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Keywords:

  • Quality of life;
  • Systemic lupus erythematosus;
  • Wegener's granulomatosis;
  • Systemic vasculitis

Abstract

  1. Top of page
  2. Abstract
  3. INTRODUCTION
  4. PATIENTS AND METHODS
  5. RESULTS
  6. DISCUSSION
  7. Acknowledgements
  8. REFERENCES

Objective

To describe the patients' perceptions of the effects of systemic lupus erythematosus (SLE) and Wegener's granulomatosis (WG) on health, function, income, and interpersonal relationships.

Methods

114 patients with SLE, and 79 patients with WG completed a self-administered questionnaire. Patients had been diagnosed with SLE or WG for a median period of 10 and 5 years, respectively.

Results

All patients experienced substantial functional morbidity. Two-thirds of the patients with SLE or WG reported either a periodic or permanent inability to perform daily activities at home and/or at work. Furthermore, SLE as well as WG had a considerable impact upon the psychological and social life, affecting their happiness and altering relationships.

Conclusion

Our study demonstrates that SLE and WG are associated with substantial medical morbidity resulting in physical and occupational disability. SLE has a profound impact on patients' lives, similar to that experienced in patients with WG.


INTRODUCTION

  1. Top of page
  2. Abstract
  3. INTRODUCTION
  4. PATIENTS AND METHODS
  5. RESULTS
  6. DISCUSSION
  7. Acknowledgements
  8. REFERENCES

Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease with significant morbidity and mortality. Survival of patients with SLE has increased greatly in the past 20 years (1–3). Early diagnosis and better treatment of the disease and its complications are the most likely explanations for the extended lifespan of most patients.

Although prolonging life is of clinical importance, health status, and quality of life are also important outcomes in SLE. Health status, quality of life, and the psychosocial impact of the disease in patients with SLE have been studied extensively by several groups (4–12). From these studies, it can be concluded that quality of life is impaired in patients with SLE (4, 6). Several psychosocial-, disease-, and therapy-related factors have been identified that influence quality of life (5, 7–9, 11, 12). Some (9, 11) but not all studies (7, 8) have found that quality of life is related to disease activity or fixed organ damage. Studies estimating the socioeconomic impact of SLE are scarce (12). This is probably due to the many aspects associated with measuring the socioeconomic impact of a chronic disease.

Recently Hoffman et al (13) studied patient-perceived effects of Wegener's granulomatosis (WG) on health, function, income, and interpersonal relationships, and concluded that WG had a major socioeconomic impact. WG and SLE are both multisystem autoimmune diseases that bear many similarities with respect to organ involvement and the course of disease. Furthermore, both diseases have been reported to be associated with significant mortality and morbidity (1–3, 14). Therefore we wondered whether the effects of SLE on patients' lives are similar to that experienced in WG. In order to study patients' perceptions of the effects of SLE and WG on health, function, income, and interpersonal relationships, we sent a copy of Hoffman's questionnaire to all of our patients with these diseases who attend clinic in Groningen (the Netherlands), and compared the socioeconomic impact of SLE with that of WG.

PATIENTS AND METHODS

  1. Top of page
  2. Abstract
  3. INTRODUCTION
  4. PATIENTS AND METHODS
  5. RESULTS
  6. DISCUSSION
  7. Acknowledgements
  8. REFERENCES

The original questionnaire was developed by Hoffman et al to assess health, social, and economic impact of WG from a patient perspective (13). The majority of questions were multiple choice questions (e.g., How would you rate the present impact of the disease [including side effects of therapy] on your ability to perform your daily activities at home/or at work?: 1. No effect [no loss of ability to do all things you want to do], 2. Periodic effect [period of worsening that improved to normal or near normal following therapy], 3. Permanent inability to do at least some usual activities at home or work [for example, inability to drive or inability to participate in a favorite sport]). Questions were divided into the categories of health, function, income, and interpersonal relationships. Patients could write down additional comments at the end of the questionnaire. A separate questionnaire was developed for “life- partners” or spouses, to solicit their views on the patient's disease activity, effects of the disease on the patient's function, and psychological status of the partner (e.g., From your current perspective, has your spouse's illness affected his/her relationship with other people?: 1. Yes, 2. No, 3. Not sure). The complete questionnaire can be found on the American College of Rheumatology web site at www.rheumatology.org/ar/ 1998/hoffman.pdf. Thirty to 60 minutes are required to complete the questionnaires. Permission was obtained from Dr. Hoffman to translate this instrument into Dutch for use with WG patients, and to adapt the questionnaire for use in SLE patients. In the SLE questionnaire, minor adaptations were made with respect to questions regarding organ involvement (breathing tube, and intestine involvement were added in SLE; ear, nose, and throat (ENT) was replaced by heart involvement in SLE version), treatment (trimethoprim/sulfa was replaced by hydroxychloroquine in SLE version), medical interventions (ENT/eye surgery and lung/nerve/ nasal biopsy were deleted in SLE version), and complications of the disease or its treatment (blindness was deleted in SLE version).

Patients older than 18 years with either SLE or WG who were followed at the outpatient clinic of the University Hospital Groningen, The Netherlands, were eligible. Patients with SLE had to fulfill at least 4 of the criteria as defined by the American College of Rheumatology (ACR) for SLE (15). Patients with WG had to fulfill the ACR 1990 criteria for WG (16) and meet the 1992 Chapel Hill Consensus Conference (17) definition for WG. Furthermore, eligibility of WG patients required a positive test for PR3-ANCA (18) during an active phase of the disease, either current or in the past.

The questionnaires were mailed to 150 patients with SLE and 91 patients with WG who met these criteria. The authors requested that the patients and their partners complete their respective questionnaires independently and return their answers anonymously. Our institutional ethics review committee approved the study design.

Statistical analysis

Categorical quantities were compared using the Fisher's exact test (2 rows and 2 columns) or chi-square test (more than 2 rows and 2 columns), and the Mann–Whitney test (unpaired observations) or Wilcoxon matched pairs test (paired observations) were used for continuous quantities. Reliability between patient and spouse opinions was evaluated using a kappa statistic. The weighted kappa statistic is a chance-corrected measure of agreement that is equal to 0 if the agreement between patient and spouse is what would be expected based on chance alone, and equal to 1 if there is perfect agreement between patient and partner.

RESULTS

  1. Top of page
  2. Abstract
  3. INTRODUCTION
  4. PATIENTS AND METHODS
  5. RESULTS
  6. DISCUSSION
  7. Acknowledgements
  8. REFERENCES

Patient characteristics

One hundred and fourteen of 150 patients with SLE (76%) and 79 of 91 patients (87%) with WG responded after 1 mailing. Patients were mainly white (94%). Eighty-four of the 114 patients (74%) with SLE lived with a partner, and all of these partners (100%) responded. Fifty-nine of the 64 partners (92%) of the WG patients responded. As could be expected, there were differences in age and sex between the SLE and WG patients (Table 1). Patients with SLE were younger at the time of diagnosis and a greater number were women (P < 0.0001). Duration of disease (P < 0.0001), and patient-reported delay from onset of symptoms to diagnosis of the disease (P = 0.009) were longer in patients with SLE. Sixty-one percent of the SLE patients and 51% of the WG patients reported consultation of at least 3 physicians before the diagnosis and initiation of treatment occurred (not significant [NS]). During this period many patients were told that their problems were psychosomatic (14% WG patients versus 25% of the SLE patients; NS).

Table 1. Features of patients with WG and SLE from patient-reported data
Patients with SLEPatients with WGP
  1. SLE = systemic lupus erythematosus; WG = Wegener's granulomatosis; NS = not significant.

Responded/total patients114/15079/91NS
Responded/total partners84/8459/64NS
Age at diagnosis, mean (range) years31 (7–70)52 (20–79)<0.0001
Age at questionnaire, mean (range) years44 (20–80)60 (27–90)<0.0001
Sex (Male/Female)17/9744/35<0.0001
Diagnosis <2 years of presentation64%85%0.0016
Diagnosis, mean delay (range) months37 (0–336)10 (0–108)0.009
“Psychosomatic” cause suggested25%14%NS
Disease duration, median (range) years10 (1–46)5 (0–25)<0.0001

Organ involvement

Eighty-one percent of the SLE patients and 91% of the WG patients reported initial involvement of more than one organ system, and in 45% and 35% of the patients with SLE and WG, respectively, additional organ systems were eventually affected by the disease during followup. The distribution of organ involvement at presentation and during followup, as reported by the patients, is listed in Figures 1A and B.

thumbnail image

Figure 1. A, Frequency of organ involvement in systemic lupus erythematosus (SLE) and B, Wegener's granulomatosis (WG) as reported by 114 patients with SLE and 79 patients with WG on the study questionnaire at presentation of disease and during followup. CNS = central nervous system.

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Disease activity and effects on daily living

Sixty-six percent of the SLE patients and 68% of the WG patients described their disease as inactive at the time of the questionnaire. Two-thirds of both groups reported that their disease had either a periodic or permanent effect on their ability to perform everyday activities at present. Daily life was, therefore, to some extent compromised in most patients of both groups (Table 2 and Figure 2). Patients with SLE (patient mean 4.2, partner mean 4.8; P = 0.0013) felt that the disease had affected their daily living more severely than patients with WG (patient mean 7.0, partner mean 6.8; NS). The agreement between patients and partners about patient-reported effects on daily living was somewhat higher in the WG patient group than in the SLE group (WG patients κ = 0.65, SLE patients κ = 0.57; NS). However, two-thirds of the patients reported that their current health had improved at the time the questionnaire was answered, and that their disease had less influence on daily life than at the moment of diagnosis (Figure 2B).

Table 2. Impact of WG and SLE on daily life*
ImpactSLE Patients, %WG Patients, %
  • *

    P not significant.

  • See Table 1 for definitions.

Not compromised1214
Slightly compromised3542
Moderately compromised4128
Severely compromised1315
thumbnail image

Figure 2. Distribution of scores of 114 patients with systemic lupus erythematosus (SLE) and 79 patients with Wegener's granulomatosis (WG) who were asked to rate how much the disease had affected their daily life at diagnosis A and B, at the moment of answering the questionnaire. Scale: 1 = not at all to 10 = changed everything.

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Employment status

Patients with SLE generally had a higher level of education than patients with WG (data not shown). Regarding the patients who had been employed for at least one year, remarkably similar findings between SLE and WG were found with respect to the impact of the disease on employment status. Patients frequently had missed work due to their disease. WG patients had been absent for at least 6 consecutive weeks, which was somewhat more frequent than SLE patients (80% versus 61% of SLE patients; P = 0.05). SLE patients, however, tended to miss work for longer periods. Eighty-one percent of the SLE patients that were absent for 6 consecutive weeks had not resumed work after 6 consecutive months (WG patients 53%; NS). About half of the patients with SLE and WG had changed duties at work since they were first diagnosed; and in nearly all patients this change was attributed to the disease. Furthermore, patients often worked a reduced number of hours (56% of SLE patients versus 41% of WG patients; NS). SLE patients had to resign from their job more often (23% versus 5% of the WG patients; P = 0.05) due to their disease. Eighteen percent of SLE patients and 23% of WG patients (NS) reported an income reduction due to disease, and in 8–10% of the cases, family members had to change their employment status. More than 50% of all patients were currently unemployed, and 25% received disability benefits. In WG patients, the reason for being currently unemployed was more often “retirement” (SLE 15% versus WG 49%; P = 0.0001) and in SLE patients the reason was more often “to continue formal education programs” (SLE 10% versus WG: 0%; P = 0.04), or “fear of losing benefits” (SLE 16% versus WG 7%; P < 0.0001).

Emotional reactions

SLE and WG have a profound impact on patients' well-being. SLE and WG equally affected patient happiness (34% of SLE patients were unhappy because of the effects of illness versus 32% of WG patients; NS). Slightly more SLE patients reported disease-related depression (47% versus 33% of the WG patients; NS), and more SLE patients reported suicidal thoughts (14% versus 7% of the WG patients; NS). Interestingly, some patients reported depressive thoughts although they stated that the disease had no, or even a positive effect on their happiness. No significant difference was noted between the number of patients and partners who believed that SLE or WG had created problems with depression. However, the degree of agreement between patients and partners about patient- reported depressive thoughts was, again, higher in the WG patient group than in the SLE group (agreement in WG patients κ = 0.84, agreement in SLE patients κ = 0.53; NS).

Personal relationships

SLE and WG have a considerable impact on interpersonal relationships. About half of the patients reported that their illness had affected their relationships with people (SLE 40% versus WG 55%; P = 0.05). The disease had affected their relations with family, friends, and their partner (Table 3). Some differences between SLE and WG patients were found in the answers to questions pertaining to interpersonal relationships. SLE patients more often thought that their illness improved their relationship with their friends (SLE 20% versus WG 7%; P = 0.04), and brought their family closer together (SLE 25% versus WG 16%; NS). No remarkable differences were seen in the number of SLE and WG patients that believed that their relationship with their partners improved or worsened (Table 3). No significant difference was noted between the number of patients and partners who believed that SLE or WG had affected their relationship. However, the degree of agreement between patients and partners about the effect of the illness on their relationships was slightly higher in the WG patient group than in the SLE patient group (agreement in WG patients κ = 0.71; agreement in SLE patients κ = 0.47; NS).

Table 3. Impact of WG and SLE on patient's relationships
ImpactSLE Patients, %WG Patients, %P
  1. See Table 1 for definitions.

Brought the family closer together2516NS
Driven family apart1210NS
No effect on family6374NS
Relationship with partner improved1920NS
Relationship with partner worsened89NS
No effect on relationship with partner7371NS
Relationship with friends improved2070.04
Relationship with friends worsened24230.04
No effect on relationship with friends56700.04

Concerns

Partners of patients were asked whether they had disease-related concerns about the future. Up to one-third of the partners of SLE and WG patients were very concerned about the long-term effects of the disease and treatment, but only a minority of the partners had great concerns about the implications of the disease on financial security (Table 4).

Table 4. Partner responses of WG and SLE patients*
ConcernsSLE Patients, %WG Patients, %
  • *

    P not significant.

  • See Table 1 for definitions.

Financial security
 Not concerned6071
 Somewhat concerned2724
 Very concerned135
Long-term effects of disease
 Not concerned1113
 Somewhat concerned5561
 Very concerned3426
Long-term effects of treatment
 Not concerned2023
 Somewhat concerned5448
 Very concerned2529

DISCUSSION

  1. Top of page
  2. Abstract
  3. INTRODUCTION
  4. PATIENTS AND METHODS
  5. RESULTS
  6. DISCUSSION
  7. Acknowledgements
  8. REFERENCES

Advances in medical care have transformed SLE and WG from diseases with a high mortality to conditions with serious chronic morbidity. In this study we describe the patients' perceptions of the effects of SLE and WG on health, function, income, and interpersonal relationships.

The response rate for the subjects from our clinic was remarkable. Seventy-six percent of the SLE patients and 87% of the WG patients responded after 1 mailing. This is very high compared to other studies, in which response rates have varied from 14 to 60% after up to 2 mailings (10, 13). Due to the good response rate and the fact that disease activity was generally absent in the majority of patients in both groups, the primary bias of sampling sick patients appears to have been minimized.

The results convey a sense of profound impact of disease on the physical and occupational abilities of patients with SLE and WG. Two-thirds of the patients reported either a periodic or permanent inability to perform daily activities at home and/or at work. Two-thirds of the patients who had been employed for at least 1 year before diagnosis had been off work for at least 6 consecutive weeks due to their disease, and more than half of the patients had changed duties at work and/or reduced hours. These findings are comparable with those of Hoffman et al who studied WG patients in Cleveland, Ohio (13). In SLE patients, Thomboo et al evaluated working status, defined as stopping work (temporarily or permanently), or a change in the nature or place of employment, or working part time as a result of the disease (12). Working status in this study changed in only 24% of the SLE patients. This was, however, a prospective study and only changes in working status during the study period of 6 months were noted. Our data also suggest that the illness has a marked financial impact on patients and their families. One-fourth of patients with SLE, as well as patients with WG, received disability benefits, and one-fifth of the patients who had been employed for at least 1 year reported a reduction in income, as was also found in WG patients in the US (13).

Finally, the disease has a considerable impact upon the psychological and social life of patients with SLE or WG. About one-third of the patients reported that the disease had affected their happiness and caused depressive thoughts. Half of the patients reported altered relationships with family, friends, and partners, suggesting isolation and conflict. However, ≤12% felt that relationships between family members and partners had suffered, which was a very encouraging observation. These findings are in agreement with previous studies (10, 13) that also reported altered relationships with family, friends, and spouses in patients with SLE as well as patients with WG. In SLE patients, loss of social activity was significantly correlated with depression (10). Whether these findings are directly related to the illness is not clear. It has been demonstrated that psychological disturbances in patients with SLE may be the result of adjustment difficulties of being chronically ill or of life stresses not directly related to the illness (19). In our study, there were also patients who reported a positive effect on their happiness and improved relationships with family, friends, and partners as was also observed by Liang et al (10) and Hoffman et al (13). This suggests a reaffirmation of life, organized around new priorities.

We observed striking differences in answers on several items scored at time of diagnosis compared to the present situation. In line with observations of Hoffman et al (13), the majority of patients reported that their current health had improved and that their disease had less influence on daily life than at the moment of diagnosis.

Overall, it can be concluded from this study that SLE and WG, both chronic rheumatic illnesses for which modern treatment has had a great impact on survival, still profoundly impact patients' lives. We have also demonstrated that these findings in WG patients in The Netherlands were similar to those of WG patients in the US (20). WG has great socioeconomic impact, regardless of cultural background. Although unique differences in clinical features and treatment exist between WG and SLE, both illnesses have similar impact on life functions and mood.

Apart from similarities shared by SLE and WG, important differences in the physical and occupational disabilities were also noted. These differences may be partly explained because SLE patients are younger and more likely to be women than patients with WG.

Our study has several limitations. The questionnaire was originally designed and later validated for use in WG patients (13). Although we provided several minor adaptations to make the questionnaire more suitable for patients with SLE, it was not possible to compare specific treatments (medical or surgical) between diseases because of inherent clinical differences and needs of patients (e.g., ENT in WG, serositis and hemocytopenia in SLE). Comparison of these items was further hindered by differences in sex and age in both groups. An additional issue that needs to be established is the validity, for instance the test-retest reliability, of the questionnaire.

Recently, a study of Abu-Shakra et al revealed that in various aspects of life, patients with SLE have quality of life scores, as assessed by the quality of life scale, similar to those of healthy people, suggesting that it is important to compare patients with SLE not only with other diseases, but also with healthy controls (4). Unfortunately, we were unable to compare the results with those of healthy controls since our questionnaire is not applicable to healthy controls.

This single-site study examined the cumulative impact of SLE and WG on health-related quality of life in a cross-sectional setting. Rather than evaluating the status of patients at multiple time points as was done in the study of Thumboo et al (12), the questionnaire was developed to assess the impact of the disease over the course of the illness.

With regard to disclosure of financial and personal information, the questionnaire was anonymous. Due to this anonymity, patient-reported information pertaining to medical issues such as disease duration, delay between initial symptoms and diagnosis, and organ involvement could not be verified. However, we felt that anonymity was a primary condition in order to get adequate response regarding psychosocial/mental health status and income. Furthermore, medical issues were not the main focus of the questionnaire.

Comparison of the present study with previous studies involving SLE patients is difficult because the purpose for which our questionnaire is designed is different from, for instance, the Medical Outcomes Short-Form 36 Health Survey (21) which has been widely used in previous studies (6–8, 12).

In conclusion, our study demonstrates that SLE and WG are associated with substantial medical morbidity, resulting in physical and occupational disability. Minor differences, however, exist with respect to the influence of SLE and WG on patients and their family relationships. We hypothesize that most of the latter differences are due to age and sex differences in patients with SLE and WG. SLE, like WG, has a profound impact on patients' lives.

Acknowledgements

  1. Top of page
  2. Abstract
  3. INTRODUCTION
  4. PATIENTS AND METHODS
  5. RESULTS
  6. DISCUSSION
  7. Acknowledgements
  8. REFERENCES

The authors are very grateful to Annie Mellema for providing secretarial assistance, and Dr. Wim Sluiter for providing statistical support.

REFERENCES

  1. Top of page
  2. Abstract
  3. INTRODUCTION
  4. PATIENTS AND METHODS
  5. RESULTS
  6. DISCUSSION
  7. Acknowledgements
  8. REFERENCES