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- PATIENTS AND METHODS
The primary systemic vasculitides (PSVs) are a group of rare, life-threatening, chronic illnesses (1). They are characterized by inflammation of blood vessels, which can involve and cause damage to a many organ systems, including lungs, kidneys, and skin. This inflammation is accompanied by severe and painful symptoms (2–5). The medical treatment of the disease usually comprises high-dose steroids and cyclosphosphamide for acute disease episodes, followed by prolonged immunosuppression (e.g., azathiopine, methotrexate), which in itself causes additional symptoms.
It is likely that such a serious illness significantly impairs a patient's quality of life (QOL), causes psychological distress, and forces changes in the patient's and family's way of life (6). However, the introduction of patient-reported QOL measures to vasculitis research has only just started (7) because it is only recently that progress in medical management has turned these previously fatal illnesses to chronic ones. As a result, there is very little published research assessing the impact of the disease on QOL and psychological adjustment cross-sectionally or longitudinally. Comparative studies with other systemic diseases are scarce and limited in scope (8, 9), as are comparisons between the specific vasculitides. However, there are now studies in progress evaluating QOL and, to some degree, psychological adjustment in these conditions.
Existing research in vasculitis has focused on evaluating QOL using mainly generic instruments, such as the Short Form 36 (SF-36). Hoffman et al's (10) study is to our knowledge the only relevant study published as a full article where a disease-specific questionnaire was devised with input from a patient focus group. The questionnaire assessed the effects of Wegener's granulomatosis (WG) on the health, function, and income of 60 patients. The findings showed that patients experienced substantial medical and functional morbidity, with 78% requiring long-term immunosuppressive treatment. Daily activities were significantly reduced or constrained in 80% of these patients. There was a negative impact of the disease on patients' normal daily living, employment circumstances, and income and a variable impact on family and close relationships.
Boomsma et al (11) translated this questionnaire to Dutch and assessed the impact of WG on 79 Dutch patients. They replicated Hoffman et al's (10) main findings: WG was associated with increased medical morbidity and adverse effects on income and physical functioning. However, they reported lower impact of the disease on interpersonal relationships and attributed this finding to cultural and socioeconomic differences between The Netherlands and the US.
A recent review on the socioeconomic impact of vasculitis suggests that its adverse negative effect is greater than anticipated and recommends a full investigation (7).
The information from the following studies has been extracted from work presented in conferences and published as abstracts, which allows limited scope for critical appraisal of design and methods. Nevertheless, there are consistent patterns in the reported findings, which are relevant to the background and aims of this study. Exley et al (12) used the SF-36 to assess QOL in patients (n = 30) with active and inactive vasculitis and found impaired levels of QOL. Herlyn et al (13) also used the SF-36 to compare a group of patients with active PSV (n = 303) with a group of patients in complete remission (n = 40) and a healthy population (n = 350), assessing patients at baseline and at 12 months followup. They found limited QOL for both patient groups at both assessments. Raza et al (14) investigated the relationship between vasculitis activity and damage by combining scores in the separate SF-36 subscales into scores for physical and mental components in 83 patients with systemic necrotizing vasculitis. Their patient group reported significantly lower SF-36 scores than a healthy population. Interestingly, there were no significant correlations between disease activity or damage scores and physical or mental components of the SF-36. Grove et al (15) investigated the relationship between SF-36 physical and mental functioning scores and disease activity and damage scores in 91 PSV patients. They reported that overall physical functioning was significantly impaired (no comparisons with norms or reference to cut-off points were reported in the abstract). Mental functioning was significantly impaired for patients with active disease when compared with patients with inactive disease, and physical functioning only was significantly lower for patients with high damage.
Currently there is no vasculitis-specific measure of physical disability. The Health Assessment Questionnaire (HAQ) has been used as a disability measure in 2 published studies (8, 9) that included vasculitis patients in their assessment of physical functioning in patients with different rheumatic diseases.
In this study, we addressed generic and specific aspects of QOL and psychological adjustment in a group of UK vasculitis patients. Detailed evidence from a UK sample allows assessment of 1) impact of vasculitis on QOL in a UK context and 2) generalizability of previous findings. It also enables the identification of key areas of disease impact, as indicated by the patients themselves, that could benefit from future tailored medical or psychoeducational interventions.
We conducted an exploratory survey of vasculitis patients from a prospective regional UK register aiming specifically to 1) evaluate the impact of the disease on PSV patients' QOL using the SF-36, assessing also neuropathic and steroid treatment status, disability levels, pain, fatigue, and mood using the Hospital Anxiety and Depression Scale (HADS) (16); 2) compare levels of QOL between the different PSV diagnostic groups; 3) assess internal consistency and concurrent and discriminant validity of the HAQ as a measure of disability in PSV; and 4) evaluate the relationship between the clinical marker of permanent disease damage (modified Birmingham Vasculitis Activity Score [BVDI]) with patients' self-reported scores on the above measures.
- Top of page
- PATIENTS AND METHODS
In this study we evaluated the impact of PSV on patients' QOL, psychological adjustment, and physical functioning. The results of the assessment of QOL using the SF-36 showed that this vasculitis group demonstrated impaired levels on all aspects of QOL, except for mental health, when compared with normative data. In addition, this group had lower QOL scores than a German vasculitis group (13). These results support previous findings in PSV when the SF-36 was used to assess QOL from work currently published as abstracts (e.g., 12–15); and they support findings from 2 published studies that used a vasculitis-specific assessment of QOL (10, 11). The SF-36 results from this study showed that patients reported significantly impaired levels of physical and social functioning, energy and vitality, and role limitation due to physical and emotional problems; they also reported increased levels of pain and lower perceptions of general health when compared with the norms. Given the severity of the illness itself, the unpredictability of its course, and the significant side effects of its treatment, the observed low levels of QOL correspond well with the clinical features of the illness and clinical experience.
However, no statistically significant differences were observed in this study between the vasculitis group and normative data for the SF-36 mental health subscale. Using the HADS, however, we found that 25.5% of the whole vasculitis sample reported high depressive symptoms and 43.2% reported high anxiety symptoms to a variable degree. Prevalence levels obtained with the HADS in a healthy population (22) were 5% for depression (cut off 8), and 7% for anxiety (cut off 10). In a study investigating mood levels in cancer patients (31) using the HADS, 8.7% of patients scored within the range for possible clinical disorder for depression, and 27% did so for anxiety. In this study, we observed 5.1 times the depressive symptoms and 6.17 times the anxiety symptoms reported in the healthy population (22), and obtained 2.93 times the incidence of depressive symptoms and 1.6 times the incidence of anxiety symptoms when compared with the cancer patients' mood levels (31). Although the generalizability of these results needs to be assessed, they indicate that psychological distress is high in this disease group, and this is likely to be a major issue for many patients. It is reasonable to suggest that psychological distress should be assessed and addressed as part of the disease management efforts. Our findings also indicate that there might be a discrepancy in levels of psychological distress obtained by the SF-36 mental health subscale and the HADS. This requires further investigation to determine the most psychometrically sound way to evaluate distress in this population.
As part of the aims of this study, we also investigated differences in QOL and psychological adjustment between the 3 different diagnostic subgroups of PSV. Despite the small power for the MPA and CSS groups, statistically significant differences were observed for some of the outcome variables, providing some preliminary information on areas of differentiation between the diagnoses that could guide future research.
Patients with moderate to severe pain showed significantly impaired scores in all aspects of QOL as assessed by the SF-36, except for mental health, although they still scored lower than the little or no pain group in this subscale. Furthermore, patients with pain reported significantly higher depression scores and higher anxiety scores (using the HADS), although not significantly so. They also scored significantly higher on symptom severity over the past month, levels of fatigue, and problems with sleep. These findings suggest that pain, whatever its cause, is a major determinant of QOL in a similar way in PSV as in most other diseases. It is reasonable to suggest that appropriate and adequate pain management should be a high priority of clinicians treating PSV patients, given its significant association with impaired levels of QOL in a range of dimensions. Patients with neuropathic symptoms and patients on higher levels of steroid treatment had significantly impaired scores on some, but not as many, of the relevant measures. Presence of neuropathy was associated with impaired scores on SF-36 physical functioning, disability (HAQ), and symptom severity; treatment with higher steroid dose was associated with significantly higher levels of pain, depression (HADS), and impaired levels of SF-36 scores for social function and energy and vitality.
In addition to the disability subscale of the SF-36, we used the HAQ to assess physical functioning and evaluated its psychometric properties when used in PSV. Overall, there was evidence of high internal consistency and concurrent and discriminant validity of the HAQ as a measure of functional disability in PSV. Future research should assess its reliability.
The HAQ scores were significantly correlated with patient-reported illness symptoms (pain today and pain this week, fatigue, sleep, and symptom severity) and with the SF-36 subscale scores (physical functioning, social functioning, energy and vitality, pain, mental health, and general health perception), suggesting that these were the areas of difficulty for patients experiencing adverse effects on their physical functioning. The exception was SF-36 scores for impact on role due to emotional problems, where no significant correlation with the HAQ was observed.
However, the scores derived from the modified BVDI clinical measure were weakly and not significantly correlated with patient reports of illness symptoms or with QOL scores. Studies of QOL in other chronic rheumatic illnesses have shown that patients' mood is significantly related to the way they perceive their illness and not to markers of disease activity or damage (32, 33). It seems that self-report measures provide better information for the experience of living with a systemic illness than disease markers, and simple VAS illness-related measures (e.g., for pain, sleep, fatigue) could easily be incorporated in clinical assessment to capture this.
Pincus et al (9) described a modified version of the HAQ (the Multidimensional Health Assessment Questionnaire [MDHAQ]) and suggested it may be suitable for use with a range of rheumatic diseases in addition to RA. Their findings using the MDHAQ, and the results of this study, render some support for this. Future research should assess validity and reliability of different methods of assessing disability and psychological distress in PSV.
On the basis of the preliminary findings from this study and previous cross-sectional research that needs to be published in full, it seems that many aspects of QOL are significantly impaired in PSV. Additional longitudinal research is essential to establish causal pathways and assess the impact of the disease over time. Future psychological work could 1) investigate how patients and their caretakers think and cope with their illness, and how these affect their QOL; and 2) design, implement, and evaluate psychoeducational interventions that aim to address their psychosocial needs. Although a clinical marker of disease damage serves as an indicator of physical status of vasculitis, it is not necessarily related to measures of QOL or physical and emotional functioning. Self-reported pain and disability, presence of neuropathy, treatment with steroids, depression and anxiety scores, scores on the SF-36, and self-report measures of disease symptoms are significant indicators of the impact of vasculitis on the patient's life and need to be assessed and addressed in overall patient management.