School of Health Policy and Practice, University of East Anglia, Norwich NR4 4TJ, UK.
Primary systemic vasculitides are a group of rare chronic illnesses which are life-threatening. They are characterized by inflammation of blood vessels and can affect and damage to a variable degree many organ systems, including the lungs, kidneys and skin. The disease usually manifests itself with an initial acute, severe episode that requires aggressive medical management (e.g. high-dose steroids and cyclophosphamide). This is followed by a chronic course, often requiring prolonged immunosuppression (e.g. azathioprine, methotrexate, etc) and there is a significant risk of relapse. Watts et al. [1] report that the overall annual incidence of systemic vasculitis in adults is 19.8/million with peak incidence in the 6574-yr age group. Some studies suggest the incidence may be increasing with time (for a review see Watts et al. [2]). The diseases can affect all age groups and they are slightly more common in men (male:female ratio 1.31.5:1 [2, 3]). Symptoms include pain, myalgias, ear, nose and throat symptoms, skin irritation, haemoptysis, fatigue, motor weakness, and sensory and motor loss [47]. These symptoms can impair the psychological wellbeing and quality of life (QOL) of patients and their carers, and there are also additional symptoms to cope with which are caused by medical treatment of the disease. Disease symptoms and treatment side-effects are evident during the episodes of active disease and they tend to subside when patients are in a remission phase and not under active treatment. In remission, patients can be completely symptom-free, although there may be persisting symptoms or a cumulative effect of residual symptoms, as the disease can cause progressively more permanent damage [8].
It is very likely that such a severe illness causes psychological distress and forces changes in patients' and their families way of life. There is currently very little published research assessing the impact of the disease on psychological adjustment or evaluating psychological adaptation to the disease over time. However, there are a number of studies in progress evaluating the QOL and, to some degree, psychological adjustment in these patients. Existing knowledge on the effects of other chronic illnesses on patients' psychological adjustment and quality of life and the impact of psychological factors in successful adaptation to chronic illness will be discussed and related to vasculitis. Findings from research in vasculitis which have been announced in scientific meetings and published as papers [9] or abstracts will be discussed.
Rolland [10] has proposed a system of categorizing the psychosocial demands that any chronic illness makes on patients and their families on the basis of the following disease characteristics: type of disease onset (acute, gradual); expected disease course (progressive, constant, relapsing/episodic); outcome (fatal, shortened lifespan, non-fatal); and associated disability (none, mild, moderate, severe). As discussed earlier, vasculitis is characterized by acute onset for most patients and the expected course of the illness is unpredictable, but is usually associated with a relapsing/episodic nature. Over recent years, due to improved medical management of the condition, the outcome has changed from fatal to reduced life expectancy. The level of incapacitation or disability depends on the disease activity status; it can be severe, affecting both physical and social functioning. During a disease episode, physical activity, movement, and energy levels are impaired and patients suffer from general malaise caused both by the disease itself and treatment side-effects. Patients can become dependent on others, and impairment of social, work and family role functioning is unavoidable at this stage, since management often involves admission to hospital.
The patient, the family and/or caregivers need to be able to cope with the crisis and the anxiety associated with the unpredictability of the outcome. The extra caregiving demands often require the reorganization of family roles and may have significant financial implications (for a review of the socioeconomic impact of vasculitis, see Cotch [11]). Many patients in remission have to cope with treatment regimes for the illness itself, and in some cases with treatment for associated conditions resulting from permanent damage to specific systems (e.g. haemodialysis for kidney damage). Given these disease characteristics, Rolland's model [10] would predict that, in coping with vasculitis, families would need to be adaptive and flexible to move from a position of providing intense care-giving during episodes of disease activity to that of less care-giving at periods of remission, during which there can be a role change with less severe effects on the patient's normal function. Coping with uncertainty and associated anxiety over the future is an omnipresent feature of the disease for both patients and their families and, in combination with the flexibility required in family organization, could prove to be the most challenging aspect from a psychological perspective. The rarity of the disease and particular anxiety for patients and carers over knowledge about appropriate medical treatment outside centres of expertise, and the lack of public awareness of the disease, as revealed by patients themselves when interviewed about the impact of the disease, can impose additional restrictions on the QOL of vasculitis patients.
In this context, the impact of vasculitis on social relationships (familial and broader) for patients and family members needs to be better understood in order for us to develop effective management strategies. The function of social support in referring to processes by which interpersonal relationships promote psychological wellbeing and protect people from declines in health, particularly when they are facing stressful life circumstances [12], has been studied in other chronic illnesses, including rheumatic diseases {see Revenson [13] for a comprehensive review of social support in rheumatoid arthritis (RA)}. Structural aspects of social support (e.g. size of social network, number of social relationships, marital status) can be reduced due to the nature of a chronic illness without necessarily affecting the quality and functioning of the existing relationships. Fitzpatrick et al. [14] found that RA patients reported lower availability of social interactions with friends and neighbours despite the fact that compared with a normative sample they had similar availability and satisfaction, with close emotional ties. Patients satisfaction with social relationships has a stronger association with psychological wellbeing than measures of availability [14,15], and patients reporting more social support are less depressed than patients with less support [1416]. Seeking social support has been shown to be associated with better functioning in patients suffering from chronic obstructive lung disease and psoriasis [17]. Evers et al. [18] found that decreases in functional status in newly diagnosed RA patients a year from diagnosis were predicted by an initially smaller social network. In a study investigating sources of social support in spouses of RA patients [19], it was found that spouses tended to seek social support from social ties outside the marriage, in contrast to the patients. Affleck et al. [20] reported that RA patients rated as helpful gestures of support (i) being given the opportunity to express feelings and concerns, (ii) being given encouragement, hope, optimism, and (iii) being given advice and information when desired. Conversely, social support can also have a negative impact if it does not fulfil the needs of the patient. Negative unwanted or unsolicited advice from friends and family [19] and critical remarks by the husband [21] have been, respectively, related to depression and poorer adjustment to illness. On the basis of the above findings, we can generate hypotheses that can be tested empirically regarding what gestures from family members, friends and health professionals would constitute beneficial social support for vasculitis patients at the different stages of the disease, and attempt to identify what type of support would be helpful for carers, and where such support could be obtained.
The patient confronted with the diagnosis of vasculitis is likely to exhibit psychological reactions similar to those observed in other chronic illnesses. Research has shown that shock usually accompanies the diagnosis of a chronic life-threatening illness, and denial can be observed in a number of ways: ignoring the potentially serious meaning of symptoms, leading to delays in seeking medical care; and/or failing to acknowledge the implications of the diagnosis; and/or failing to follow treatment regimes during the rehabilitation phase of the illness [22]. Denial as a psychological response at the early stages of an illness can be helpful in allowing time for the patient to take in the implications of the diagnosis. However, it has been associated with negative outcomes when used as a long-term strategy [23]. Anxiety, as discussed earlier, is also a typical psychological response to illness and relates to potential lifestyle changes and the prospect of death. Denial and anxiety can be experienced intermittently during the different stages of the illness. Depression is another common emotional reaction which is usually associated with chronicity, but it can also be observed in the acute phases of a chronic illness. It is associated with negative outcomes ranging from deriving more negative interpretations of the situation (catastrophizing), to refusal of medical treatment, which can result in death [24, 25]. Depression can remain untreated, as some of its presenting symptoms, such as fatigue and loss of libido, can be confounded with physical symptoms of the disease or treatment side-effects. In these cases, cognitive and emotional manifestations of depression should be assessed in detail, to identify whether further specific treatment for depression is necessary. Pain and disability, the severity of illness, the presence of other negative life-events, social stress and the absence of social support have been identified consistently in the literature as predictors of depression in the context of chronic illness [22].
Little research has been conducted into the possible positive psychological reactions to chronic illness. However, as a result of their illness, cancer and myocardial infarction patients have reported positive changes in empathy, change in personal life priorities, greater appreciation of health and life, improved close relationships and perceptions of having been given a second chance [26]. Future research with vasculitis patients and their families should aim to identify factors that determine positive mental health and successful adaptation to the demands of the illness according to the stage of the disease.
In chronic illness, QOL is typified by a wide spectrum of dimensions. In its broadest definition, QOL encompasses: physical health, psychological state, levels of independence, relationships, employment, relationship with the environment, and socio-economic factors [27]. However, many studies focus mainly on the impact of disease on physical functioning [28, 29]. In two reviews of psychological outcomes and QOL in rheumatic diseases, it is suggested that psychological factors are of particular importance as an element of QOL, mediating the judgements a patient makes on all aspects of their QOL and playing a role in maintaining it [30, 31]. In relation to vasculitis, it is important to evaluate the impact of the disease both on psychological functioning and QOL, and also to determine what would be positive adjustment to it, given the specific characteristics of the illness.
Carr et al. [32] note that the measures of QOL used with chronic illness populations which include functional capacity are based on the assumption that full functioning determines QOL. Applied to chronic illness, where there may be states of permanent damage to systems, as is the case in vasculitis, this assumption is questionable. They argue for the individual patient's evaluation of QOL, and suggest that measures should indicate the weighted importance of different dimensions to each individual patient. In the context of vasculitis, disease-specific QOL measure(s) need to be developed with items derived from the patient's own experience of the disease. Such a measure, if used in parallel with generic QOL measures currently in use, should provide more accurate information on the impact of the disease.
A number of psychological factors have been identified as relevant to the development of and/or adaptation to chronic illness, such as personality types and specific traits (optimism, hostility), health-related behaviour (smoking, diet, exercise), cognitive variables and coping strategies. Research has attempted to discover: (i) physiological mechanisms linking personality to illness, (ii) personality differences associated with differences in health-related behaviour, and (iii) predictors of disease progression, treatment outcome and QOL.
Studies have examined personality types (e.g. type A: highly competitive, achievement-oriented, hurried and fast, hostile, active; type B: relaxed, laid-back, even-tempered) and characteristics (hostility), and positive and negative prevailing moods (depression, optimism) in relation to the development and outcome of prevalent illnesses, such as cancer and coronary heart disease (CHD) [33]. Evidence from recent large-scale prospective studies does not support a strong significant relationship between personality types and cancer [34, 35]. There is no consistent evidence for a strong link between CHD mortality and type A/B behaviour either. Despite this, there is evidence for much stronger links between CHD and traditional risk factors, some of which are dependent on health behaviours such as smoking and diet (e.g. high cholesterol levels and to some extent high blood pressure) [36]. Other trait characteristics that have been shown to affect the experience and reaction to physical illness are psychological characteristics such as hardiness, internal locus of control and self-efficacy.
More emphasis in research is placed currently on health behaviours and cognitive variables, such as beliefs about the nature, cause and control of the illness, and cognitive and behavioural coping strategies. Evidence suggests that cognitive variables and coping strategies are relevant to adjustment to chronic illness, as they relate to health behaviours and emotional adjustment and have been shown to be amenable to interventions.
Patients understanding of their condition is an important determinant of engagement in appropriate management behaviours; slips in following a medical regime in the absence of acute disease symptoms can be the result of lack of clear understanding by the patient of the underlying disease process [22]. Also, patients beliefs about the cause of their illness, for example attributions to stress, injury, infection, God's will, other peoples behaviour [37] and whether patients attribute the blame to themselves, someone else or fate, are related to a variable degree to psychological adjustment [22]. Evidence suggests that attributing the blame to others is maladaptive, while no relationships have been found between attributions of the disease to luck or environment and adjustment [38]. Interesting findings have been reported regarding patients perceptions of control of the illness and their beliefs in their ability to influence the illness and its treatment (self-efficacy). Generally, patients with a chronic illness (e.g. cancer, RA, AIDS) who believe that they have control over the illness and its treatment adjust better to it than patients without such beliefs [3840], even when the illness is progressive. Kaplan et al. [41] found an association of such beliefs with longer survival in patients suffering from chronic obstructive pulmonary disease. Interventions aimed at increasing the sense of control and belief in self-efficacy in chronic pain and cardiac rehabilitation patients have had favourable outcomes [42]. It seems, though, that the successful function of increased control beliefs depends on focusing on these aspects of the illness and related behaviours for which exerting or increasing control is a realistic option, otherwise it may have the opposite effect [22].
Coping is defined as the process of managing demands (external or internal) that are appraised as taxing or exceeding the resources of the person [43]. Managing refers to mastering, tolerating, reducing or minimizing environmental and internal demands and conflicts among them [44]. Coping is viewed by Lazarus and Folkman [45] as a transactional process, as the interaction of a person who has a set of resources, values and commitments with the environment over time. They have identified two stages in the coping process: the primary appraisal of the event (e.g. in this context, does the illness threaten the patient's survival, future, wellbeing and QOL?); and the secondary appraisal, in which the individual reviews the available resources that could be used to manage the illness and its consequences for themselves and their family. A chronic illness is usually appraised as a threat/stressor that requires from the individual extra and/or different resources to manage it, reorganization of lifestyle and access to new resources, such as medical services. Coping can be action-oriented (problem-focused), where behaviours are directed at resolving or managing the problematic situation (e.g. managing the symptoms and life changes caused by the onset of the disease), and/or intrapsychic (emotion-focused), in which behaviour is directed at managing the unpleasant emotions (anxiety, sadness, anger) associated with the stressful experience. In health-related situations both forms of coping are required.
Some preliminary evidence suggest that certain active coping strategies are associated with better psychological adjustment and avoidant ones with worse adjustment in chronic non-fatal illnesses. In patients suffering from RA, the use of wish-fulfilling fantasy (wishing that the illness had never happened to them) as a coping strategy was related to psychological maladjustment, while information-seeking was related to higher levels of wellbeing [46]. In a recent RA study by Murphy et al. [47], it was found that becoming self-conscious, avoiding situations, resting and being alone in pain, correlated positively with depression and disability scores; however, when levels of disability were controlled for, the associations of coping strategies with depression were no longer significant. In certain chronic illnesses functional disability and pain may be the important factors in determining coping strategy use and associated distress.
In evaluating coping strategies used by cancer patients, Dunkel-Schetter et al. [48] found that the patients reported as the most stressful aspects of their illness the fear and uncertainty about the future (41%), limitations in physical abilities, appearance and lifestyle (24%), and pain management (12%). Patients used as coping strategies: seeking social support, direct problem-solving, distancing, positive focus, cognitive escape/avoidance (not thinking about the situation), and behavioural escape/avoidance (through drinking, eating, sleeping).
As diseases like vasculitis are lifelong conditions it is likely that, for coping efforts to be adaptive, they should be directed at increasing acceptance and tolerance of the condition, its treatment and the associated side-effects, and at achieving satisfactory levels of QOL according to disease stage and severity. The identification of coping strategies associated with successful psychological adjustment in vasculitis (i.e. where depression and anxiety levels are low and QOL is satisfactory for patients and family members) at different stages of the disease and over time could inform and help tailor the psycho-educational interventions offered to these patients. It would also allow comparisons between different types of chronic illnesses, generating more information on the psychological impact of different disease types and on factors facilitating successful adaptation to them.
Studies of QOL in chronic rheumatic illnesses have shown that mood is significantly related to illness perceptions but not to markers of disease activity or damage. Drent et al. [49] measured QOL in Dutch patients suffering from sarcoidosis, a chronic rheumatic disease that can affect any system, causing inflammation and granulomas. They used the Sickness Impact Profile (SIP) to measure QOL, and the Cognitive Depression Index (CDI) of the Beck Depression Inventory (BDI) and the Positive and Negative Affect Schedule (PANAS) were used to assess mood levels. In the analysis, illness symptoms were controlled for and it was found that QOL was related to the patients perceptions of their complaints and depressive symptoms and not related to disease status, as measured by pulmonary markers, complaints and illness duration.
Burkhardt et al. [28] asked four groups of patients suffering from RA, osteoarthritis, ostomy or diabetes mellitus to state: (i) what QOL meant to them and (ii) what was important to their QOL. They report differences between the illnesses; for example, osteoarthritis patients valued being pain-free where as diabetes patients valued control over their symptoms. However, all patients identified independence, being able to do things for oneself, as a major aspect of their QOL. Burckhardt et al. [29], in another study, investigated QOL in Swedish women suffering from fibromyalgia, RA or systemic lupus erythematosus. They measured QOL with the Quality of Life Scale (QOLS-S) and pain with the McGill Pain Questionnaire. Other measures used were the Rheumatology Attitudes Index (RAI) and the Arthritis Impact Measurement Scales (AIMS). The fibromyalgia sufferers scored significantly lower on the QOLS-S total score and on specific items measuring health, relationships and recreation. Depression and anxiety were significant predictors of QOL for all three groups.
Previous research in vasculitis has focused on evaluating QOL using mainly generic instruments such as the Short Form-36 (SF-36). Hoffman et al.'s study [9] is to our knowledge the only relevant study in which a disease-specific questionnaire was devised with input from a patient focus group and the only one that has been published as a full research paper to date. The questionnaire assessed the effects of Wegener's granulomatosis, a form of vasculitis, on 60 patients health, function and income. 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 the patients normal daily living, employment circumstances and income and a variable impact on family and close relationships.
Exley et al. [50] used the SF-36 to assess QOL in 30 patients with active and inactive vasculitis. Patients reported lower scores than the general population, patients with inactive disease reporting similar scores at 36 months follow-up. The authors noted that patients with active disease reported changing scores over time. As this is information obtained from an abstract publication, more information is needed to appraise the study (e.g. information on sample characteristics and study findings). Herlyn et al. [51] compared a group of patients with active primary systemic vasculitis (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 of follow-up. They used the SF-36 to measure health-related QOL and they found that, for patients with primary systemic vasculitis, QOL was limited at baseline and at 12 months follow-up. Patients in complete remission reported limited QOL at both periods when compared with a healthy population in terms of general health, physical functioning and their role functioning due to physical limitations.
Raza et al. [52] investigated the relationship between vasculitis activity (using the Birmingham Vasculitis Activity Score, BVAS) and damage (using the Vasculitis Damage Index, VDI), combining scores in the separate SF-36 subscales into scores for physical and mental components in 83 patients suffering from systemic necrotizing vasculitis. They found that, overall, this patient group reported significantly lower SF-36 scores than a healthy population. Interestingly, there were no significant correlations between disease activity or disease damage scores and physical or mental components of the SF-36.
Koutantji et al. [53] examined the relationship between QOL scores using the SF-36 and physical characteristics of vasculitis in 51 patients with PSV. Data on disease-related variables were collected using modified versions of the BVAS and the VDI, and scores on pain, fatigue, symptom severity and sleep (101-point Visual Analogue Scales) were also obtained. The SF-36 was used to assess the patients QOL and the Hospital Anxiety and Depression Scale (HAD) to assess mood. There were no significant correlations between BVAS or VDI scores with any of the SF-36 subscales or with the other self-report disease-related measures, except for a negative correlation between the VDI and the SF-36 pain score. SF-36 scores in this patient cohort appear to be lower than those obtained from patients attending a tertiary referral centre [51] and lower than those in a normal population (of similar age). Patients with increased pain (VAS score >10) when compared with those without pain or with little pain (VAS score 10) had significantly impaired scores in all SF-36 subscales except for mental health. They also scored significantly higher on measures of depression, symptoms of fatigue, problems with sleep, and perceived symptom severity. The two groups did not differ in age, duration of illness, BVAS or VDI scores.
On the basis of the preliminary findings of the last two studies [52, 53], it seems that although clinical markers serve as indicators of physical status of vasculitis they are not necessarily related to measures of functioning. Self-reported pain or disability scores, scores for depression, 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 taken into account in overall management.
Koutantji et al. [54], as part of the above study, investigated the relationships between: (i) the use of specific coping strategies, mood and disease status, and (ii) patient perceptions of illness and mood. Coping strategies were assessed using the Ways of Coping Questionnaire (WOC) and patients perceptions of their illness were assessed using the Illness Perception Questionnaire. Multiple linear regression analyses were used to predict depression and anxiety scores from the degree of use of specific coping strategies, BVAS, VDI and pain scores. Correlations were computed for illness perception scores and mood. Overall, the most significant predictor of depression was the degree of use of self-control as a coping strategy (WOC) followed by pain severity and planful problem-solving (WOC). There was a negative correlation between planful problem solving and depression scores. The most significant predictor of anxiety was pain severity followed by the use of self-control and escape-avoidance (WOC). Anxiety levels were significantly correlated with the number of disease symptoms reported by the patients and the perceived consequences of the illness. Perceptions of low control over the illness were significantly correlated with high anxiety scores. Patient reports on the consequences of their illness were correlated with depression scores, severe perceived consequences being associated with higher depression scores. Depression and anxiety levels in PSV were significantly related to pain severity, the use of specific coping strategies, and patients illness perceptions, but not to disease activity or severity scores as measured by modified versions of BVAS and VDI.
There is a need for ongoing research into how vasculitis patients cope with their illness. The research findings from the different research centres need to be published in full so that their ecological validity and reliability can be assessed. Emerging evidence suggests that many aspects of patients QOL are impaired. Patients own evaluations of QOL levels of pain, coping strategies and perceptions about their illness seem to have a stronger relationship to their psychological adjustment than disease-related variables, as is the case in other chronic rheumatic diseases. Preliminary findings suggest that vasculitis has an impact on patients life similar to that of other chronic illnesses, and is associated with decreased QOL and increased distress. Psychological theory and methods can be applied to study unique aspects of the disease and similarities with other chronic conditions, to assess the psychological impact of changes in disease status, and to monitor the longitudinal impact of the disease. This information can lead to improved and tailored management of the condition by adding the development of management approaches that also address the psychosocial demands of the condition to the current medical management of the disease. Further research ought to address a number of issues such as: (i) the development of vasculitis-specific measures evaluating coping, functioning (impairment-disability) and other aspects of QOL; (ii) the investigation of meaning of the illness; (iii) the assessment of patients and carers concerns about the illness and negative and positive effects on their life, and (iv) the evaluation of the possible impact of stressful experiences and psychological variables as contributing factors in the recurrence of disease episodes.
We would like to thank D.G.I. Scott, S. Lane and R.A. Watts for stimulating our interest in vasculitis and supporting us with their specialist clinical knowledge of the disease.
Notes
Correspondence to: M. Koutantji
References