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- PATIENTS AND METHODS
Systemic sclerosis (SSc; scleroderma) is a chronic, disfiguring, painful, and currently incurable multisystem connective tissue disease that is associated with significant morbidity and mortality. There are 2 main subtypes. Diffuse cutaneous disease is associated with a high risk of life-threatening internal organ involvement early in the disease course. In contrast, the onset of limited cutaneous disease is often insidious; the diagnosis being made several years after the development of Raynaud's phenomenon. Internal organ involvement, with the exception of gastrointestinal involvement, is less common in patients with the limited cutaneous subtype. In recent years, there has been an increasing trend to examine psychological factors associated with SSc. A number of studies have reported associations with anxiety and depression (1, 2), somatization, obsessive-compulsiveness, and interpersonal sensitivity as compared with healthy controls (3). In line with these studies, there is increasing understanding that the relationship between clinical severity or disease factors within the condition and psychological impact is not linear (2). The lack of such a linear or direct relationship may suggest that illness-related variables and demographics cannot be relied upon to fully explain psychological adjustment to illness (4, 5).
Chronic conditions often present the individual with a profound array of disruptions and difficulties that may show considerable variation in type and severity as perceived by the patient. There is a rapidly increasing pool of data in the literature suggesting that to make sense of and respond to the difficulties illness may present, patients construct their own common sense cognitive model of their condition (6, 7). These illness representations are based on information patients may have received from doctors; general memorized information about health and illness; and lay information from family, friends, and the wider media. Illness representations are said to influence psychological outcomes such as distress (8), coping (9), and functional disability (10). Patient-held beliefs about disease have been shown to be of fundamental importance in adjustment to a wide range of chronic and major medical conditions, including rheumatoid arthritis (11), heart disease (12), and chronic obstructive pulmonary disease (13), in addition to conditions that have a disfiguring aspect, such as psoriasis (8). Thus, investigation of patients' views of their illness may have implications for heightening our understanding of how individuals with SSc comprehend and respond to their condition. No published studies to date have examined illness perceptions in patients with SSc.
The beliefs patients hold about their condition are predicated upon a complex set of illness perceptions that constitute the patient's implicit model of their particular condition. This implicit model is made up of perceptions about potential causes of the condition; perceived consequences of the illness; beliefs about cure or control, which includes personal control and the belief in treatment or recommended advice; timeline or expected duration of the condition; illness coherence or the understanding the individual has of their condition; and illness identity, which concerns the symptoms patients experience as part of their condition. Emotional representations, that is the emotional responses generated by the illness, are also considered to be a vital component of an individual's illness representations (14).
Given the increasing evidence documenting the importance of illness representations in adaptation to chronic conditions, we set out to examine patients' beliefs about their SSc and investigate the relationship between these beliefs and symptom report, clinical parameters of SSc, and other demographic variables.
- Top of page
- PATIENTS AND METHODS
This study set out to examine patients' cognitive appraisals or beliefs about their SSc, the emotional impact associated specifically with it, and the relationship of these beliefs to clinical and demographic variables. The results have shown that beliefs patients hold about their SSc are not determined by the 4 clinical parameters examined. This is in line with previous research literature in the area, which supports the assertion that objective severity of SSc is not associated in a linear manner with patients' subjective experience of their condition, whether that be distress, coping, or beliefs (1, 4, 5).
The clinical parameters of skin score, severity of ischemia, and disease subtype were not found to be related to increased symptom reporting (illness identity). Perhaps predictably, those with the more severe diffuse subtype did report increased consequences of their SSc and less personal control of their condition, but they did not differ in terms of their beliefs about treatment control, understanding of their condition, or their emotional responses. Although stronger beliefs in SSc having serious consequences does not necessarily equate to depressive symptoms, negative cognitions regarding the consequences of having SSc may be pertinent. Indeed, a study in RA (11) identified significant differences between depressed and nondepressed patients on both the consequence and control/cure dimensions of the original IPQ (20). Additionally, 1 study has reported control appraisals to be of importance in predicting psychological distress in SSc (5).
In the current study, multiple regression analysis indicated that beliefs associated with consequences of the condition strongly affect the patients' emotional response to the condition over and above other clinical and demographic variables. A similar pattern of results has been reported in multiple sclerosis (14) and implies emotional response, as assessed in the IPQ-R, has more to do with illness beliefs than clinical severity. It is, however, important at this juncture to recognize that the emotional representations subscale contained within the IPQ-R is not intended as a substitute measure of general mood, but rather a measure of emotional responses specifically generated by the condition. Given the apparent absence of a difference between disease subgroups and their emotional representations, and the presence of a significant difference in terms of consequences associated with the condition, it would appear important to consider the impact of SSc on both disease-specific and more general levels of psychological distress.
In terms of illness coherence, in excess of 50% of the sample felt that they did not have a clear picture or understanding of their SSc. It has been suggested that this may be important in terms of adjustment to illness. This is an important observation, because previous studies have shown that not only do patients want to learn about their condition and indeed may profit clinically from such information, but also that physicians may underestimate the degree to which this is so (21–23). Patients with a good understanding of their disease are more likely to achieve emotional distance from it and become more self motivated, thereby increasing their sense of control.
The results of the study are somewhat surprising in that more than 50% of patients ascribed the cause of their condition to stress. Although there is a comprehensive literature linking stress with the onset and exacerbations of a range of conditions (24, 25), there is a paucity of research associating stress with onset and exacerbations of SSc (26). In the absence of any evidence for a physiologic explanation linking stress and SSc, it may be that behavioral factors (such as poor sleep and engaging in less self care at times of distress, with the consequence of increased physical symptomatology) may link patients' perceptions of psychological distress with causes of the condition. The subject numbers within this study were too low to permit any further analysis of the cause subscale of the IPQ-R; however, it may be of interest in further work to disentangle the causes people attribute to the onset of their condition. Indeed, 1 study of people with psoriasis identified that pathologic worrying was strongly associated with the attribution of an emotional cause of their condition (27).
In line with this, it is interesting to note that within the current study a large proportion of patients were shown to worry about their condition. Although we did not undertake an examination of worrying in particular and are unable to make any firm statements in relation to this, worry has been demonstrated to have some clear clinical implications in a number of other conditions. Chronic worrying has been shown to be a significant risk factor for myocardial infarction at 20-years followup in the normative aging study (28), and within RA, worry is a strong predictor of poor functional status at 1-year followup (29). The findings of the current study would suggest examining this aspect of psychological adjustment further in patients with SSc.
It is recognized that a limitation of this study is the small sample size. However, the population included within the current study represents approximately one-third of current attendees at the tertiary referral clinic for SSc. Given the consecutive nature of sampling undertaken and the high response rate, the findings are considered representative of such a tertiary population. Whether the findings are generalizable to other populations of SSc patients is unknown.
The aim of the current article was to describe patients' beliefs about their SSc and relate these to clinical features of the condition. Additional work will be well placed examining the relationship of these illness beliefs to psychological adaptation and adjustment in patients with SSc. We are currently undertaking an examination of such.