Impact of systemic sclerosis on occupational and professional activity with attention to patients with digital ulcers

Authors


Abstract

Objective

To evaluate the impact of systemic sclerosis (SSc; scleroderma) and digital ulcers (DUs) on daily living and professional activities.

Methods

We prospectively evaluated 189 SSc patients for employment status and disability during meetings of the French SSc patient association (n = 86, 45.5%) or during hospitalization (n = 103, 54.5%).

Results

Seventy-eight (41.2%) patients had diffuse SSc. The mean ± SD age was 54 ± 13 years, and the mean ± SD disease duration was 9.3 ± 8.4 years at the time of evaluation. Sixty (31.7%) patients had at least one DU. Assessed using the Health Assessment Questionnaire (mean ± SD 1.12 ± 0.79 versus 1.39 ± 0.84; P = 0.001), the Cochin Hand Function Scale (mean ± SD 20.2 ± 18.3 versus 27.8 ± 19.1; P < 0.0001), and the Hospital Anxiety Scale (mean ± SD 9.9 ± 5 versus 8.5 ± 4.2; P = 0.04), global disability, hand disability, and anxiety, respectively, were significantly higher in patients with DUs than in others. Most patients reported a limitation in daily activities related to SSc, as assessed by a daily activity limitation scale (mean ± SD 4.4 ± 2.9) and an increased need for help in the home. Patients reported needing mean ± SD 4 ± 13.5 hours per month of paid household help related to SSc and mean ± SD 1.5 ± 10 hours per month related to DUs, with significant differences between patients with or without DUs (P = 0.004). Among the 113 patients in the workforce, 67 (59.3%) were employed, 42 (37.2%) were employed full time, 36 (31.8%) received full disability pension, and 27 (23.9%) were on sick leave, with no difference between patients with or without DUs.

Conclusion

SSc has a significant impact on activities of daily living and work disability. The need for external home help and disability are increased for those patients with DUs.

INTRODUCTION

Systemic sclerosis (SSc; scleroderma) is a connective tissue disease characterized by excessive collagen deposition in the dermis and internal organs, vascular hyperreactivity (1), and diminished life expectancy (2). SSc is also responsible for tendon, joint, and vessel damage and leads to disability, handicap, and worsened health-related quality of life (HRQOL) (3). Hand involvement, responsible for marked disability in SSc (4), can be assessed using the Cochin Hand Function Scale (CHFS) (3, 5).

Digital ulcers (DUs) occur in 30–58% of patients with SSc (6–8). DUs may become infected, gangrene may occur, and amputation of fingers may be necessary (6). In addition, recurrent DUs may be responsible for pain and tissue loss. We recently reported that SSc patients with DUs have reduced wrist and hand mobility, increased global and hand disabilities, and altered mental HRQOL as compared with those without DUs (9).

Work ability and influencing factors have rarely been studied in SSc. In a recent study, we reported that patients with SSc commonly have to change jobs and go on full-time sick leave. In addition, we found that hand function was significantly altered in patients on sick leave (5, 10). Despite the availability of disability pensions, the socioeconomic burden seems to be considerable (10). However, the number of patients included in this study was limited, and we focused on professional and not on daily activity.

To better characterize the impact of SSc on daily living and professional activities, we conducted a prospective study. Since few data are available on the impact of DUs on these activities in SSc patients, we focused on this subgroup of patients with DUs.

PATIENTS AND METHODS

Study design.

Between January 2008 and June 2009, 189 patients with SSc were prospectively included during annual meetings of the French SSc patient association, the Association des Sclérodermiques de France (ASF) (n = 86), or during their hospitalization in the internal medicine departments of Cochin (n = 87) or Claude Huriez (n = 16) hospitals. Data were collected on employment status, socioeconomic burden, disability, and HRQOL. To check for completed questionnaires for all patients and to gather clinical data in the case of patients from the ASF, patients had to complete self-administered questionnaires first and then undergo an interview with a physician.

Ethical considerations.

This survey was conducted in compliance with the Good Clinical Practices protocol and Declaration of Helsinki principles. In accordance with French law, a formal approval from an ethical committee is not required for this kind of project. Patients gave their consent to participate after being informed orally about the study protocol.

Patients.

To be eligible for the study, patients had to fulfill the American College of Rheumatology criteria (11) and/or the LeRoy and Medsger criteria (12) for SSc. Patient members of the ASF were asked to participate during 1 of 2 annual meetings held in the spring in 2008 and 2009. Demographic parameters recorded were age; sex; ethnicity; year of onset of Raynaud's phenomenon; age at diagnosis; year of onset of the first non–Raynaud's phenomenon symptom; disease duration; disease form, i.e., limited SSc (with no detectable skin involvement) (12), limited cutaneous SSc (lcSSc; with skin involvement essentially limited to the hands and face), and diffuse cutaneous SSc (dcSSc; with proximal skin involvement) (13); interincisor distance (measured in millimeters); dyspnea (assessed by the New York Heart Association 4-point scale); pitting scars; DUs; calcinosis; history of esophagus, gastrointestinal, joint and/or muscle, heart involvement, and interstitial lung disease; pulmonary arterial hypertension; renal crisis; and physical therapy. Evidence of esophagus, joint and/or muscle involvement, heart involvement, interstitial lung disease, pulmonary arterial hypertension, and scleroderma renal crisis was based on patient reports during interviews for patients evaluated at the association meetings and on clinical charts for patients evaluated during hospitalization.

Health status and HRQOL.

Health status was assessed by the Karnofsky Performance Status (KPS) score, which ranged from 0 (death) to 100 (normal, no symptoms, and no evidence of disease) (14). HRQOL was assessed by the French version of the Medical Outcomes Study 36-Item Short Form Health Survey (SF-36) (15). This self-administered questionnaire covers 8 areas: physical functioning, physical role, bodily pain, general health perception, mental health, vitality, emotional role, and social functioning. For each area, the score ranges from 0 (poorer health status) to 100 (better health status). These scores can also be summarized in 2 global scores: the physical component score (PCS) and the mental component score.

Disability.

Global disability.

Global disability was assessed by use of the standard disability index of the Health Assessment Questionnaire (HAQ) that contains 20 items divided into 8 domains with each score ranging from 0 (no disability) to 3 (maximal disability) (16). Organ-specific disability was assessed by the 5 scleroderma visual analog scales (VAS; for Raynaud's phenomenon, digital tip ulcers, gastrointestinal symptoms, pulmonary symptoms, and overall disease severity from the patient's perspective) of the scleroderma-modified HAQ (17). Each VAS was scored on a 15-cm line that is converted to a continuous scale from 0–3 (1 cm = 0.2 points on the VAS).

Hand disability.

Hand disability was assessed by the CHFS (18), a self-administered questionnaire that contains 18 items related to daily activities. Each question is scored on a scale from 0 (performed without difficulty) to 5 (impossible to do). The total score is obtained by adding the scores of all items (range 0–90).

Patients' perceived disability.

Patients' perceived disability was assessed by the McMaster Toronto Arthritis Patient Preference Disability Questionnaire (MACTAR) (19). Patients were asked to select the 3 situations among activities of daily living (ADL) that caused them maximal trouble (14). Each item is scored on an 11-point semiquantitative scale (range 0–10). The global score ranges from 0 (no disability) to 30 (maximal disability). This scale has been validated in SSc (14). To classify the activities identified by patients, we used the domains of the International Classification of Functioning, Disability, and Health (ICF) (20) with the 10 linking rules given by the World Health Assembly in May 2001. According to these rules, each item of an activity should be linked to the most precise ICF category, and if concepts refer to more than one ICF category, then all the ICF categories to which the concepts refer should be linked (21), as was previously reported (22).

Anxiety and depression.

Anxiety and depression were assessed by the Hospital Anxiety and Depression Scale (HADS). This scale has 7 questions for the anxiety dimension (HADSa) and 7 for the depression dimension (HADSd) (23). Each point is scored on a scale of 0–3, and the total score ranges from 0 (no depression, no anxiety) to 21 (maximal depression, maximal anxiety).

Employment status.

For work status, we asked the patients to provide us with the following information regarding their employment status: employed, retired, homemaker, student, disabled, on sick leave, or looking for a job. Data were also collected on work duration (full time or part time), occupational changes since the diagnosis of SSc was made, and participation in voluntary work or associations. Patients were considered for work analysis if they were age >18 years and <61 years. Four students and 7 housewives were excluded from the employment status analysis. Finally, 113 patients were considered for the study of risk factors of work disability. Patients were considered disabled if they were currently disabled or on sick leave because of SSc. Decreased productivity at work during the previous month due to SSc and to DUs was scored on two 11-point semiquantitative scales from 0 (no decrease) to 10 (major decrease). In addition, patients were asked for their feeling of decreased income, lack of promotion, and discrimination using questions with “Yes” or “No” answers.

Daily activity limitations.

Daily activity limitations related to SSc and to DUs were scored on two 11-point semiquantitative scales (0–10), where 0 means no limitation in daily activity and 10 means major limitations in daily activity. Patients were asked to report if during the previous month they had been unable to perform their household tasks because of SSc and/or DUs. If patients answered yes, they were asked to indicate if they needed paid or nonpaid help to perform household tasks and the corresponding number of hours per week.

Environmental factors.

Patients were asked to complete a questionnaire regarding their current and past smoking history. The questionnaire was adapted from the National Health Interview Survey (24). Subjects were considered current smokers if they reported having previously smoked at least 100 cigarettes during their lifetime and were still smoking at least 1 cigarette per week. Never-smokers were defined as subjects who had smoked <100 cigarettes during their lifetime. Former smokers reported smoking at least 100 cigarettes ever and currently smoking <1 cigarette per week. Information was collected on professional exposure to toxins and solvents: silica, dust, silicon, vinyl chloride, welding fumes, organic solvents, epoxy resins, or pesticides.

DU status.

Patients self-reported their DU history. Data on pitting scars were collected by physicians in ASF patients, as well as in hospitalized patients. Pitting scars and DUs were also checked upon physical examination in both the patients evaluated at the association meetings and the patients evaluated during hospitalization. Current DUs were defined as loss of surface epithelialization at the time of enrollment. The investigators noted the number of DUs at the time of examination and categorized the DUs according to the possible mechanism: ischemic for fingertip DUs, mechanical for DUs occurring above the bone relief (referred to as DUs proximal to the fingertip), and DUs associated with calcinosis.

Statistical analysis.

Results are expressed as numbers and/or percentages for categorical variables and as means ± SD or median (interquartile range) for continuous variables, unless stated otherwise. To identify parameters associated with DUs and work disability, demographic, clinical, and functional characteristics were compared between patients with or without DUs and between disabled and nondisabled patients. Univariate analyses compared qualitative variables using a chi-square test or, when appropriate, Fisher's exact test, and quantitative variables were compared using Student's t-test. Multivariate analyses were then conducted to determine independent risk factors associated with DUs and the variables associated with work disability status. For all multivariate analyses, variables with P values less than 0.20 on univariate analysis were entered into the model and selected using a stepwise regression. For all statistical analyses, P values less than or equal to 0.05 were considered significant. Statistical analyses involved use of SAS software, version 9.1.

RESULTS

Demographic and clinical data.

One hundred eighty-nine patients were included: 86 (45.5%) patients who were ASF members and enrolled during ASF annual meetings and 103 (54.5%) patients who were evaluated during hospitalization. Patients who were ASF members and included in the study during annual meetings were not included during hospitalization. Demographic and clinical parameters for the 189 patients at baseline evaluation are listed in Table 1.

Table 1. Demographic and clinical characteristics, environmental factors, and treatments in 189 patients with SSc*
ParametersWhole group (n = 189)DUs (n = 60)No DUs (n = 129)Univariate analysisMultivariate analysis
PPOR (95% CI)
  • *

    Values are the number (percentage) unless otherwise indicated. SSc = systemic sclerosis; DU = digital ulcer; OR = odds ratio; 95% CI = 95% confidence interval; NYHA = New York Heart Association.

Tertiary care patients103 (54.5)38 (36.9)65 (63.1)   
Patient association members86 (45.5)22 (25.6)64 (74.4)   
Demographic characteristics      
 Age at evaluation, mean ± SD years54.1 ± 13.353.6 ± 15.454.3 ± 12.20.74  
 Age at onset of SSc, mean ± SD years44.2 ± 13.742.4 ± 1645 ± 12.40.23  
 Women164 (86.8)51 (85)113 (87.6)0.65  
 Ethnicity   0.83  
  White164 (87.7)53 (89.8)111 (86.7)   
  African American15 (7.9)4 (6.8)11 (8.6)   
  Asian8 (4.3)2 (3.4)6 (4.7)   
Disease form, no./total (%)   0.01  
 Diffuse cutaneous SSc78/179 (43.6)32/54 (59.3)46/125 (36.8)   
 Limited cutaneous SSc76/179 (42.5)18/54 (33.3)58/125 (46.4)   
 Limited SSc25/179 (13.9)4/54 (7.4)21/125 (16.8)   
SSc disease duration, mean ± SD years9.3 ± 8.49.7 ± 99.2 ± 8.20.74  
SSc disease duration at first DU, mean ± SD years3.2 ± 4.63.7 ± 5.22.4 ± 3.90.2  
Telangiectasias129 (68.2)44 (73.3)85 (65.9)0.30  
Interincisor distance, mean ± SD mm35 ± 7.934.9 ± 835.4 ± 7.90.71  
Raynaud's phenomenon185 (97.9)60 (100)125 (96.9)0.68  
History of DUs113 (59.8)54 (90)59 (45.7)< 0.0001  
Pitting scars105 (55.5)55 (91.7)50 (38.7)< 0.0001< 0.000120.0 (6.5–62.1)
Calcinosis55 (29.1)24 (40)31 (24)0.02  
Esophagus/gastrointestinal tract involvement143 (75.6)45 (75)98 (75.9)0.88  
Arthralgias120 (63.5)45 (75)75 (58.1)0.020.0053.8 (1.5–9.4)
Myalgias96 (50.8)35 (58.3)61 (47.3)0.15  
Dyspnea, mean ± SD NYHA classified2.1 ± 0.82.1 ± 0.92.2 ± 0.840.63  
Interstitial lung disease84 (44.4)29 (48.3)55 (42.6)0.46  
Pulmonary arterial hypertension19 (10)8 (13.3)11 (8.5)0.30  
Scleroderma renal crisis17 (9)5 (8.3)12 (9.3)0.83  
Exposure, no./total (%)      
 Current smoker15/187 (8)4/59 (6.7)11/128 (8.6)0.44  
 Former smoker45/187 (24)11/59 (18.6)18/128 (13.9)0.44  
 Professional cold exposure22/189 (11.6)6/60 (10)16/129 (4.6)0.63  
 Professional toxins/solvents exposure36/189 (19)10/60 (16.6)26/129 (20.1)0.73  
Treatments, no./total (%)      
 Calcium-channel blockers136/187 (72.7)48/60 (80)88/127 (69.3)0.12  
 Iloprost, current DU episode13/186 (7)11/60 (18.3)2/126 (1.6)< 0.0001  
 Iloprost, prior DU episode19/186 (10.2)13/60 (21.7)6/126 (4.8)0.0003  
Bosentan, no./total (%)24/187 (12.8)12/60 (20)12/127 (9.4)0.04  
Current physiotherapy, no./total (%)61/180 (33.8)25/59 (42.3)36/121 (29.7)0.09  
History of physiotherapy, no./total (%)95/186 (51)34/60 (56.6)61/126 (48.4)0.29  

Functional impact of SSc.

Overall, the level of global disability and hand disability of the SSc patients was high, with mean ± SD HAQ and CHFS scores of 1.12 ± 0.8 and 20.2 ± 18.3, respectively (Table 2). SSc patients displayed a significantly increased anxiety level (mean ± SD HADSa 8.9 ± 4.5) and a tendency toward depression (mean ± SD HADSd 6.3 ± 4.2).

Table 2. Functional impact of DUs in 189 patients with systemic sclerosis*
ParametersWhole group (n = 189)DUs (n = 60)No DUs (n = 129)Univariate analysisMultivariate analysis
PPOR (95% CI)
  • *

    Values are the mean ± SD unless otherwise indicated. DU = digital ulcer; OR = odds ratio; 95% CI = 95% confidence interval; KPS = Karnofsky performance status; HAQ = Health Assessment Questionnaire; VAS = visual analog scale; MACTAR = McMaster Toronto Arthritis Patient Preference Disability Questionnaire; HADS = Hospital Anxiety and Depression Scale; PCS = physical component score; MCS = mental component score.

KPS (range 0–100)76.3 ± 11.874.2 ± 10.977.4 ± 2.10.09  
HAQ (range 0–3)1.12 ± 0.81.4 ± 0.841 ± 0.740.001  
Raynaud's phenomenon (VAS, range 0–3)0.94 ± 0.91.2 ± 10.8 ± 0.80.0070.051.55 (0.9–2.4)
DU symptoms (VAS, range 0–3)0.6 ± 0.91.3 ± 10.3 ± 0.6< 0.0001  
Gastrointestinal symptoms (VAS, range 0–3)0.8 ± 0.91 ± 10.8 ± 10.33  
Pulmonary symptoms (VAS, range 0–3)0.7 ± 0.930.9 ± 1.10.6 ± 0.80.02  
Overall disease (VAS, range 0–3)1.1 ± 0.91.3 ± 11.1 ± 0.90.14  
Perceived individualized handicap (MACTAR, range 0–30)19 ± 7.420.3 ± 6.818.4 ± 7.70.13  
Cochin Hand Function Scale (range 0–90)20.2 ± 18.327.8 ± 19.116.7 ± 17< 0.0001  
Depression HADS (range 0–21)6.3 ± 4.26 ± 4.66 ± 40.18  
Anxiety HADS (range 0–21)8.9 ± 4.510 ± 58.5 ± 4.20.04  
Short Form 36 health survey PCS34.2 ± 7.533.2 ± 8.334.7 ± 7.00.21  
Short Form 36 health survey MCS27.3 ± 8.725.9 ± 8.228 ± 90.13  

MACTAR results were available for 183 patients. The perceived mean ± SD disability score was 19 ± 7.4. Overall, patients cited 38 different items classified according to the ICF activities corresponding to the 8 ICF domains. Fourteen (7.6%) patients cited only 2 activities, 6 (3.3%) patients only 1 activity, and 9 (4.9%) cited “any activity.” For the 3 main activities selected, the ICF domains were community, social, and civic life (17 activities, cited 177 times, 96.7% of patients), domestic life (7 activities, cited 150 times, 81.9% of patients), mobility (10 activities, cited 124 times, 67.7% of patients), self-care (3 activities, cited 33 times, 18% of patients), major life areas (1 activity, cited 32 times, 17.5% of patients), learning and applying knowledge (1 activity, cited 2 times, 1.1% of patients), interpersonal interactions and relationships (1 activity, cited 2 times, 1.1% of patients), and communication (1 activity, cited 5 times, 2.7% of patients).

Considering the first priority, the 5 activities cited most often as being affected by disability were sports activities (n = 34, 18.6% of participants), cleaning living area (n = 32, 17.5%), cooking (n = 24, 13.1%), work and employment (n = 13, 7.1%), and walking (n = 11, 6%).

Impact of SSc on ADL.

SSc was associated with decreased performance in ADL (Table 3). The mean ± SD daily activity limitation related to SSc was 4.4 ± 2.9 (range 0–10), and the daily activity limitation related to DUs was 2.1 ± 3. This limitation related to SSc led to a need for help to perform home tasks for 87 (46.7%) patients. Therefore, patients reported needing mean ± SD 4 ± 13.5 paid hours and 9 ± 27 unpaid hours of household help related to SSc. Participation in voluntary work or associations was reported by 15 (7.9%) and 96 (50.8%) patients, respectively.

Table 3. Impact of SSc and DUs on activities of daily living*
ParametersWhole group (n = 189)DUs (n = 60)No DUs (n = 129)P
  • *

    Values are the mean ± SD unless otherwise indicated. SSc = systemic sclerosis; DU = digital ulcer.

Daily activity limitation, range 0–10    
 SSc-related4.4 ± 2.95.3 ± 3.054.05 ± 2.740.006
 DU-related2.1 ± 34.8 ± 3.340.8 ± 1.82< 0.0001
Need help for home tasks, no./total (%)87/186 (46.7)39/59 (66.1)48/127 (37.8)< 0.0001
 SSc-related88/184 (47.8)40/59 (67.8)48/125 (38.4)< 0.0001
 DU-related41/182 (22.5)31/57 (54.4)10/125 (8)< 0.0001
Household help, hours    
 SSc-related paid4 ± 13.58 ± 21.52 ± 6.30.004
 DU-related paid1.5 ± 103.8 ± 16.50.4 ± 2.460.03
 SSc-related nonpaid9 ± 2718.7 ± 40.84 ± 150.001
 DU-related nonpaid5.5 ± 2215 ± 361 ± 8< 0.0001

Impact of SSc on professional activity.

A total of 76 patients were not part of the workforce (7 housewives, 4 students, and 65 retired patients) at study entry. Among the 113 patients in the workforce, 67 (59.3%) were employed, including 42 (37.2%) with full-time employment and 23 (20.3%) with part-time employment. Two (1.7%) patients did not mention whether they had full-time or part-time employment. Among patients in the workforce, 39 (34.5%) had changed their profession since the diagnosis of SSc.

Patients in the workforce worked, on average, mean ± SD 23 ± 17 hours per week (Table 4). Thirty-six patients (31.8%) were receiving a full disability pension and 27 (23.9%) were on sick leave at the time of inclusion. Seven (6.2%) patients had retired early due to SSc. The socioeconomic burden related to SSc was important, with decreased income, lack of promotion, and a feeling of discrimination at work for 46.9%, 43.3%, and 10.6% of patients, respectively. As compared with disabled patients, workers were younger (mean ± SD age 45 ± 9.4 years versus 52 ± 6.9 years; P < 0.0001), had a shorter mean ± SD disease duration (6.1 ± 5.2 years versus 9.5 ± 7.2 years; P = 0.005), lower global disability (mean ± SD HAQ score 0.8 ± 0.7 versus 1.5 ± 0.75; P < 0.0001) and hand disability (mean ± SD CHFS score 15.4 ± 17 versus 24 ± 18; P = 0.007), lower mean ± SD MACTAR score (18 ± 8.5 versus 22.5 ± 4; P = 0.002), increased mean ± SD KPS score (79 ± 14 versus 71 ± 9.5; P = 0.001), and a better quality of life as assessed by the SF-36 PCS (mean ± SD 36.4 ± 7.4 versus 31.4 ± 6.8; P = 0.001) (Table 5). On multivariate analysis, age (adjusted odds ratio [aOR] 1.1, 95% confidence interval [95% CI] 1.05–1.2), disease duration (aOR 1.09, 95% CI 1.01–1.2), HAQ score (aOR 6.1, 95% CI 2.2–16.7), and CHFS (aOR 0.97, 95% CI 0.93–1) were identified as independent risk factors for work disability.

Table 4. Working status of 113 patients with SSc in the workforce*
ParametersWhole group (n = 113)
  • *

    Values are the number (percentage) unless otherwise indicated. SSc = systemic sclerosis; DU = digital ulcer.

Employment67 (59.3)
 Full time42 (37.2)
 Part time23 (20.3)
 Unspecified2 (1.7)
Physical job21 (18.6)
Working hours/week, mean ± SD23 ± 17
Full disability pension36 (31.8)
Duration of disability pension, mean ± SD years5.6 ± 4.4
Sick leave at time of inclusion, no./total (%)27/113 (23.9)
 SSc-related23/27 (85.2)
 DU-related1/27 (3.7)
 Related to both3/27 (11.1)
Duration of sick leave, mean ± SD weeks3.4 ± 4.6
Early retirement due to SSc7 (6.2)
Decreased work productivity 
 SSc-related (range 0–10), mean ± SD3.4 ± 3.8
 DU-related (range 0–10), mean ± SD1.4 ± 3.1
Decreased income53 (46.9)
Lack of promotion49 (43.3)
Feeling of discrimination12 (10.6)
Table 5. Demographic, clinical, and functional characteristics of SSc patients in the workforce*
VariableWhole group (n = 113)Workers (n = 67)Work disabled, nonworkers (n = 46)Univariate analysisMultivariate analysis
PPOR (95% CI)
  • *

    Values are the number (percentage) unless otherwise indicated. SSc = systemic sclerosis; OR = odds ratio; 95% CI = 95% confidence interval; DU = digital ulcer; HAQ = Health Assessment Questionnaire; MACTAR = McMaster Toronto Arthritis Patient Preference Disability Questionnaire; CHFS = Cochin Hand Function Scale; HADS = Hospital Anxiety and Depression Scale; PCS = physical component score; MCS = mental component score.

Recruitment   0.31  
 Tertiary care63 (55.7)41 (61.2)22 (47.8)   
 Patient association members50 (44.4)26 (38.8)24 (52.2)   
Age at evaluation, mean ± SD years47.9 ± 945 ± 9.452 ± 6.9< 0.00010.00061.1 (1.05–1.2)
Age at SSc onset, mean ± SD years40.5 ± 9.939 ± 10.142 ± 9.50.09  
Women96 (84.9)59 (88)37 (80.4)0.26  
SSc duration, mean ± SD years7.6 ± 6.36.1 ± 5.29.5 ± 7.20.0050.031.09 (1.01–1.2)
Diffuse SSc51 (45.1)27 (40.3)24 (52.2)0.28  
Raynaud's phenomenon113 (100)67 (100)46 (100)0.23  
Pitting scars62 (54.9)38 (56.7)24 (52.2)0.51  
History of DUs68 (60.2)42 (62.7)26 (56.5)0.39  
Current DUs30 (26.5)18 (26.9)12 (26.1)0.93  
Calcinosis21 (18.6)12 (17.9)9 (19.6)0.82  
Arthralgias74 (65.5)40 (59.7)34 (73.9)0.12  
Myalgias55 (48.7)29 (43.3)26 (56.5)0.17  
Interstitial lung disease55 (48.7)28 (41.8)27 (58.7)0.08  
Pulmonary arterial hypertension9 (7.9)5 (7.4)4 (8.7)0.85  
Scleroderma renal crisis8 (7.1)6 (8.9)2 (4.3)0.34  
Karnofsky performance status, mean ± SD75.8 ± 12.779 ± 1471 ± 9.50.001  
HAQ score (range 0–3), mean ± SD1.1 ± 0.80.8 ± 0.71.5 ± 0.75< 0.00010.00056.1 (2.2–16.7)
Perceived individualized handicap (MACTAR, range 0–30)20 ± 7.318 ± 8.522.5 ± 40.002  
CHFS (range 0–90), mean ± SD19 ± 17.615.4 ± 1724 ± 180.0070.130.97 (0.93–1)
Depression HADS (range 0–21), mean ± SD6.3 ± 45.8 ± 4.17 ± 3.90.12  
Anxiety HADS (range 0–21), mean ± SD8.6 ± 4.48.8 ± 4.68.3 ± 4.20.6  
Short Form 36 health survey PCS, mean ± SD34.4 ± 7.536.4 ± 7.431.4 ± 6.80.001  
Short Form 36 health survey MCS, mean ± SD27 ± 9.227 ± 9.326.4 ± 90.72  

Patients with DUs.

In total, 113 (59.8%) patients reported a previous episode of DU, and 105 (55.5%) patients had at least one pitting scar at the time of evaluation. Sixty patients had ≥1 DU at the time of evaluation, for a total of 221 DUs (Table 6). Among these 60 patients, only 6 had no previous history of DUs. Twenty patients (33.3%) had one DU, 40 (66.7%) had >1 DU, and 24 (40%) had ≥4 DUs at the time of evaluation. A total of 54 patients (90%) had ≥1 fingertip DU. Twelve (20%) patients had ≥1 DU associated with calcinosis, 15 (25%) patients had mechanical DUs proximal to the fingertip, and 16 (26.6%) had ≥1 DU resulting from more than one mechanism. On univariate analysis, patients with DUs (n = 60) more frequently had dcSSc (P = 0.01), a history of DUs (P < 0.0001), pitting scars (P < 0.0001), arthralgias (P = 0.02), and calcinosis (P = 0.02) than patients without DUs (Table 1). Multivariate analyses identified pitting scars (aOR 20.0, 95% CI 6.5–62.1), arthralgias (aOR 3.8, 95% CI 1.5–9.4), and severity of Raynaud's phenomenon (aOR 1.55, 95% CI 0.9–2.4) as independent factors associated with DUs.

Table 6. Characteristics of DUs in patients with systemic sclerosis*
  • *

    Values are the number (percentage) unless otherwise indicated. DU = digital ulcer.

DU, no.221
Patients having ≥1 DU60 (31.7)
 1 DU20 (33.3)
 Multiple DUs40 (66.7)
 ≥4 DUs24 (40)
DU mechanisms 
 Ischemic DU54 (90)
 DU complicating calcinosis12 (20)
 Mechanical DU15 (25)
 DU related to >1 mechanism16 (26.6)

Functional impact of DUs.

Patients with DUs had a significantly higher mean ± SD HAQ score (1.4 ± 0.84 versus 1.0 ± 0.74; P = 0.001), mean ± SD HAQ Raynaud's phenomenon (1.2 ± 1.0 versus 0.8 ± 0.8; P = 0.007), mean ± SD HAQ pulmonary symptoms (0.9 ± 1.1 versus 0.6 ± 0.8; P = 0.02), and mean ± SD CHFS score (27.8 ± 19.1 versus 16.7 ± 17; P < 0.0001) than patients without DUs. Patients with DUs experienced higher levels of anxiety than did patients without DUs on the HADSa scale (mean ± SD 10 ± 5 versus 8.5 ± 4.2; P = 0.04). Analysis of SF-36 subscales showed impaired mental health (mean ± SD 11.5 ± 3.4 versus 13.1 ± 3.5; P = 0.004) and higher bodily pain (mean ± SD 3.05 ± 1.38 versus 3.5 ± 1.3; P = 0.05) in patients with than without DUs, whereas no significant difference was observed for other subscales (data not shown).

Impact of DUs on daily living and professional activities.

DUs were associated with decreased performance in ADL (Table 3). The mean ± SD limitation related to DUs was 4.8 ± 3.34 (range 0–10). This limitation induced a need for help in the completion of home tasks for 39 (66.1%) patients, with a mean ± SD 3.8 ± 16.5 and 15 ± 36 hours per month of paid and nonpaid household help, respectively.

Limitation in daily activities related to SSc was higher in patients with than without DUs (mean ± SD 5.3 ± 3.05 versus 4.05 ± 2.74; P = 0.006). In addition, patients with DUs needed more external home help (66.1% versus 37.8%; P < 0.0001), as well as more paid (mean ± SD 8 ± 21.5 versus 2 ± 6.3 hours per month; P = 0.004) and nonpaid household help (mean ± SD 18.7 ± 40.8 versus 4 ± 15 hours per month; P = 0.001) related to SSc. Decreased productivity at work due to DUs was estimated at mean ± SD 1.4 ± 3.1 (range 0–10). No significant difference was observed for work status between patients with or without DUs.

DISCUSSION

In the present study, we provide evidence that SSc has a significant impact on ADL and work disability, and more than half of those in the workforce were on sick leave or full disability pension. To our knowledge, only 2 studies from the same group have focused on ADL capacity and performance of work in SSc patients (25, 26). In a cohort of female patients, including 73% with lcSSc, activities requiring hand and/or arm function were more difficult to perform than those depending on lower extremity function (25). In another study of 43 patients with early SSc, including 31 patients with lcSSc, hand mobility and ADL capacity were acceptable and did not differ significantly at followup, whereas grip force and perceived hand function were moderately impaired at baseline and at followup (26).

Taken together, these results suggest that the need for help with self-maintenance and domestic chores could be associated with less satisfaction with daily activities and an important socioeconomic burden. Patients needing help from their spouses or children may negatively influence both the familial balance and their own well-being, as well as contribute to the lower mental health domain score of the SF-36 in DU patients, as was reported in a previous study from our group (27).

In addition to marked global and hand disability and altered quality of life in SSc patients, we have previously reported the fair correlation and the nonredundance of the MACTAR with the HAQ (14). In the present study, patients with SSc showed limitations in their performance of domestic chores and self-care, as well as in social areas. Among the first priority of patients, sports activities were cited more often as being affected by disability (18.6% of patients), followed by cleaning of living area (17.5%), cooking (13.1%), work and employment (7.1%), and walking (6%). Our results on patient disability priorities are not comparable with those obtained by Sandqvist et al because of major differences in the selection of patients and the use of different scales to assess hand mobility and the patients' priorities in disability (26). The high proportion of dcSSc patients in our sample may explain in part the differences from previous studies in mobility outdoors and in transferring items (25, 26).

In the present study, 113 patients were in the workforce: 67 (59.3%) were in full-time employment, and 23 (20.3%) worked part time. Among patients in the workforce, 39 (34.5%) had changed their profession since the diagnosis of the disease. Multivariate analysis revealed global disability as the strongest predictor of work disability during SSc. Age and disease duration were also strongly associated with work disability. DUs were not retained in the multivariate model because of the small sample size and/or the lack of statistical power. Finally, the HAQ score was high in our cohort (mean ± SD 1.12 ± 0.8), but 34.5% of patients in the workforce had previous job accommodation before inclusion, which might also help explain this result.

In our study, the economic burden related to SSc was important, with decreased income, lack of promotion, and a feeling of discrimination at work, which confirms the results of a previous study (10). Interestingly, Ouimet et al reported 56% work disability and an important association with the HAQ in a group of 61 SSc patients (28), whereas Hudson et al reported that 36% of 643 SSc patients were working and 21% were disabled (29). In this study, work disability continued to increase steadily to 44.8% at 10–15 years of disease duration (29). Finally, Sandqvist et al reported in a small series that more time spent at work was related to a greater well-being (25, 30).

We provide evidence that the presence of DUs is associated with decreased performance in ADL, increased global and hand disability, the need for external help in the home, and more paid and nonpaid household help related to SSc. In a previous study, we reported that SSc patients with DUs have reduced wrist and hand mobility and increased global and hand disabilities (9). However, the major limitation of that study was that no distinction was made regarding the mechanisms of DUs. In the present study, 90% of patients had at least one fingertip DU. These DUs are mainly the consequence of vascular mechanism and play a major role in the occurrence of hand disability, although DUs proximal to the fingertip (resulting from limited joint motility of wrists and fingers and responsible for the occurrence of microtrauma) and DUs associated with calcinosis might also contribute to disability. Interestingly, patients with a history of DUs but no active DUs reported DU-related ADL limitations, emphasizing the importance of ADL limitations as a consequence of active or history of DUs.

We report that patients with DUs needed more external home help and more paid and nonpaid household help related to SSc. To our knowledge, this is the first time that evidence is provided that the cost of DUs might be increased beyond treatments costs. Finally, we observed that anxiety was significantly higher in patients with DUs than in others, whereas in a previous study conducted in 213 patients, the HADSa score did not differ significantly between patients with DUs and others (9). Further studies will be necessary to confirm this result.

Our study contains a number of limitations. The definition of work disability may vary from that used in other studies. Other parameters that may vary include the age at work cessation, whether cessation is attributed to SSc or to early retirement, and the proportion of patients taken into account among the whole population. Finally, the procedure used to recruit patients at a patient association meeting and in a tertiary care center may influence the results obtained (31).

In conclusion, SSc has a significant impact on ADL and work disability. The decision to stop work is likely influenced by the patient's health. Disability and the need for external home help are increased in SSc patients with DUs. Further studies are needed to identify whether the cure of DUs is associated with hand and/or global disability. Finally, additional studies are needed to evaluate the financial burden of SSc and DUs.

AUTHOR CONTRIBUTIONS

All authors were involved in drafting the article or revising it critically for important intellectual content, and all authors approved the final version to be submitted for publication. Dr. Mouthon had full access to all of the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.

Study conception and design. Bérezné, Guillevin, Mouthon.

Acquisition of data. Bérezné, Seror, Morell-Dubois, de Menthon, Fois, Dzeing-Ella, Nguyen, Hachulla, Guillevin, Poiraudeau, Mouthon.

Analysis and interpretation of data. Bérezné, Seror, Mouthon.

ROLE OF THE STUDY SPONSOR

Actelion Pharmaceuticals France had no role in the study design or in the collection, analysis, or interpretation of the data. Actelion Pharmaceuticals France had no influence on the publishing of the data.

Acknowledgements

We want to thank Dr. Frédérique Dupuis for her help. We also thank patients from the ASF and patients followed in the Internal Medicine Departments of Cochin and Claude Huriez hospitals for their participation in the study. We thank members of the ASF for their logistical help.

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