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Rheumatology Advance Access originally published online on June 4, 2006
Rheumatology 2006 45(10):1298-1302; doi:10.1093/rheumatology/kel189
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© The Author 2006. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

Impact of pain in health related quality of life of patients with systemic sclerosis

C. Georges, O. Chassany1, C. Toledano, L. Mouthon2, K. Tiev3, O. Meyer4, D. Ilie, J. Rambeloarisoa, Z. Marjanovic, J. Cabane3, D. Sereni, J. Pouchot5 and D. Farge

Internal Medicine and 1Clinical Research Delegation, Saint Louis Hospital, 2Internal Medicine, Cochin Hospital, 3Internal Medicine, Saint Antoine Hospital, 4Rheumatology, Bichat Hospital and 5Internal Medicine, Georges Pompidou Hospital, Paris, France.

Correspondence to: Dr C. Georges, Internal Medicine Department, Saint Louis Hospital, Service de Médecine Interne, Hôpital Saint Louis, 1 avenue Claude Vellefaux, 75010 Paris, France. E-mail: claire.georges{at}sls.aphp.fr


   Abstract
Top
 Abstract
Introduction
 Patients and methods
 Results
 Discussion
 References
 
Objectives. Systemic sclerosis (SSc) has an heterogenous clinical pattern, with variable organ involvement and degrees of severity. Like in other rheumatic diseases, the self-questionnaires have been used to evaluate SSc globally. The aim of the study is as to evaluate the quality of life (QoL) in patients with either diffuse or limited SSc, and to examine the impact of pain on the QoL scores.

Methods. Patients with SSc, eitheir diffuse or limited SSc, were included in a cross-sectional study. The QoL was evaluated with the short-form 36 (SF-36) and the functional repercussion with the SSc-modified Health Assessment Questionnaire (S-HAQ).

Results. A total of 89 patients (67 with diffuse and 22 with limited SSc) were included. The SF-36 score values were lower in SSc patients than those reported in the general population. The physical component scores (PCS) of the SF-36 was significantly worse in diffuse compared with limited SSc (P < 0.05). The PCS was significantly negatively related to the number of clinical manifestations (ANOVA, P < 0.0001). The mental component score (MCS) was not influenced by the type of SSc or the number of clinical manifestations presented by the patient. The QoL of SSc patients was highly correlated with pain (R = 0.69) and disability (R = 0.70). Interestingly, the QoL of SSc patients was only slightly correlated with cutaneous (R = 0.42) and pulmonary involvement (R = 0.57).

Conclusion. The QoL of patients with SSc is strongly influenced by the type of SSc, the burden of clinical manifestations, the functional disability and by the pain, whatever its cause. The treatment of pain should be considered as priority to improve the QoL of SSc patients.

KEY WORDS: Systemic sclerosis, Pain, Quality of life


   Introduction
Top
 Abstract
 Introduction
Patients and methods
 Results
 Discussion
 References
 
Systemic sclerosis (SSc) is a rare debilitating disease with a heterogenous clinical pattern, variable organ involvements and degrees of severity. This disabling disease may alter the health related quality of life (QoL), depending on the extent of skin, heart, lung, kidney or digestive tract involvement. Painful symptoms of various causes [Raynaud's phenomenon (RP), digital ulcerations, oesophagitis, arthritis, etc.] may contribute to alter the QoL in this disease.

Like in other rheumatic or non-rheumatic diseases, it has been recently reported that the short-form 36 (SF-36) is an accurate tool to evaluate QoL in SSc [1] and that the QoL evaluated with this score was altered in SSc and correlated with the severity of the disease [1–3].

Therefore, the aims of this prospective multicentre observational study were to examine the impact of SSc on QoL and to determine the impact of pain in the QoL of SSc patients with either a limited or a diffuse form of SSc.


   Patients and methods
Top
 Abstract
 Introduction
 Patients and methods
Results
 Discussion
 References
 
Patients
We included 89 SSc consecutive patients, who were either hospitalized or seen at the outpatient clinics in four hospitals from March 2001 to September 2004. To be included, every patient had to fulfil the American College of Rheumatology (ACR) criteria for SSc or the criteria for limited SSc proposed by LeRoy and Medsger [4] and possess a good knowledge of French language, with sufficient capacity for abstract thought to understand the concept of visual analogue scale (VAS).

The SSc patients were defined as having diffuse SSc if they had a proximal cutaneous skin sclerosis and at least one type of visceral involvement. Otherwise, they were classified as limited.

Methods
We performed a prospective cross-sectional study. After a brief explanation of the aims of the study, each SSc patient was asked to complete two self-administered questionnaires, the French versions of the SSc health assessment questionnaire (HAQ) [5] and the SF-36 [6]. At the same time, they underwent a complete clinical examination by their referring physician. All patients gave written, informed consent.

For each patient included in the study, the following data were recorded: age, gender, type of SSc (diffuse or limited), duration of the disease and coexisting disease. Clinical and paraclinical parameters were recorded for each SSc patient as part of the information gathered during their routine follow-up.

The medical outcome study (MOS) short-form 36 (SF-36)
We measured the health-related quality of life using the ‘Medical Outcome Study (MOS) SF-36 Health Survey’. SF-36 is a 36-item self-questionnaire that evaluates the QoL within eight areas of general health: physical function (PF), role physical (RP), bodily pain (BP), general health (GH), vitality (VT), social function (SF), role emotional (RE) and mental health (MH).

Scores were calculated for each of these different aspects of health in the range of 0–100, the higher values reflecting a better QoL.

The 36 items leading to eight domain scores may also be presented in two global scores: the physical component summary score (PCS) and the mental component summary score (MCS). The French version of SF-36 was translated and validated by Leplege et al. [6] in 1998.

Scleroderma-modified HAQ (S-HAQ)
The functional repercussion of the SSc was evaluated by the SSc-modified Health Assessment Questionnaire (S-HAQ) [7]. The standard disability index of the Health Assessment Questionnaire (HAQ-DI) was initially developed in rheumatoid arthritis. This self-administered questionnaire contains 20 items (each scored 0–3), divided into eight domains of daily activity. The highest scores in each domain were added and then divided by eight to determine the HAQ-DI value, which is calculated as a continuous variable, ranging from 0 (no disability) to 3 (severe disability). A pain visual analogue scale (VAS) is also associated with the HAQ-DI [8].

When constructing the S-HAQ, Steen and Medsger [7], added five VASs to assess the functional impairment due to the following SSc symptoms RP, digital lesions, gastrointestinal symptoms, pulmonary symptoms and the overall severity of the disease, from the patient's perspective.

The S-HAQ was recently validated by our group in French language [5].

Clinical and paraclinical data collection
For each patient included in the study, clinical examination and functional evaluation were performed on the same day. Paraclinical parameters were recorded within a 3-month interval and compared with functional evaluation for each SSc patient as part of the information gathered during routine follow-up. The following data were recorded and analysed: age, gender, type of SSc (diffuse or limited), duration of the disease (defined by the time elapsed between the first symptoms of SSc and the study entry) for all patients and coexisting disease.

Patient evaluation was made from Medsger's proposed criteria [9]:

  1. Skin evaluation using the Rodnan modified total skin score [10], which was calculated as the total score from 17 body areas (right and left fingers, hands, forearms, upper arms, thighs, legs and feet plus face, anterior chest and abdomen), each body area being assessed by palpation on a 0–3 scale from uninvolved to severe thickening (range 0–51).
  2. Presence of
    1. RP crisis during the past week;
    2. digital lesions defined by pitting scars, digital tip ulceration and/or digital gangrene on examination;
    3. musculoskeletal involvement defined by myalgia, muscular weakness, peripheral arthralgia or arthritis during the previous week; finger contracture on physical examination was also noted;
    4. gastrointestinal symptoms defined by heartburn, dysphagia, episodes of pseudo-occlusion or anal incontinence during the past week;
    5. pulmonary involvement defined by carbon monoxide diffusing capacity (DLCO) <60%, as measured on pulmonary function tests;
    6. heart involvement was defined by the presence of any of the following criteria after clinical examination, EKG and cardiac ultrasound scan performed by an experienced cardiologist: a conduction defect including second- and third-degree auriculo-ventricular block, intraventricular conduction disturbance, left axis deviation, atrial or ventricular rhythm disturbance, pericarditis as defined by the presence of moderate or severe pericardial effusion >1 cm on cardiac echocardiography, left ventricular ejection fraction below 50%;
    7. pulmonary arterial hypertension was defined by pulmonary arterial pressure >35 mmHg on cardiac ultrasound scan after ruling out any other non-scleroderma related causes and
    8. renal involvement defined by serum creatinine >1.3 mg/dl or bedside urinary proteins >1+, unexplained by any other cause but SSc.

Statistical analysis
Descriptive statistics were expressed as mean ± S.D., unless otherwise stated. Student's t-tests were used to compare the continuous variables. A Chi-square test was used to analyse categorical variables. Pearson correlation coefficients were used to examine the degree of association among SF-36 scores and other continuous variables. Comparison of SF-36 scores was based on Mann–Whitney U-tests for ordinal data (i.e. absence or presence of various organ involvement) and on Wilcoxon tests for continuous data. Comparison of SF-36 scores according to the number of organ involvements was performed by ANOVA. A P-value below 0.05 was considered statistically significant.


   Results
Top
 Abstract
 Introduction
 Patients and methods
 Results
Discussion
 References
 
A total of 89 patients (71 women) answered the SF-36 and were clinically analysed at the same time [median age 51 yrs (19–77)], 67 with diffuse SSc and 22 with limited SSc. Table 1 shows the characteristics of the patients. The disability (S-HAQ) and health-related QoL (SF-36) scores are reported in Table 2.


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  		TABLE 1. Demographic data and characteristics of patients included in the study (n = 89). All the results were assessed for 89 patients except when specified (*n = 87)

 

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  		TABLE 2. Functional disability measured with the S-HAQ and health-related QoL measured with the SF-36

 
The highest correlations with the PCS of the SF-36 with the VAS of the S-HAQ values were found for the general VAS (R = 0.71) and for the pain VAS (R = 0.69) (Pearson correlation) (Table 3). Figure 1 reports high correlation between the PCS of the SF-36 and the pain VAS (R = 0.69, P < 0.0001). In comparison, although not as high, there was a significant correlation between the MCS of the SF-36 and the pain VAS (R = 0.34, P = 0.001) (Fig. 1).


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  		TABLE 3. Correlations between the PCS and the MCS of the SF-36 with the seven VAS of the S-HAQ

 

Figure 1
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  		FIG. 1. Correlation between physical component summary score (PCS) and mental component summary score (MCS) and visual analogue pain scale, n = 89. PCS: R = 0.69 (P < 0.0001). MCS: R = 0.34 (P = 0.001).

 
The PCS of the SF-36 highly correlated with the HAQ (R = 0.74, P < 0.0001), whereas the MCS correlated moderately (R = 0.35, P = 0.001) (Pearson correlation) (Table 3).

The SF-36 scores were lower in patients with diffuse than with limited SSc. The PCS of the SF-36 was statistically lower in diffuse than in limited patients SSc, respectively, 34 ± 11 vs 42 ± 10 (P < 0.05) (Fig. 2). In the presence of either RP, digital lesions, gastrointestinal, pulmonary, musculoskeletal or finger contracture, the PCS of the SF-36 was significantly lower. The MCS of the SF-36 was significantly lower only in the presence of RP or digital lesions (Table 4).


Figure 2
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  		FIG. 2. Comparison of SF-36 subscale scores in both diffuse (n = 67) and limited SSc (n = 22). Scores of each of the eight subscales of the SF-36 can vary between 0 and 100, higher values reflecting better quality of life. PF, Physical functions, RP, role limitation-physical, BP, bodily pain, GH, general health, VT, vitality, SF, social functions, RE, role limitation-emotional, MH, mental health, PCS, physical component summary score, MCS, mental component summary score. The mean value for MCS and PCS in the general population is 50 with a S.D. of 10. Bars represent SE. *P < 0.05 (t-test). PF: P = 0.009, RP: P = 0.044, BP: P = 0.024. NS for the other dimensions. PCS: P = 0.033, MCS: NS (t-test).

 

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  		TABLE 4. PCS and MCS of the SF-36 according to the presence or absence of various clinical manifestations

 
The higher was the number of SSc organ involvements, the lower were the SF-36 scores. The PCS of the SF-36 was significantly correlated with the number of clinical manifestations, with scores ranging from 51 ± 9 for the patients without any clinical involvement to 22 ± 8 with six clinical involvements (ANOVA, P < 0.0001) (Fig. 3).


Figure 3
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  		FIG. 3. Relation of physical and mental component summary scores of the MOS-SF36 with the number of clinical involvement in systemic sclerosis. Mean value of the physical component summary score and mental component summary score of the SF-36 related to the number of clinical involvement (Raynaud's phenomenon crisis during the past week, digital lesions on examination, gastro-intestinal symptoms during the past week, pulmonary involvement, musculoskeletal involvement during the previous week and finger contracture). The mean value for MCS and PCS in the general population is 50 with a S.D. of 10. Bars represent SE. PCS: P < 0.0001, MCS: P = 0.014 (ANOVA).

 
The PCS of the SF-36 was slightly correlated with the Rodnan modified total skin score (R = 0.42), as well as with the DLCO (R = 0.41). In contrast, the MCS of the SF-36 was neither correlated with the Rodnan modified total skin score nor with the DLCO (R < 0.01). The score of DLCO obtained with functional respiratory tests was highly correlated with the pulmonary VAS of the S-HAQ (R = 0.57).

There was no correlation between the age and the PCS (R = 0.04) or the MCS (R = 0.07) of the SF-36. There was no correlation between the disease duration and the scores of the PCS or the MCS of the SF-36 either, except for the mental health (MH) score which was slightly correlated with the disease duration (R = 0.26, P = 0.01). The MH was the only dimension of the SF-36, more altered in women (MH = 53 ± 19) than in men (MH = 64 ± 24) (P < 0.05).


   Discussion
Top
 Abstract
 Introduction
 Patients and methods
 Results
 Discussion
References
 
The present multicenter prospective study, performed on a larger cohort, of 89 SSc patients with both diffuse (n = 67) and limited (n = 22) forms of the disease, than the previous similar studies in SSc patients [2, 3, 11], confirmed that the QoL is diminished in SSc. All the SF-36 subscale scores of SSc patients were lower than those already reported in general population [6], as shown in Table 5. Results were influenced by the type of SSc and both PCS and MCS of the SF-36 were lower in diffuse than in limited SSc, which could be related to the disease extension. Such a result was not found by Del Rosso et al. [2], who observed a lower mental score in 9 diffuse and 15 limited SSc. Differences in the total number of patients and the respective ratio of localized and diffuse forms could explain the different findings.


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  		TABLE 5. Results of SF-36 scores of our study in comparison with three other SSc studies (Danieli [3], Del Rosso [2], Khanna [13]) and with normative data in general (US and French) population (Ware, Leplege [6])

 
The QoL as assessed by the SF-36 appeared related to the burden of clinical manifestations, in relation to the number of clinical involvements, the functional disability and the pain, whatever may be its cause. We found a high correlation between pain and health-related QoL in SSc. Comparatively, the respective correlations between the cutaneous involvement and pulmonary function and QoL were moderate. Therefore, although it might be a truism for any clinician, the effective control of the pain, which can be related to the presence of either RP, digital ulcerations, musculoskeletal or digestive involvement should constitute the primary therapeutic goal to achieve a better QoL in SSc-treated patients.

Our study carries a few limitations. One could argue that the correlation between pain and the PCS of the SF-36 is directly related to the item ‘bodily pain’, which constitutes one of the eight SF-36 domains. However, the correlation between the pain VAS and the PCS of the SF-36 was one of the highest correlations observed in the present study. Moreover, it should be pointed out that the correlations between pain VAS and each domain of the SF-36 were high, not only with bodily pain (BP) domain (R = 0.71, P < 0.0001), but also with physical functionning (PF), role physical (RP) and general health (GH), respectively, R = 0.58, 0.54 and 0.57 (P < 0.0001).

This important finding for clinical practice appears as a reminder of the importance of pain for the clinician in charge of treating the numerous symptoms observed in SSc.

Although the number of patients in our study did not allow to perform a multivariate analysis of the various determinants of health-related QoL in SSc, the present work included the largest number of patients with diffuse SSc studied today with the primary aim to evaluate SSc QoL. Their scores were globally quite lower than those reported by Danieli et al. [3] and Del Rosso et al. [2] probably due to more severe forms of the disease, since 12 patients in our study were included in the Intensification et Autogreffe dans les Maladies Auto-Immunes Résistantes (ISAMAIR) study [12] and treated by haematopoiteic stem cell transplantation for rapidly progressive diffuse SSc resistant to prior treatment. In the study by Khanna et al. [13], 239 patients were included with the primary aim to test the potential benefit from relaxin treatment in SSc patients, and the evaluation of SSc patients functional repercussion and QoL were not the primary end point of this work [13]. Moreover, unlike in this study, we chose to present the results of each eight subscales of SF-36, which are of interest to precise the domains where QoL is altered in SSc patients.

In conclusion, altered QoL in SSc is a major therapeutic challenge for the clinician. The benefits in using self-questionnaires’ analysis are important in daily clinical practice since they appear to have a good correlation with the disease severity. Our study yields to point out the impact of pain in health-related QoL of patients with SSc.

The authors have declared no conflicts of interest.


   References
Top
 Abstract
 Introduction
 Patients and methods
 Results
 Discussion
 References
 

  1. Georges C, Chassany O, Mouthon L, et al. (2004) Quality of life assessment with the MOS-SF36 in patients with systemic sclerosis. Rev Med Interne 25:16–21 (French).[Web of Science][Medline]
  2. Del Rosso A, Boldrini M, D'Agostino D, et al. (2004) Health-related quality of life in systemic sclerosis as measured by the Short Form 36: relationship with clinical and biologic markers. Arthritis Rheum 51:475–81.[CrossRef][Web of Science][Medline]
  3. Danieli E, Airo P, Bettoni L, et al. (2005) Health-related quality of life measured by the Short Form 36 (SF-36) in systemic sclerosis: correlations with indexes of disease activity and severity, disability, and depressive symptoms. Clin Rheumatol 24:48–54.[CrossRef][Web of Science][Medline]
  4. LeRoy EC and Medsger TA Jr. (2001) Criteria for the classification of early sclerosis. J Rheumatol 28:1573–6.[Abstract/Free Full Text]
  5. Georges C, Chassany O, Mouthon L, et al. (2005) Validation of French version of the Scleroderma Health Assessment Questionnaire (SSc HAQ). Clin Rheumatol 24:3–10.[CrossRef][Web of Science][Medline]
  6. Leplege A, Ecosse E, Verdier A, Perneger TV. (1998) The French SF-36 Health Survey: translation, cultural adaptation and preliminary psychometric evaluation. J Clin Epidemiol 51:1013–23.[CrossRef][Web of Science][Medline]
  7. Steen VD and Medsger TA. (1997) The value of the health assessment questionnaire and special patient-generated scales to demonstrate change in systemic sclerosis patients over time. Arthritis Rheum 40:1984–91.[Web of Science][Medline]
  8. Fries JF, Spitz PW, Young DY. (1982) The dimensions of health outcomes: the health assessment questionnaire, disability and pain scales. J Rheumatol 9:789–93.[Web of Science][Medline]
  9. Medsger T, Silman A, Steen V, et al. (1999) A disease severity scale for systemic sclerosis: development and testing. J Rheumatol 26:2159–67.[Web of Science][Medline]
  10. Clements Ph, Lachenbruch P, Siebold J, et al. (1995) Inter and intraobserver variability of total skin thickness score (modified Rodnan TSS) in systemic sclerosis. J Rheumatol 22:1281–5.[Web of Science][Medline]
  11. Cossutta R, Zeni S, Soldi A, Colombelli P, Belotti Masserini A, Fantini F. (2002) Evaluation of quality of life in patients with systemic sclerosis by the SF-36 questionnaire. Reumatismo 54:122–7 (Italian).[Medline]
  12. Farge D, Marolleau JP, Zohar S, et al. (2002) Autologous bone marrow transplantation in the treatment of refractory systemic sclerosis. Br J Haematol 119:729–39.
  13. Khanna D, Furst DE, Clements PJ, et al. (2005) Responsiveness of the SF-36 and the Health Assessment Questionnaire Disability Index in a Systemic Sclerosis Clinical Trial. J Rheumatol 32:832–40.[Abstract/Free Full Text]
  14. Ware JE Jr. (1993) SF-36 health survey: manual and interpretation guide. Boston: Health Institute, New England Medical Center.
Submitted 7 February 2006; revised version accepted 25 April 2006.
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