Takayasu's Arteritis from the Patients' Perspectives: Measuring the Pulse to the Patient-Reported Outcomes.

BACKGROUND: Patients with Takayasu's arteritis (TA) experience important changes in lifestyle, quality of life, and functional status due to ischemic symptoms or treatment toxicity. PURPOSE: To describe the clinical characteristics and the patient-reported outcomes (PROs), such as quality of life, disability, fatigue, and perception/impact of the disease in Mexican patients with TA. METHODS: Cross-sectional study including patients with established diagnosis of TA recruited at a tertiary care center. Demographics, comorbidities, clinical characteristics, laboratory, imaging, and treatment were retrieved. Disease activity (the Indian Takayasu Clinical Activity Score (ITAS) 2010), damage (Vasculitis Damage Index (VDI)), quality of life (Short Form 36 (SF-36)), disability (Health Assessment Questionnaire Disability Index (HAQ-DI)), fatigue (Multidimensional Fatigue Inventory-20), and patient's disease perceptions were assessed. RESULTS: Fifteen women were included, with a median age of 41 years (interquartile range (IQR) 30-45) and disease duration of 108 months (IQR 55-197). Median ITAS 2010 and VDI scores were 0 (IQR 0-2) and 3 points (IQR 2-6), respectively. Mean SF-36 score was 71.38 ± 13.39, with mean physical and mental component summaries of 66.52 ± 13.37 and 76.24 ± 14.89, respectively. HAQ-DI mean score was 0.48 ± 0.62, being grip the most affected domain. Among fatigue subscales, the higher scores were present in the physical fatigue (16.3 ± 5.8). Correlations between the HAQ-DI and the VDI score (r = 0.64, P = 0.03); between the general fatigue, score, and disease duration (r = -0.71, P = 0.01); and between the SF-36 total score and the HAQ-DI (r = -0.87, P = 0.0004) were found. CONCLUSIONS: It is important to identify disease-specific outcomes of interest to the patients to develop tools that assess them with a holistic approach.

Health-related outcomes of importance to patients with Takayasu's arteritis.

OBJECTIVES: The need to include patients' perspectives as key outcomes in clinical trials is widely accepted. No disease-specific patient-reported outcomes have been developed in Takayasu's arteritis. This project was designed to identify outcomes of importance to patients with Takayasu's arteritis during active disease and remission across 2 different cultures. METHODS: Patients with Takayasu's arteritis from the US and Turkey were recruited to participate in semi-structured, one-on-one interviews or focus groups. The interviews and group sessions were recorded, transcribed, and entered into an Nvivo database. A line-by-line review of narrative data was used to develop themes describing the impact of Takayasu's arteritis on patients' life. US Patients were invited to freelist terms that they associated with disease states (active disease and remission). The Smith's Salience Index (SSI) was used to identify the most salient terms. RESULTS: Results. A total of 31 patients with Takayasu's arteritis participated in this study. Interviews and focus groups identified pain, fatigue, and emotional impact as common themes. Outcomes did not differ between the 2 countries. The most salient terms identified through freelisting were pain/discomfort and fatigue/low energy levels (SSI=0.56 and 0.33, respectively) during active disease and pain/discomfort and emotional impact (SSI=0.51 and 0.37, respectively) during remission. CONCLUSIONS: Patients with Takayasu's arteritis report a range of disease-specific symptoms across different cultures and disease states that are generally not specifically captured by generic patient-reported outcome tools currently used in research. Identifying disease-specific outcomes would advance clinical trials methodology to best capture the full spectrum of disease activity in Takayasu's arteritis.

Development of a Core Set of Outcome Measures for Large-vessel Vasculitis: Report from OMERACT 2016.

OBJECTIVE: Among the challenges in conducting clinical trials in large-vessel vasculitis (LVV), including both giant cell arteritis (GCA) and Takayasu arteritis (TA), is the lack of standardized and meaningful outcome measures. The Outcome Measures in Rheumatology (OMERACT) Vasculitis Working Group initiated an international effort to develop and validate data-driven outcome tools for clinical investigation in LVV. METHODS: An international Delphi exercise was completed to gather opinions from clinical experts on LVV-related domains considered important to measure in trials. Patient interviews and focus groups were completed to identify outcomes of importance to patients. The results of these activities were presented and discussed in a "Virtual Special Interest Group" using telephone- and Internet-based conferences, discussions through electronic mail, and an in-person session at the 2016 OMERACT meeting. A preliminary core set of domains common for all forms of LVV with disease-specific elements was proposed. RESULTS: The majority of experts agree with using common outcome measures for GCA and TA, with the option of supplementation with disease-specific items. Following interviews and focus groups, pain, fatigue, and emotional effect emerged as health-related quality of life domains important to patients. Current disease assessment tools, including the Birmingham Vasculitis Activity Score, were found to be inadequate to assess disease activity in GCA and standardized assessment of imaging tests were felt crucial to study LVV, especially TA. CONCLUSION: Initial data from a clinician Delphi exercise and structured patient interviews have provided themes toward an OMERACT-endorsed core set of domains and outcome measures.

Remarkable damage along with poor quality of life in Takayasu arteritis: cross-sectional results of a long-term followed-up multicentre cohort.

OBJECTIVES: We aimed to assess the outcome of a large Takayasu arteritis (TAK) cohort using the vasculitis damage index (VDI) and quality of life (QoL) scale, tools which have been validated for vasculitis. METHODS: Disease activity, damage and QoL were cross-sectionally evaluated in 165 TAK patients from 6 centres. SF-36 were applied to 51 age-matched healthy controls (HC). Persistent activity for ≥6 months was considered as treatment resistance (r-TAK). The correlation between VDI, clinical characteristics and mental (MCS)/physical (PCS) component scores of SF-36 were analysed. SF-36 and VDI scores were compared between TAK subgroups and HC. RESULTS: The median age, follow-up time and disease duration were 40 (17-68), 60 (6-384), and 72 (6-396) months, respectively. 35% of them were r-TAK. VDI scores (VDIs) in TAK 4 (1-12) were mainly due to the disease itself [4 (1-10)]. VDIs in r-TAK were significantly higher than nr-TAK [5 (2-12) vs. 3 (2-10), p<0.001)]. In the TAK patients, MCS and PCS were found as 43±10 and 38±11, respectively. A high proportion of poor MCS (70%) and PCS (80%) were demonstrated in TAK. A significantly negative but weak correlation was observed between VDI and MCS (p=0.003, r=-0.23), PCS (p<0.001, r=-0.34). Higher VDIs were detected in patients with PCS <50 [5 (1-12) vs. 2 (1-6) p<0.001)]. SF-36 score was significantly lower in TAK than HC. CONCLUSIONS: Disease-related damage mainly caused by peripheral vascular involvement was more predominant than treatment-related damage without reaching the level of severe damage scores, but contributing to poor QoL, in the TAK cohort.

Relapse of Takayasu arteritis as a cause of suicidal poisoning and subsequent major ischemic stroke successfully treated with thrombolytic therapy.

Introduction: Takayasu arteritis (TA) is a rare large vessel arteritis, affecting primarily aorta and its major branches. Its clinical manifestations can vary significantly - from asymptomatic to serious vascular events. Acute neurological complications are frequent at the onset of the disease and in relapses. Anxiety and depression are more frequent in TA patients than in general population as well as during relapses. Prevalence of transient ischemic attack or ischemic stroke in TA patients is approximately 10-20%. Case report: We presented a patient with TA that began with a depressive episode resulting in attempted suicide by bromazepame poisoning. This was subsequently followed by major ischemic stroke caused by thrombosis of the left middle cerebral artery (probably due to aortic arch embolism) successfully treated with intravenous thrombolysis. Conclusion: Intravenous thrombolysis appears to be safe and effective in patients with TA and stroke.

Impaired quality of life, disability and mental health in Takayasu's arteritis.

OBJECTIVE: Patient-reported outcomes (PROs) are increasingly accepted to be among the major tools for outcome assessment in rheumatic disorders. In this study we aimed to assess quality of life (QoL), disability, anxiety and depression in patients with Takayasu's arteritis (TAK). METHODS: Patients followed with the diagnosis of TAK (n = 165) and healthy controls (HCs) (n = 109) were enrolled to the study. The 36-item Short Form Health Survey (SF-36) and hospital anxiety and depression scales (HADS) were used to assess QoL and mental status together with HAQ for disability. RESULTS: In SF-36 subscale assessment, all items were observed to be statistically lower in TAK patients; similarly HAQ scores were also higher (P < 0.001) in this group. In mental assessment, anxiety was found to be more common in TAK patients [90 (54.5%) vs 38 (34.9%), P = 0.001]. Depression also tended to be higher in TAK patients [70 (66.7%) vs 35 (33.3%)], without reaching significance (P = 0.086). Most of the SF-36 subgroup parameters were lower in TAK patients with active disease. Patients having anxiety and depression or with high HAQ scores reported worse SF-36 scores. In multivariate analysis, HADS-A, HADS-D and HAQ were associated with most SF-36 subscales. CONCLUSION: PROs demonstrate that not only general health but also physical and social functioning with physical role limitations and mental health parameters were impaired in TAK. Our results, especially in active disease, suggest that PROs such as SF-36 can be core domains of disease assessment in TAK, similar to ANCA-associated vasculitides.

Long-term efficacy and improvement of health-related quality of life in patients with Takayasu's arteritis treated with infliximab.

OBJECTIVES: To explore the efficacy in the long-term and the impact on Health Related Quality of Life (HRQOL) of infliximab in patients suffering from Takayasu's arteritis (TA). METHODS: Clinical data were retrospectively collected in 15 patients with TA. Evaluation of Medical Outcomes Study Short Form 36 (SF-36) questionnaires was made at baseline and at the last follow-up in 10 patients continuing infliximab at the last follow-up. RESULTS: Follow-up after initiation of infliximab was 71±44 months (range 10-162). Remission at the last follow-up was noted in 11/15 (73.3%). Significant reduction in BVAS score was noted at the last follow-up [from 4.0 (1-16) to 3.0 (0-9), p=0.003]. Significant steroid dose reduction was recorded [from 10 mg/day (0-50) to 2.5 mg/day (0-15), p=0.005)]. Steroid suspension occurred in 5/11 responder patients. Inflammatory markers were normalised in about two thirds of the patients. Radiological disease activity was assessed in 13/15 during infliximab therapy, with evidence of improvement in 2/13, stable disease activity in 9/13, and worsening in 2/13. No relevant side effects or severe infections were recorded during the whole follow-up under infliximab. One patient stopped infliximab at the third infusion for acute reaction. HRQOL in patients with TA was impaired, with major involvement of physical domains [(body pain (BP) and global health (GH)]. Infliximab significantly improved HRQOL, in particular BP (40.0±32.3 vs. 67.2±27.6, p=0.035), GH (31.2±21.5 vs. 54.9±21.1, p=0.007) and Vitality (VT) (47.0±28.7 vs. 67.0±20.3, p=0.01) domains. CONCLUSIONS: Infliximab determined a sustained clinical improvement in the long-term in TA, with significant benefits on HRQOL.

Quality of life of patients with Takayasu's arteritis.

OBJECTIVE: Takayasu's arteritis (TA) is a chronic immune vasculitis that causes inflammation of the aorta and its branches and is clinically characterized by exacerbations and remissions. This study examined the quality of life (QoL) of patients with TA using the Medical Outcomes Study Short Form 36 (SF-36) Health Survey, a validated health related QoL questionnaire. METHODS: Questionnaires that included the SF-36 and demographic related variables were mailed to 392 patients enrolled in the Takayasu's Arteritis Research Association. Raw SF-36 scores, as well as Physical Health Summary (PHS) and Mental Health Summary (MHS) scores, were calculated according to standard protocols. Data were analyzed for predictors of superior QoL using univariate and stepwise logistic regression analysis. SF-36 scores were also compared with those of other chronic diseases associated with peripheral vascular disease (PVD) published in the literature. Results are reported as mean +/- standard error of the mean. RESULTS: A total of 158 patients (144 women, 14 men) with average age of 42.2 +/- 1.1 years responded to the questionnaire. Mean onset of symptoms occurred at 30.5 +/- 1.2 years, with a mean age at diagnosis of 34.7 +/- 1.2, and a median of four doctors were seen before diagnosis. The group underwent 299 TA-related surgical procedures (1.9 +/- 0.3), including coronary (38%), carotid (35%), upper extremity (30%), and lower extremity (26%) revascularization. PHS and MHS summary scores (39.2 +/- 1.0 and 44.5 +/- 1.0, respectively) were worse than mean scores for an age-matched healthy population as well as nationally reported scores for diabetes mellitus, hypertension, and coronary artery disease (all P < .0001). Multivariate predictors of better physical QoL were younger age (P = .003) and remission of the disease (P = .0002). The use of immunomodulating medications was associated with inferior physical QoL (P = .02). The sole predictor of better mental QoL was remission of disease (P = .002). CONCLUSION: TA is a rare disease with profound consequences on QoL. Scores for physical and mental health are worse compared with many other chronic diseases associated with PVD. Superior physical QoL is seen in younger patients, whereas inferior physical QoL is encountered in those who take immunomodulating medications. Because the only factor to influence positively both physical and mental QoL is disease remission, every effort should be directed to attenuate disease activity.

Quality of life in patients with Takayasu's arteritis is impaired and comparable with rheumatoid arthritis and ankylosing spondylitis patients.

The aims of the study were to assess the health-related quality of life (QOL) in patients with Takayasu's arteritis (TA) by two different generic QOL instruments and to compare the results with those patients with rheumatoid arthritis (RA), ankylosing spondylitis (AS), and healthy controls (HC). A cross-sectional study was performed in 51 patients with TA (41 women; mean age 38.4 +/- 13.5), 43 RA (36 women; 55.2 +/- 9.6), 31 AS (12 women; 41.2 +/- 13.1), and 75 HC (53 women; 38.8 +/- 10.9). Quality of life was assessed by using Short-Form 36 (SF-36) and Nottingham Health Profile (NHP). Separate dimensions of SF-36 and NHP and physical and mental summary scores of SF-36 as well were compared between patients and control groups. Physical and mental health summary scores and all SF-36 subscales, except for social functioning, were significantly lower in patients with TA than healthy controls. No significant differences between TA, RA, and AS patients were found in all SF-36 subscales and summary scores. NHP scores for energy level, pain, emotional reactions, and physical mobility were significantly higher in TA patients than controls. All NHP subscales, except for pain, were comparable in patients with TA, RA, and AS. Pain score was worse in RA patients. The NHP scores for sleep and social isolation were not different between patients and controls. Many aspects of QOL in patients with TA are significantly impaired in comparison with local healthy controls and similar to those in patients with RA and AS.