mean age of 55 .7 years, 55 % men) admitted to a tertiary care stroke center with validated first-ever stroke between January 2000 and January 2012. Disability rated according with modified Rankin Scale (mRS> 3), and mortality defined as all-cause fatal event were measured the day of first-ever stroke and at 90 days of follow up. Demographics and clinical manifestations were compared between those with SV and age- and gender –matched non SV controls with their first-ever stroke (n= 24) from the same tertiary stroke center.  Significant variables in these analyses and the National Institute of Health Stroke Severity Scale (NIHSS) were entered into the multivariate analyses and Cox proportional hazards analyses to determine their contribution to disability and mortality related to CVD.

Results:

Patients from the SV and non SV groups had comparable ethnic distribution, socioeconomic features, smoking, and their stroke type (ischemic, hemorrhagic) and Charlson comorbidity scores were similar. Disability was more frequent among SV patients as compared to controls (odds ratio= 4.2, 95 % Confidence interval: 1.2- 14.4, p value =0.04). Within the SV patients 18 were disabled (mRS > 3), and 6 were not. Intractable disease activity and cumulative damage involving the pulmonary and renal organs were higher in disabled than the non disabled SV patients, although the difference was not statistically significant. Four patients died among SV group as compared to one patient among the controls. Death due to infection was more frequent among SV patients compared to controls (odds ratio= 2.0; 95 % CI: 0.2- 21.4, p value = 0.50).  

Table 1.  Proportional Cox hazard analyses of factors associated with disability related to first-ever CVD in patients with SV

                                                            Proportional Hazard Model

Factors                                                           Hazard Ratio (95 % Confidence interval)

BVAS2                                                             7.2 (1.1 – 47.9)                                

VDI                                                                  10.0 (1.4 – 69.3)                                  

ANCA PR 3                                                     0.7 (0.03 – 12.57)                             

ANCA MPO                                                     5.0 (0.5 – 51.7)                                  

C Reactive Protein                                       1.6 (0.4- 11.1)                              

Long -term Prednisone use                       8.5 (1.3 – 54.9)

_____________________________________________________________                          

Brimingham Vasculitis Activity Score (BVAS2), Vasculitis Damage Index (VDI)

Conclusion: SV patients are at an increased risk for disability and mortality after the first –ever CVD event. Intractable disease activity, cumulative damage and long-term prednisone use are independently associated with disability related to CVD after adjustment for stroke severity. Death due to infection is frequent.

---

« Back to 2012 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/disability-and-mortality-related-to-cerebrovascular-disease-in-systemic-vasculitis/