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Keywords:

  • Quality of life;
  • Giant cell arteritis;
  • Questionnaire

Abstract

  1. Top of page
  2. Abstract
  3. INTRODUCTION
  4. METHODS
  5. RESULTS
  6. DISCUSSION
  7. Acknowledgements
  8. REFERENCES
  9. APPENDIX A

Objective

To determine aspects of quality of life (QOL) important to people with giant cell arteritis (GCA).

Methods

We explored the domains of QOL affected by GCA in audiotaped focus groups. We then created an Importance Rating Questionnaire (IRQ) by constructing questions related to the domains most frequently mentioned. Of 214 GCA patients to whom the IRQ was sent, 145 (68%) responded. We calculated frequencies of responses and then ranked items by the proportion selecting the top category of importance and also according to a mean item rank. We compared the domains of QOL covered by the IRQ with those in the Short Form 12 (SF-12).

Results

The highest rated QOL item was “losing sight in both eyes permanently.” Of the top 20 items, 12 were in domains not covered directly by the SF-12.

Conclusion

We have identified aspects of QOL important to GCA patients. Assessment of QOL in GCA should include vision and other domains that are not included in standard QOL questionnaires.


INTRODUCTION

  1. Top of page
  2. Abstract
  3. INTRODUCTION
  4. METHODS
  5. RESULTS
  6. DISCUSSION
  7. Acknowledgements
  8. REFERENCES
  9. APPENDIX A

Giant cell Arteritis (GCA) is the most common form of primary systemic vasculitis, with a prevalence in the United States of greater than 110,000 cases (1). Since Hutchinson described the first case of GCA in 1890, many clinical studies have shown that both the disease and its treatment are likely to profoundly impact patients' health-related quality of life (QOL) (2–7). GCA can cause nonspecific symptoms of inflammation (e.g., fever, malaise, weight loss, or polymyalgia rheumatica) and symptoms directly attributable to inflammatory destruction of arteries (e.g., headache, painful temporal artery, jaw claudication, visual loss, or stroke) (2–17). Blindness, one of the most feared complications of GCA, develops in 10–20% of patients (2–7).

Corticosteroids, the cornerstone of therapy in GCA, frequently produce side effects (2, 16, 17). In contrast to the comprehensive study of clinical outcomes, there has been no previous attempt to measure formally how GCA and the complications of treatment influence patients' assessments of QOL. Patients' assessments of QOL are important because they add depth to our understanding of how a disease and its treatments affect them (18, 19). In studies of patients with other diseases, including diabetes (20), human immunodeficiency virus infection (21–24), and chronic renal failure (19), QOL information has been more relevant to both clinicians and patients than many other outcomes in making treatment decisions. Generic health survey instruments, such as the Short Form-12 (SF-12) and Short Form-36 (SF-36), may be useful for making comparisons among different kinds of patients. However, to assess impact of a disease on health-related QOL, such measures often need to be supplemented by disease-specific QOL measures. We, therefore, conducted this study to determine domains of quality of life affected by GCA.

METHODS

  1. Top of page
  2. Abstract
  3. INTRODUCTION
  4. METHODS
  5. RESULTS
  6. DISCUSSION
  7. Acknowledgements
  8. REFERENCES
  9. APPENDIX A

This project was conducted by the Johns Hopkins Vasculitis Center in conjunction with other participating members of the International Network for the Study of Systemic Vasculitides (Beth Israel Medical Center [New York, New York], Boston University [Boston, Massachusetts], Cleveland Clinic Foundation [Cleveland, Ohio], Dartmouth Hitchcock Medical Center [Lebanon, New Hampshire], Hospital Avicenne at Paris University [Paris, France], Hospital Clínic, University of Barcelona [Barcelona, Spain], Lahey Clinic [Burlington, Massachusetts], and the University of Alabama [Birmingham, Alabama]). This study was approved by the Johns Hopkins University's Joint Committee for Clinical Investigation and the institutional review boards of the other participating centers.

Focus groups.

We conducted focus groups with GCA patients to explore the domains of QOL affected by the disease and its therapy. We invited the 41 GCA patients who had been evaluated in The Johns Hopkins Vasculitis Center since 1996 to participate in 1 of 3 focus group sessions. All patients who participated in this study met American College of Rheumatology (ACR) criteria for the classification of GCA (25). Of the 41 patients invited to participate, 17 (41.5%) provided informed consent and participated in the sessions. A trained facilitator conducted 3 90-minute focus group sessions, each of which included 5 or 6 patients. The facilitator asked open-ended questions about the impact of GCA and its treatment on the patients' QOL (Appendix A).

Group sessions were audiotaped and transcribed. Two members of the research team independently classified each comment into domains and subdomains (e.g., pain, vision, activity) of QOL. Differences were resolved by consensus between these 2 investigators.

Importance Rating Questionnaire.

We created an Importance Rating Questionnaire (IRQ) by constructing questions related to each of the domains and subdomains most frequently mentioned by the focus group participants, using the patients' own language. When possible, items used positively worded stems, asking patients how important the different items were to their QOL (e.g., “How important to your quality of life is being active?”). For items that did not lend themselves to positive wording, we asked the respondents to rate the importance of the items in interfering with QOL (e.g., “How important an interference with your quality of life would each of the following be if you had it: Having to wear glasses to read?”). The response categories for all items were extremely important, very important, somewhat important, not very important, not at all important, and don't know.

We piloted this draft of the IRQ with 5 GCA patients and edited it based on their feedback. The revised questionnaire was circulated among the research group physicians, and revised further based on their comments. The final IRQ, which contained 79 variables (plus demographic information), was translated into Spanish and French. To verify the accuracy of the translations, the Spanish and French versions were translated back into English and compared with the original. The final IRQ contained 38 items that were positively worded and 41 that were negatively worded.

We administered the IRQ to GCA patients in the United States, Spain, and France. Physicians at all participating centers identified the 10–20 most recently diagnosed patients with GCA receiving care at their center. Informed consent was obtained from each patient who completed a questionnaire. Questionnaires were administered by mail.

Data analysis.

For each center, we calculated the frequency of the ordinal rating rank for each item. We also calculated a sample mean rating rank for each center. Proportions and mean ranks for each center were averaged to determine an average study-wide proportion and a grand mean ordinal rating rank for each item. Items were then ranked by the proportion selecting the top category of importance, “extremely important,” because there were better discrimination and fewer ties using this method.

We compared the domains of QOL covered by the IRQ with those represented in a standard QOL instrument, the SF-12. We determined whether there was an effect of negative or positive wording by comparing the importance ranking of a similar item that was worded both positively and negatively in the IRQ (being able to see with both eyes, having lost sight in 1 eye permanently).

RESULTS

  1. Top of page
  2. Abstract
  3. INTRODUCTION
  4. METHODS
  5. RESULTS
  6. DISCUSSION
  7. Acknowledgements
  8. REFERENCES
  9. APPENDIX A

IRQ respondents.

We invited a total of 214 patients with GCA to participate. Among these, 145 (68%) completed the IRQ. All 145 met ACR criteria for the classification of GCA, and 75% of the patients with known temporal artery biopsy status had positive biopsies. Of the respondents, 95 (65%) were American, 30 (21%) Spanish, and 20 (14%) French. Their mean age was 75 years (range 55–89 years). Nearly 98% were white, and 77% were female. The population had an average of 12 years of education (range 0–24 years). Forty-three percent of the patients reported having decreased vision (although not specifically related to GCA or other diseases), and 44% rated their eyesight as fair or poor. The mean highest dose of prednisone ever taken was 60 mg (range 2.5–165 mg). Forty-eight percent of the respondents had disease duration of less than 2 years.

IRQ responses.

Table 1 lists the 20 items that received the highest average importance ratings, ranked in descending order by the average proportion of patients across centers who rated the item extremely important. The following 2 columns give the average percentage of patients who ranked a given item extremely important, and the grand mean ordinal rating rank (out of a total possible score of 5.0). Table 2 lists the 20 items that received the lowest average importance rating. Tables 1 and 2 consider both positively and negatively worded items together.

Table 1. Top 20 items as ranked by average center proportion of respondents indicating extremely important*
ItemRank by average % EIAverage % EI (center range)Grand Mean ordinal rating (center range)
  • *

    Ordinal rating 1 = not important, 5 = extremely important. EI = extremely important.

  • Not included in top 10 positively worded items.

Losing sight in both eyes permanently194 (81–100)4.9 (4.4–5.0)
Losing sight in one eye permanently284 (69–100)4.7 (4.3–5.0)
Losing control of your arms or legs383 (50–100)4.8 (4.4–5.0)
Being able to see with both eyes480 (50–100)4.7 (4.3–5.0)
Being able to shower shave and take care of personal hygiene577 (50–90)4.7 (4.3–4.9)
Being able to walk without losing your balance675 (50–100)4.7 (4.5–5.0)
Being able to eat, chew, and swallow774 (47–87)4.6 (4.2–4.9)
Having intense or severe pain870 (33–89)4.6 (4.3–4.9)
Being able to walk without difficulty969 (41–89)4.7 (4.4–4.9)
Having fevers for a long time without knowing why1067 (53–88)4.3 (4.5–4.9)
Being able to control body movements1167 (47–92)4.6 (4.2–4.9)
Being able to walk without pain1266 (47–100)4.6 (4.3–5.0)
Being out of breath most of the time1366 (45–87)4.6 (4.3–4.9)
Pain that lasts a long time1465 (33–83)4.6 (4.3–4.8)
Being able to see well enough to read1564 (45–78)4.5 (4.2–4.8)
Feeling useless1664 (50–88)4.5 (4.2–4.9)
Being able to cross the street1762 (45–90)4.4 (4.1–4.8)
Having your legs feel very heavy1861 (43–89)4.5 (4.3–4.9)
Knowing you can cope with things1959 (36–78)4.5 (4.4–4.7)
Being able to get out of a chair2059 (47–92)4.5 (4.3–4.7)
Table 2. Bottom 20 items as ranked by average center proportion of respondents indicating extremely important*
ItemRank by average % EIAverage % EI (center range)Grand mean ordinal rating (center range)
  • *

    Ordinal rating 1 = not important, 5 = extremely important. EI = extremely important.

Having to wear glasses to read8113 (3–33)2.6 (2.3–3.7)
Feeling angry about your illness8021 (7–63)3.6 (3.1–4.6)
Being able to get through the day without resting or lying down7922 (8–47)3.6 (2.8–3.8)
Feeling different than you expected7822 (13–38)3.7 (3.6–3.8)
Being able to garden7722 (17–36)3.3 (2.5–3.6)
Having a tender scalp7623 (15–40)3.8 (3.3–4.1)
Having to watch out for more symptoms7525 (17–32)3.8 (3.7–4.0)
Being able to work at a job or profession or business7425 (13–33)3.2 (2.3–4.0)
Getting a bloated, round, or fat face7328 (13–50)3.5 (2.6–4.1)
Having no appetite7228 (22–46)3.8 (3.6–4.3)
Losing your hair over several weeks7129 (15–56)3.7 (2.7–4.5)
Being able to travel7029 (17–41)3.7 (3.5–4.1)
Being able to watch TV6929 (17–50)3.8 (3.6–4.5)
Being able to clean the house6830 (17–50)3.8 (3.3–4.5)
Feeling afraid of doing things6731 (17–53)3.9 (3.4–4.4)
Worrying about what could go wrong6631 (17–43)3.9 (3.5–4.4)
Being content with things6532 (20–44)4.1 (3.8–4.3)
Being able to exercise, for example, swim, walk, bike, tennis, aerobics, or treadmill6433 (5–44)3.7 (2.8–4.3)
Feeling afraid of what will happen6333 (8–50)3.9 (3.4–4.4)
Having to push yourself6234 (8–63)4.1 (3.8–4.6)
Having episodes of feeling numb6136 (20–60)4.0 (3.8–4.5)

In descending order, the QOL items rated most important were 1) losing sight in both eyes permanently; 2) losing sight in 1 eye permanently; 3) losing control of arms or legs; 4) being able to see with both eyes; 5) being able to shower, shave, and take care of personal hygiene; 6) being able to walk without losing one's balance; 7) being able to eat, chew, and swallow; 8) having intense or severe pain; 9) being able to walk without difficulty; and 10) having fevers for a long time without knowing why (see Table 1).

Comparison with the SF-12.

Of the top 20 items, 12 are in domains that are not covered directly by the SF-12, such as vision, personal hygiene, and loss of extremity control. In addition, 8 other items among the top 20 reflect domains that are covered on the SF-12 (e.g., activity, pain, or emotional issues) and are much more specific than the SF-12 items in these areas.

Effects of positive and negative wording.

Determining the top 10 most important positively worded items and the 10 most important negatively worded items separately yielded the results shown in Tables 3 and 4. Because Table 1 is almost evenly divided (11 positive and 9 negative) between positively and negatively worded items, the top 20 items from Table 1 are also in 19 of the 20 positions in Tables 3 and 4. One additional item not in Table 1 was ranked among the top 10 negatively worded items: “feeling weak, tired, or exhausted.” “Being able to get out of a chair” is in the top 20 in Table 1, the combined ranking, but is ranked 11 among positively worded items.

Table 3. Top 10 positively worded items as ranked by average center proportion of respondents indicating extremely important*
ItemsRank by average % EIAverage % EI (center range)Grand mean ordinal rating (center range)
  • *

    Ordinal rating 1 = not important, 5 = extremely important. EI = extremely important.

Being able to see with both eyes480 (50–100)4.7 (4.3–5.0)
Being able to shower shave and take care of personal hygiene577 (50–90)4.7 (4.3–4.9)
Being able to walk without losing your balance675 (50–100)4.7 (4.5–5.0)
Being able to eat, chew, and swallow774 (47–87)4.6 (4.2–4.9)
Being able to walk without difficulty969 (41–89)4.7 (4.4–4.9)
Being able to control body movements1167 (47–92)4.6 (4.2–4.9)
Being able to walk without pain1266 (42–100)4.6 (4.3–5.0)
Being able to see well enough to read1564 (45–78)4.5 (4.2–4.8)
Being able to cross the street1762 (45–90)4.4 (4.1–4.8)
Knowing you can cope with things1959 (36–78)4.5 (4.4–4.7)
Table 4. Top 10 negatively worded items as ranked by average center proportion of respondents indicating extremely important*
ItemsRank by (average % EI)Average % EI (center range)Grand mean ordinal rating (center range)
  • *

    Ordinal rating 1 = not important, 5 = extremely important. EI = extremely important.

  • Item not ranked among the top 20.

Losing sight in both eyes permanently194 (81–100)4.9 (4.4–5.0)
Losing sight in one eye permanently284 (69–100)4.7 (4.3–5.0
Losing control of your arms and legs383 (50–100)4.8 (4.4–5.0)
Having intense or severe pain870 (33–89)4.6 (4.3–4.9)
Having fevers for a long time without knowing why1067 (53–88)4.5 (4.3–4.9)
Being out of breath most of the time1366 (45–87)4.6 (4.3–4.9)
Having pain that lasts a long time1465 (33–83)4.6 (4.3–4.8)
Feeling useless1664 (50–88)4.5 (4.2–4.9)
Having your legs feel very heavy1861 (43–89)4.5 (4.3–4.9)
Feeling weak, tired, or exhausted2854 (35–82)4.5 (4.4–4.7)

Differences among US and non-US centers.

We compared the most important items as ranked “extremely important” by respondents from United States and non-United States centers. We found that 7 of the top 10 items were the same for the 2 groups. The top item was in the same position (losing sight in both eyes permanently). Items 7, 8, and 10 in the US top 10 were lower in the non-US rankings, and 3 additional items were in the top 10 for non-US centers. Two of these were “being able to walk without difficulty” and “being able to walk without pain,” which were in the top 20 but not in the top 10 for the US centers. The third item, “being able to cross the street,” moved up from below the top 20 (US) to the tenth position (non-US). None of these movements was statistically significant.

Effects of vison loss.

We analyzed the rankings according to vision, based on whether the patients reported excellent, very good, or good vision compared with fair or poor vision. We found that both groups agreed on 8 of the top 10 ranked items, including all the items pertaining to vision.

DISCUSSION

  1. Top of page
  2. Abstract
  3. INTRODUCTION
  4. METHODS
  5. RESULTS
  6. DISCUSSION
  7. Acknowledgements
  8. REFERENCES
  9. APPENDIX A

This study's contribution lies in its exploration of which health-related QOL domains are most important to patients with GCA. The results demonstrate the value of developing and using a disease-specific QOL instrument in studies of GCA, and provide essential background data for the development of such an instrument.

Although many studies have been conducted on the presentation, course, and treatment of GCA (2–7), our study is the first to provide detailed information about the domains of QOL affected by GCA. To our knowledge, only 1 previous study (26) has directly addressed the impact of GCA on QOL. That study, involving 20 GCA patients followed for 1 year, used the SF-36, and the Activities of Daily Vision Scale (ADVS), an objective assessment of visual function (26). Neither the ADVS nor the SF-36 revealed significant disability in this small group of GCA patients. That study did not attempt to define the broad areas of QOL that are affected by GCA, but merely relied upon currently available, generic QOL instruments. It should be emphasized that the SF-36 is a health survey instrument, and that health is only 1 domain of quality of life (27).

Our results show that vision ranks as the number 1 QOL concern of patients with GCA. Indeed, 3 of the 4 highest-rated QOL items concerned vision. Both positively and negatively worded items that related to vision were rated very high in importance by the patients in our study. Moreover, concerns about vision ranked at the top regardless of whether the patient was American, French, or Spanish. Together, these data reinforce the primacy of concerns about vision to the QOL of patients with GCA.

Several other highly rated QOL items relate to the maintenance of control over one's body and the maintenance of independence. After visual loss, avoiding loss of control of arms or legs and maintenance of body movement control were among the most highly rated items. The only mental health item that patients ranked in the top 20 concerned being able to “cope with things.” For this group of relatively elderly patients, maintenance of vision and body control were more important QOL concerns than preserving appearance or hobbies. Patients attached comparatively less importance to concerns about wearing eyeglasses, developing a round face, and losing hair temporarily (Table 2).

Some of the QOL domains important to patients with GCA have been identified as important to patients with other chronic, relapsing and remitting diseases (28–32). For example, Archenholtz et al found that systemic lupus erythematosus patients, like our GCA patients, were especially concerned with their perceived control over their bodies (29). Patients with rheumatoid arthritis have been found to be particularly concerned with threats against their independence (29). However, the many different methods used to survey QOL in these other diseases make direct comparisons with our study difficult.

The results of this study demonstrate the importance of using disease-specific instruments for measuring health-related QOL. Although generic instruments are very useful for making comparisons among different kinds of patients, they were not designed to provide information about the impact of a specific disease and its therapy on QOL. Therefore, generic instruments such as the SF-12 often need to be supplemented by more responsive, disease-specific questionnaires that include domains of QOL not covered in the SF-12. Our results emphasize the importance of this issue for patients with GCA: Of the top 20 most important QOL items identified by GCA patients in this study, only 8 are included in the SF-12. It is possible that the SF-36, which includes items such as vitality, would have been better than the SF-12 in capturing the health-related QOL concerns of patients with GCA. Neither the SF-12 nor the SF-36 contains any items pertaining to vision. Still, future research on QOL in GCA should include both the SF-12 and the SF-36.

QOL ratings are important for clinical research because they capture a dimension of health that cannot be measured by morning stiffness, the erythrocyte sedimentation rate, or other clinical and laboratory variables (33–47). These QOL ratings can help physicians and patients choose treatments that optimize the average patient's QOL. The importance of QOL ratings will increase as new therapeutic options for GCA become available. Indeed, trials of steroid-sparing agents have been completed recently (48–50).

Various sources of bias may have affected our study. For example, if patients in the focus groups had experienced more visual complications than typical patients, then vision might have emerged as a more important area to patients than it is. Forty-three percent of our patients reported reductions in vision from any cause (related to GCA and its treatment or not), a percentage 2 to 3 times greater than the percentage of patients with visual loss due to GCA reported by most studies in the literature (2, 13, 14). However, the importance of vision loss to QOL is underscored in our study by the finding that patients with normal vision ranked visual changes as high in importance as those who reported reductions in vision. Utility assessments have also demonstrated that blindness is among the QOL states that receive the lowest ratings from healthy people as well as those with visual loss (51).

The inclusion of patients from several different countries offered potential advantages and disadvantages. By recruiting patients from multiple centers in 3 countries, we accrued a larger sample than had we confined the study to a single center or to 1 country. All the countries included were Western and economically developed. We also undertook a back-translation and revision process to develop the questionnaire translation. Nevertheless, national, cultural, and subtle language differences could have influenced the QOL ratings, and our study was not powered to test this hypothesis. Additional testing will be needed to validate the translated questionnaire. However, it is reassuring that patients from the different countries gave similar rankings (data not shown).

Having items with both positive and negative wording might have biased our study if respondents placed different importance on the same concepts when they are negatively or positively worded. However, 3 lines of evidence suggest this type of bias did not occur in our study. First, 11 of the top 20 items (55%) were positively worded, similar to the proportion of positively worded items in the questionnaire (38 of 81, 47%). Second, when the top 10 positively and the top 10 negatively worded items are used to derive the top 20 items rather than the combined ranking, only 1 item (“feeling weak, tired, or exhausted”) changed. Finally, 2 items that represented a similar concept using positive and negative wording both received ranks in the top 5. These findings support the validity of combined ranking of positively and negatively worded items.

In conclusion, we have identified QOL issues that are important to patients with GCA. The most important issues relate to vision and body control. Few of the top 20 QOL items identified as most important are measured specifically by standard QOL instruments. Disease-specific QOL instruments should be included in future treatment trials of patients with GCA. Using the items ranked most important by GCA patients in this study, we plan to develop and validate a QOL questionnaire that can be used to measure this dimension of health in patients with GCA.

Acknowledgements

  1. Top of page
  2. Abstract
  3. INTRODUCTION
  4. METHODS
  5. RESULTS
  6. DISCUSSION
  7. Acknowledgements
  8. REFERENCES
  9. APPENDIX A

We thank Ms. Tammy Noethen for her word processing expertise.

REFERENCES

  1. Top of page
  2. Abstract
  3. INTRODUCTION
  4. METHODS
  5. RESULTS
  6. DISCUSSION
  7. Acknowledgements
  8. REFERENCES
  9. APPENDIX A

APPENDIX A

  1. Top of page
  2. Abstract
  3. INTRODUCTION
  4. METHODS
  5. RESULTS
  6. DISCUSSION
  7. Acknowledgements
  8. REFERENCES
  9. APPENDIX A
Table  . List of open-ended questions used to conduct the focus groups
1. Tell us about your quality of life as a person with giant cell arteritis (GCA)?
2. What are (other) good things about your quality of life?
3. What are (other) bad things about your quality of life?
4. How does prednisone affect your quality of life?
5. How have other treatments for GCA affected your quality of life?
6. What would you like to improve about your quality of life?
7. What is the best thing about your quality of life at present?
8. What is the worst thing about your quality of life at present? (What 1 thing do you miss the most)?
9. Is there anything else you would like to tell us about your quality of life with GCA?