DOCTORA L T H E S I S
Department of Health Sciences Division of Health and Rehabilitation
Patients’ Experiences and Patient-Reported
Outcome Measures in Systemic Lupus
Erythematosus and Systemic Sclerosis
Malin Mattsson
ISSN 1402-1544ISBN 978-91-7439-990-5 (print) ISBN 978-91-7439-991-2 (pdf) Luleå University of Technology 2014
Malin Mattsson P
atients’
Exper
iences and P
atient-Repor
ted Outcome Measur
es in Systemic Lupus Er
ythematosus and Systemic Scler
osis
Patients’ experiences and patient-reported outcome measures in systemic lupus erythematosus and systemic sclerosis
Malin Mattsson
Division of Health and Rehabilitation Department of Health Sciences Luleå University of Technology
Printed by Luleå University of Technology, Graphic Production 2014 ISSN 1402-1544 ISBN 978-91-7439-990-5 (print) ISBN 978-91-7439-991-2 (pdf) Luleå 2014 www.ltu.se
The doctoral thesis
Patients’ experiences and patient-reported outcome measures in systemic lupus erythematosus and systemic sclerosis
Malin Mattsson
was performed at Luleå University of Technology in collaboration with Karolinska Institutet and the County Council of Norrbotten
To my family Per-Erik, Julia and Lukas
CONTENTS ABSTRACT 1 ORIGINAL PAPERS 3 BACKGROUND 4 Person-centred care 4 Physiotherapy 4
International classification of functioning, disability and health 5 Description and categorisation of personal factors 6
Rheumatic systemic connective tissue disease 7
Systemic lupus erythematosus (SLE) 7
Systemic sclerosis (SSc) 9
Non-pharmacological care and physiotherapy in SLE and SSc 9 Experiences of SLE and SSc from a patient perspective 10 Patient reported outcome measures (PROMs) in SLE and SSc 13
Measuring patient perspective in SLE and SSc 13
Measuring fatigue in SLE 14
RATIONALE 15
AIMS, Overall aim, Specific aims 16
METHODOLOGICAL FRAMEWORK 17
Reliability and validity of PROMs 17
Reliability and validity of the Fatigue Severity Scale (FSS) in SLE 18
Trustworthiness in qualitative studies 19
Focus groups interviews 20
Content analysis 20
Meaning condensation 21
Linking qualitative data to the ICF and literature review 21
METHODS 22
Design 22
Participants 23
Data collection and procedure 24
Data analysis 27
Study I 27
Construct validity 27
Feasibility, ceiling and floor effects 27
Internal consistency and test–retest reliability 28
Content validity 28
Study II Content analysis 28
Study III Meaning condensation and linking of concepts to the ICF 29
Study IV 30
Sorting of concepts to the personal factors structure by Geyh et al. 30
Literature review of PROMs in SSc research 30
PROMs in SSc research coverage of personal factors 31
RESULTS 33 Reliability and validity of the FSS in Swedish (study I) 33
Construct validity 33
Feasibility, ceiling and floor effects 33
Internal consistency and test–retest reliability 33
Content validity 33
Experiences of illness in everyday life in persons with SLE (study II) 36
Multifaceted uncertainty 36
Focus on health and opportunities 36
Experiences of functioning and health in persons with SSc (study III) 37 Meaning condensation and linking concepts to the ICF 37 Experiences of personal factors in SSc (study IV) 38
Experiences of personal factors according to Geyh et al.’s structure 38
Literature review of PROMs in SSc research 38
PROMs in SSc research and their coverage of personal factors 38
DISCUSSION 44
Result discussion 44
Reliability and validity of the FSS in Swedish (study I) 44
Experiences of illness in everyday life in persons with SLE (study II) 45
Experiences of functioning and health in persons with SSc (study III) 48
Experiences of personal factors in SSc and PROMs covering these (study IV) 50 Personal factors according to the ICF and Geyh et al.’s structure (study III, IV) 51
Overall similarities in the experiences of persons with SLE and SSc 52
Method discussion 53
Quantitative methodology (study I) 53
Qualitative methodology (study II-IV) 54
Literature review (study IV) 55
Implications 56
Conclusions 57
SUMMARY IN SWEDISH - SAMMANFATTNING PÅ SVENSKA 60
ACKNOWLEDGEMENTS 62
References 65
Appendix 81
PAPERS I-IV
DISSERTATIONS FROM THE DEPARTMENT OF HEALTH SCIENCE, LULEÅ UNIVERSITY OF TECHNOLOGY, SWEDEN
ABSTRACT
The overall aim of this thesis was to describe experiences of persons with systemic lupus erythematosus (SLE) and systemic sclerosis (SSc), with a focus on functioning and health, and to evaluate patient reported outcome measures (PROMs).
Methods The Fatigue Severity Scale (FSS), measuring fatigue and its consequences in
SLE, was translated into Swedish and tested for aspects of reliability and validity (study I). In order to understand fatigue in its context, persons with SLE described their experiences of illness in everyday life. Focus group interviews were conducted and analysed using qualitative content analysis (study II). Experiences of functioning and health in persons with SSc in different European countries were described in order to obtain a biopsychosocial understanding. A multi-centre study with focus group interviews of persons with SSc in four different countries was conducted. The data was analysed with modified meaning condensation, resulting in concepts that were linked to the International Classification of Functioning, Disability, and Health (ICF) (study III). To deepen the understanding of personal factors in the experiences of functioning and health in SSc, the concepts from Study III were re-analysed using Geyh et al’s personal factor structure. Further, PROMs in SSc research were identified in a literature review, and whether and to what extent they included the personal factors found was analysed(study IV).
Results No important ceiling or floor effects, substantial test-retest reliability,
satisfactory internal consistency and content validity, and support for construct validity for the Swedish version of the FSS were found (study I). Two themes described experiences of illness in everyday life in persons with SLE: i) Multifaceted uncertainty including an unreliable body; obtrusive pain and incomprehensible fatigue; reliance
on medication and health care; mood changes and worries and ii) Focus on health and
opportunities including a learning process implying personal strength; limitations and
possibilities in activities and work; a challenge to explain and receive support; and living an ordinary life incorporating meaningful occupations (study II). Concepts
identified in common for persons with SSc in all four European countries involved the ICF-components body function and structure, activities and participation, and
environmental and personal factors. Most concepts were linked to body functions and
structures followed by environmental factors. Most concepts, in common in all four countries, within body function and structure and activity and participation were in line with suggested domains to be measured in SSc. Environmental factors, in common in all four countries, involved experiences such as too much heat and cold, support from significant others, healthcare, drugs and side effects,
non-pharmacological treatment and social security system. Personal factors, found in all four countries, involved struggle to master one’s life with the disease, such as unclear future, change of expectations and positive experience of the disease, being in control, being strong owing to having mastered the disease (study III). Using the personal factor structure, 19 concepts related to Patterns of experience and behaviour, 16 to
Thoughts and beliefs, nine to Feelings, one to Motives, and one to Personal history and Biography were found. Thirty-five PROMs in SSc research were identified, and
feelings (51%); less covered were patterns of experience and behaviour (14%), and the identified PROMs did not cover motives and personal history and biography (study IV).
Implications and conclusions The FSS in Swedish is reliable and valid for measuring
fatigue and its consequences in persons with SLE with no-moderate disease activity and no or low organ damage. However, the results also indicate that measuring fatigue is complex and more aspects of its validity such as sensitivity to change have to be studied. Multifaceted uncertainty and focus on health and opportunities were experienced among persons with SLE. Aspects of uncertainty and opportunities were also experienced in SSc. The findings are in line with theories focusing on uncertainty in illness and personal growth following adversity. This highlights the importance for healthcareprofessionals to identify and understand patients’ experiences of uncertainty to support focus on health and opportunities in patients with SLE and SSc. PROMs that capture uncertainty and opportunities needs to be developed. The experiences of functioning and health in SSc, found in all four European countries, may be used to guide clinical assessment and the development of an outcome measurement core set in SSc. For a full understanding of the aspects of the disease that are most relevant to people with SSc, people with SSc from different partof the world needs to be involved. The personal factor structure used was supportive to identify, organise, and deeper understand personal factors in SSc. Almost half of the PROMs in SSc research, including provisional and proposed PROMs, did not cover any of the personal factors found,which highlights the importance of further developing PROMs that covers personal factors in SSc. To increase the knowledge of the role of personal factors, PROMs that cover personal factors found such as personal history and biography, feelings, thoughts and beliefs, motives, and patterns of experience and behaviour ought to be considered in clinical practice and SSc research. In person-centred care, health professionals need to recognise environmental and personal factors in those with SLE and SSc to tailor individual interventions.
Key words Experiences, fatigue, international classification of functioning disability
and health, patient-reported outcome measures, personal factors, qualitative
interviews, questionnaire, reliability, systemic lupus erythematosus, systemic sclerosis, validity
ORIGINAL PAPERS
This thesis is based on three published papers (Study I, II, III) and one submitted manuscript (Study IV).
I. Mattsson M, Möller B, Lundberg I E, Gard G, Boström C. Reliability and validity of the Fatigue Severity Scale in Swedish for patients with systemic lupus erythematosus. Scand J Rheumatol. 2008;37:269-77.
II. Mattsson M, Möller B, Stamm T, Gard G, Boström C. Uncertainty and opportunities in patients with established systemic lupus erythematosus: a qualitative study. Musculoskeletal Care. 2012;10(1):1-12.
III. Stamm TA, Mattsson M, Mihai C, Stöcker J, Binder A, Bauernfeind B, Stummvoll G, Gard G, Hesselstrand R, Sandqvist G, Draghicescu O, Gherghe AM, Voicu M, Machold KP, Distler O, Smolen JS, Boström C. Concepts of functioning and health important to people with systemic sclerosis: a qualitative study in four European countries. Ann Rheum Dis. 2011;70(6):1074-9.
IV. Mattsson M, Boström C, Mihai C, Stöcker J, Geyh S, Stummvoll G, Gard G, Möller B, Hesselstrand R, Sandqvist G, Draghicescu O, Gherghe AM, Voicu M, Distler O, Smolen JS, Stamm TA. Personal factors in systemic sclerosis and their coverage by patient-reported outcome measures: a multi-centre European qualitative study and literature review. (Submitted manuscript)
BACKGROUND Person-centred care
Person-centred care has been described as important in improving the quality of
health care.1 Three core issues have been identified in person-centred care:
patients’ involvement and participation, the relationship between the patient and the professional, and the context of care in system issues such as policies and
organisation.2 Person-centred care can be defined as the attempt to view the
person as an individual and to try to understand what the illness means to the person; to understand the needs, values, and context; and to listen to the person’s
viewpoint and share responsibility for care.3 Care that is tailored to the
individual can contribute to better communication between the professional and the patient and improve understanding and adherence to treatment. Person-centred care can also increase patients’ knowledge and autonomy, and their
ability to influence care and decision-making.1, 3 In rehabilitation, person
centeredness implies a positive perspective, with a focus on abilities and
participation rather than on disability and handicap.4 To be able to individualise
the care to the person, health professionals need information about their patients’ experiences, feelings, needs, resources, coping styles, and goals. Patient
narratives are a starting point in the person-centred care model.1
Physiotherapy
The World Confederation for Physical Therapy (WCPT) describes physiotherapy as follows:
… physical therapy is concerned with identifying and maximising quality
of life and movement potential within the spheres of promotion, prevention, treatment/intervention, habilitation and rehabilitation. This encompasses physical, psychological, emotional, and social wellbeing.5
Body, movement, function, and interaction have been described as central
concepts in physiotherapy related to health from a biopsychosocial perspective.6
Health in physiotherapy has been described as the individual’s ability to reach
his or her goals in the environment.6 The body in physiotherapy is viewed to
include the whole person,7 the experience of the body, its movements and
cognitive, emotional and social aspects.6 According to Cott et al.,8 movement is
a continuum with different levels, from a microscopic level to the person in society. A holistic view of movement is applied in physiotherapy, covering
physical, emotional, sociocultural, and existential factors.9 Function in
physiotherapy can be viewed in line with the International Classification of
Functioning, Disability and Health(ICF), a biopsychosocial framework
developed by the World Health Organization (WHO).6 In physiotherapeutic
practice, the interaction between the physiotherapist and the patient is important
The physiotherapist can facilitate the interactions with the patient by listening to the patient and identifying the patients’ emotions and body language and thereby
increase the possibility of positive treatment outcomes.10 Good interaction are
important when identifying and utilising patients’ resources,11 which are
essential in person-centred care. The physiotherapy process includes assessment, evaluation, and a physiotherapy diagnosis; planning together with the patient;
intervention/treatment; and reassessment.5
International classification of functioning, disability and health The WHO defined health in 1948 as “a state of complete physical, mental, and
social well-being and not merely the absence of disease or infirmity”.12 Further,
health is described by WHO as “a resource for everyday life, not the object of living, and it is a positive concept emphasising social and personal resources as
well as physical capabilities”.12 In the ICF, health is viewed from a
biopsychosocial perspective, which means an integrated biological, individual, and social perspective. The ICF provides a framework and standardised
language to describe, study, and understand health and health-related states from
this biopsychosocial perspective.13 The ICF has been described as a core issue in
person-centredness in rehabilitation,4 and is considered to be a valuable
instrument in physiotherapy.9 In the ICF model, the focus is on health and
functioning, rather than on disability.14 Thus, it classifies components of health
rather than consequences of diseases.13 Functioning is viewed in three levels:
biological, individual, and social. In the ICF “Functioning and disability are understood as umbrella terms denoting the positive and negative aspects of
functioning”.15
Functioning and disability consist of two components: i) Body functions and body structures
ii) Activities and participation
Contextual factors also consist of two components: i) Environmental factors
ii) Personal factors
Functioning is developed through dynamic interactions with the person’s health
condition as well as environmental and personal factors.15 The components of
the ICF consist of a hierarchically ordered category system that is worded in a neutral language in order to describe both positive and negative aspects of
Description and categorisation of personal factors Personal factors are described in the ICF as:
…the particular background of an individual’s life and living, and
comprise features of the individual that are not part of a health condition or health states. These factors may include gender, race, age, other health conditions, fitness, lifestyle, habits, upbringing, coping styles, social background, education, profession, past and current experience (past life events and concurrent events), overall behaviour pattern and character style, individual psychological assets and other characteristics, all or any of which may play a role in disability at any level.13
Because of differences in cultural and societal issues related to personal factors and a lack of a clearly defined conceptualisation of them, personal factors have
not yet been classified in the ICF.13, 15 However, they play an important role,
since they encompass the influence of functioning related to the individual, for example, through the person’s life events, behaviour patterns, and psychological
assets.15 Personal factors can be seen to have a shifting role, and to be an
outcome, moderator, or determinant of functioning.16
Suggestions as to how personal factors can be categorised have been presented in the literature. In a recent study, eight different suggestions about how
personal factors can be categorised were discussed.17 The categorisations
differed in development and background.17 For example, structures for personal
factors have been developed with the support of a theoretical model in
occupational medicine,18 from qualitative interviews with persons with motor
neuron disease19 as well as from audiological research.20
A personal factor structure was developed by Geyh and colleagues through spinal cord injury (SCI) research, using the analysis of a data pool that involved information from focus groups comprised of persons with SCI, systematic literature reviews, SCI registers, and a categorisation system created for social
medical reports.21 This personal factor structure is described as the most
comprehensive one of its kind for the categorisation of personal factors as well
as for use in a broader clinical context.22 Thus, the structure developed by Geyh
et al.21 was chosen to be used in this thesis for studying personal factors. This
personal factor structure consists of three parts that are subdivided into seven
more specific areas21, 22 (Figure 1), in this thesis the focus was on experiences
and patterns. The personal factor structure has been used, for example, to code medical work-capacity evaluations in the context of pension claims for people
with pain disorders22 and to structure correlates or determinants of participation
Structure of personal factors PART I: FACTS
The person’s position in the physical, social, and temporal context 1. Area: Sociodemographic personal characteristics
2. Area: Position in the immediate social and physical context 3. Area: Personal history and biography
PART II: EXPERIENCE
The person’s concurrent experience, as well as contextual and situation-specific internal reactions
4. Area: Feelings
5. Area: Thoughts and beliefs 6. Area: Motives
PART III: PATTERNS
Generalised, context-independent, cross-situational, recurrent, persistent patterns in the person’s experience and behaviour
7. Area: Patterns of experience and behaviour
Figure 1. The personal factor structure developed by Geyh et al. 21, 22
Rheumatic systemic connective tissue diseases
Inflammatory rheumatic diseases can be divided into arthritis, spondylarthritis, and systemic connective tissue diseases. The latter includes diseases such as systemic lupus erythematosus (SLE), systemic sclerosis (SSc), Sjögren’s
syndrome, inflammatory muscle diseases, and overlapping syndromes.25
Common features of these diseases are autoimmunity with the development of specific autoantibodies and inflammatory processes in several organs and
systems.25-27 This thesis focuses specifically on experiences of functioning and
health in two of the systemic connective tissue diseases, SLE and SSc. Systemic lupus erythematosus
The most frequent onset of SLE occurs among women from their late teens to
early forties.26 The ratio between females and males is 9:1.26 In Western Europe,
the incidence has been described as varying between 2.2 and 5.0/100 000/year,
of Rheumatology (ACR) has established classification criteria for SLE.29 Four of the following 11 criteria must be met for an adequate SLE classification in research: malar rash, discoid rash, photosensitivity, oral ulcers, arthritis, serositis, renal disorder, neurologic disorder, hematologic disorder,
immunologic disorder, and antinuclear antibody. The disease is characterised by an activated immune system, including the occurrence of autoantibodies and complement activation resulting in inflammation in different organs and systems with a variety of disease expressions and organ affections. The most frequent clinical manifestations described in a European population are arthritis (48%), malar rash (31%), nephropathy (28%), photosensitivity with skin rashes (23%),
and neurological involvements (19%).30 Among the most frequent symptoms
reported by persons with SLE are pain and fatigue.31, 32Furthermore, patients
often express a depressed mood 33 and a decreased health-related quality of life
(HRQL) in SLE.34, 35
One part of this thesis deals with fatigue in SLE. Fatigue can occur in inactive,
mild, and recent-onset SLE,36 and is greater than in the general population or in
healthy controls.35, 37, 38 The aetiology of fatigue is unclear and seems to be
multifactorial, although poor sleep may play a role in the cause of fatigue.39
There is a lack of consensus of the definition of fatigue in SLE.40, 41 However,
fatigue as an “extreme and persistent tiredness, weakness, or exhaustion—mental,
physical or both” is one way to define fatigue.42 There are conflicting results
concerning the relationship between disease activity and fatigue.35, 40 However,
several studies have shown that disease activity and organ damage have no or
low correlation to fatigue.36, 43-50 Disease duration has not shown any clear
association with fatigue levels,35 and age has shown no or a little correlation with
fatigue.36, 48, 50, 51 However, higher level of fatigue has been linked to lower levels
of HRQL36, 44, 50 and fatigue has been found to have a moderate correlation with
aerobic capacity.52
The pharmacological treatment of SLE can involve antimalarial agents,
corticosteroids, and immunosuppressive agents.26, 53 As a result of improvements
in diagnosis and therapy in the last several decades, the survival rate for SLE has
increased, and the ten-year survival rate is now approximately 93%.54 However,
increased mortality continues to be a factor in SLE compared to the general population, with the most frequent causes of death being cardiovascular diseases, infections, and high cumulative disease activity with simultaneous involvement
Systemic sclerosis
SSc is more frequent among women than men, with a ratio 4–14:1.55 Disease
onset usually occurs between the ages of 40 and 60. The incidence is 3.7-3.8/1 000 000/year, and the prevalence is 31-71/1 000 000 in Western
Europe.27 In 1980, the ACR developed classification criteria for SSc; the major
criterion is skin thickening proximal to the metacarpophalangeal joint, and the minor criteria are: i) sclerodactyly, ii) digital pitting scars of fingertips or loss of substance of the distal finger pad, and iii) bilateral basilar pulmonary fibrosis. For the classification of SSc, the major criterion or at least two of the three
minor criteria are required.56 The disease is characterised by microangiopathy
and increased deposition of matrix molecules in skin and organ systems.55, 57
According to Gu et al., 55 Raynaud’s phenomenon (92%) are most common
among patient with SSc in a Eropean population, with increased sensitivity for
emotional stress and cold.58 This is followed by esophageal disturbance (79%),
telangiectasias (67%), lung involvement (64%), digital pitting (62%), and
polyarthralgia (52%).55 Common symptoms and impacts in SSc are fatigue, pain
and limitations in hand functions.59 Furthermore, HRQL is known to be
decreased60 and depressed mood can also be common in SSc.61, 62 Body image
distress in SSc is associated with severe skin involvements in hands and to a
depressed mood and negative psychosocial functioning.63 In regard to the
appearance of skin involvements, the disease can be distinguished as limited or diffuse SSc. In limited SSc, skin involvements are limited to distal extremities and the face, in contrast to diffuse SSc, where all of the skin on the body can be
affected.57 Further, in the diffuse form, the patient is more disposed to
developing internal organ involvement and, therefore, risks a more severe
prognosis.27, 64
Disease-modifying medications are lacking in SSc; recommendations for drug treatments are related to digital vasculopathy, pulmonary arterial hypertension, gastrointestinal involvement, renal crisis, intestinal lung disease, and skin
involvement.65 The survival rate has been improved by advances in diagnosis
and treatments, and the ten-year survival rate has been reported as 66%. The most common causes of death are pulmonary fibrosis and pulmonary arterial
hypertension.27
Non-pharmacological care and physiotherapy in SLE and SSc Together with other health care professionals, the physiotherapist has an important role in the non-pharmacological care and rehabilitation of patients
with rheumatic diseases.66 There is however scarce research about the role of
physiotherapy in SLE and SSc.
Patient education and self-management is of importance in SLE. Encouragement to a lifestyle with physical exercise, smoking cessation, and weight control are
included in the general management recommendations for SLE by the European
League Against Rheumatism (EULAR).53 Monitoring of physical activity and
exercise are recommended once a year. Further, photo-protection is suggested
for patients with skin involvements.53 Positive effects of patient education and
physical exercise has been presented in several studies in SLE.67-75 However,
there is no general recommendation of non-pharmacological care in SLE. Research concerning patient’s experiences of their illness as a starting point for physiotherapy interventions such as patient education covering
self-management, motivational counselling, physical activity and exercise is sparse. Existing studies in SSc cover a variety of non-pharmacological interventions and diverse measurement outcomes that have limited the possibility of fully
understanding the effectiveness of non-pharmacological interventions.76
Consequently, there is a lack of general recommendations for
non-pharmacological interventions in the treatment of SSc.65 However, EULAR
recommendations for the treatment of systemic sclerosis and the Swedish scleroderma study group suggest in their programme for SSc care of patients that patient education, self-management, knowledge about assistive devices and advice about how to stop smoking should be included as well as how to avoid
stress and cold.65, 77 Research covering patient experiences of their illness in SSc
as a starting point for physiotherapy interventions is rare.
Experiences of SLE and SSc from a patient perspective The multifaceted nature of SLE and SSc emphasise the significance of increasing the understanding of patients and researching patient experiences.
Understanding rare diseases is important in optimising health care.78 A
discrepancy between the patients’ and health professionals’ views on the
patients’ health has been described.79-82
It has also been expressed by persons with SLE that professionals do not fully understand the consequences of the
illness, and as a result, do not provide them with optimal information.83
Different views between patients and professionals may be influenced by
different focus for example focusing on psychological or physiological effects of
the disease.81 An increased understanding of patient experiences of SLE and SSc
may improve patient-health professional interactions and be beneficial in the development of person-centred care and physiotherapy.
A recent thematic synthesis of qualitative studies in SLE described experiences
resulting in five themes, subdivided into 23 sub-themes.84 These are:
i) Restricted life style; pervasive pain, debilitating fatigue, mental deterioration,
disruptive episodic symptoms, postponing parenthood. ii) Disrupted identity; gaining diagnostic closure, prognostic uncertainty, being a burden, hopelessness, heightened self-consciousness, fearing rejection, guilt and punishment.
iii) Societal stigma and indifference; illness trivialization, socially ostracized,
averse to differential treatment. iv) Gaining resilience; optimism, control and
empowerment, being informed and involved, valuing mutual understanding, and
v) Treatment adherence; preserving health, rapport with clinicians, negotiating
medication regimens, and financial burden. When planning this thesis, only one
qualitative study of women with SLE in Sweden was published, and there were rather few studies including both men and women with SLE confirmed by the ACR criteria.
There are few qualitative studies illustrating experiences of SSc. The qualitative studies in SSc have studied experiences of living with the disease, body image dissatisfaction, emotional distress, and management of work life (Table 1).When planning this thesis; no qualitative studies had been conducted describing how persons with SSc in different European countries experience their functioning and health. There were also rather few studies including both genders.
Table 1. Experiences of persons with systemic sclerosis (SSc) published in qualitative studies. Author Year Country Aim Methodological framework/ Analysis
Results presented as themes, categories, domains, concepts, etc. Oksel, et al.85 2014 Turkey Life experiences Phenomenology analysis
Self-perception: self-esteem disturbance, fear, anxiety. Role in the relationship: altered family and social process, altered role performance. Activity and exercise: activity intolerance, fatigue, physical difficulties. Sexuality: sexual dysfunction. Ennis, et al.86 2013# England Body image dissatisfaction in telangiectases Mixed method/ Thematic analysis
Changes in behaviour as result of telangiectases. Public and private self-image. Negative emotional impact of
telangiectases. Appreciation of life. Mendelson, et al.87 2013# USA Challenges and adaptations in work Content and thematic analysis
Work as a daily challenge: the work environment with opportunities, challenges, and accommodations, career planning, support of others.
Cinar, et al.88 2012 Turkey Daily life experiences Phenomenology analysis
Physical effects of the illness. Emotional effects of the illness: disappointment, anxiety over the future. Social isolation. Coping strategies of the participants: individual coping, family support, interaction of the patients with one another. Newton, et al.89 2012 Canada Experiences of emotional distress Content-thematic analysis
The meaning of “depression” and the experience of distress: the specific use of the word depression, taking issue with the term depression, ability to find pleasure. The causes of distress: the beginning is hard, worse disease, worse mood, specific symptoms that caused distress, the loss of autonomy, two-way street between distress and disease. Coping with distress: putting it out of your mind, keeping busy, finding new ways to stay autonomous, avoiding support groups and psychologists, relying on friends and family. Sandqvist et al.90 2012# Sweden Management of work life Thematic content analysis
Adjustment to the work situation: reducing work hours, work flexibility, adapting work tasks and work environment. Adapting to own resources: prioritising of activities and use of energy, receiving assistance. Disclosing limitations.
Suarez-Almazor, et al.91 2007# USA Disease and symptoms burden Grounded theory/ Thematic analysis
Patient awareness. SSc-related problems: emotional distress, appearance, lifestyle, marital/sexual problems, social issues. Disease activity and progression. Symptoms: pain, GI system, energy/fatigue, skin, distal extremities, lungs. Expectations. Other emergent themes: patient-doctor relations, self-efficacy, coping and adaptation.
Mendelson, et al.92 2007 USA Living with SSc Immersion and crystallization/ Thematic analysis
Become your own advocate: secure effective medical management, live your life, learn everything you can.
Joachim, et al.78 2003 Canada Living with SSc Thematic analysis
Physical manifestations. Disclosure/non-disclosure to others. Living with scleroderma. Being normal. Facing the future.
PROMs in SLE and SSc
Measuring patient perspective in SLE and SSc
The importance of describing the patient’s perspective of rheumatic diseases has been recognised by the international network, Outcome Measures in
Rheumatology Clinical Trials (OMERACT).93 Recommendations about which
PROMs and other measurement instruments to use in clinical trials in
rheumatology are under development. Core sets of outcomes and domains that are of interest to measure in clinical trials are being developed through an OMERACT process. In SLE, OMERACT has recommended the following domains as core outcomes: disease activity, damage, HRQL, and adverse
events.94 In SSc, the following domains have been suggested: soluble
biomarkers, cardiac events, digital ulcers, gastrointestinal involvement, global health, HRQL, musculoskeletal symptoms, pulmonary involvement, Raynaud’s
phenomenon, renal involvement, and skin affection.95 The development of a
generalised framework for the development of core sets in rheumatology is on-going within the OMERACT process. Contextual factors have been recognised as important to measure in order to interpret and better understand study
results.96 Further, an ICF core set, a selection of the most essential ICF
categories for a specific condition, is under development in SLE97 as well as in
SSc.98
The use of PROMs has increased over the years and is now an important part of
clinical practice and clinical trials.99 PROMs can be seen as a tool, usually a
questionnaire, used to assist professionals and researchers in gaining insight into
a patient’s perspective of health.100
PROMs can be used in screening, monitoring, or evaluating interventions or to stimulate dialogue between the
patient and the clinician.101 Questionnaires which are one-dimensional results in
one total score and multidimensional ones are divided into subscales.42
In 2011, a series of reviews were published covering measurement instruments
of patient outcomes in rheumatology.99 Among these 23 PROMs were included
with descriptions of reliability and validity specified for adult populations of persons with SLE and SSc. In SLE, the following PROMs to measure fatigue have been described: the Fatigue Severity Scale (FSS), the Chandler Fatigue Questionnaire (CFQ), the Functional Assessment Chronic Illness Therapy – Fatigue (FACIT-F), the Multidimensional Assessment of Fatigue (MAF) and the
Multidimensional Fatigue Inventory (MFI).102 To assess disease activity in SLE,
the Systemic Lupus Activity Questionnaire for Population Studies has been
described (SLAQ).103 Concerning general function, the Health Assessment
Questionnaire (HAQ) has been illustrated,104 and to assess work disability and
productivity, the Workplace Activity Limitations Scale (WALS) has been
described.105 Finally, for an examination of general health and HRQL in SLE,
Life (LupusQoL), the Systemic Lupus Erythematosus – Specific Quality of Life Questionnaire (SLEQOL), and the Systemic Lupus Erythematosus Quality of
Life Questionnaire (L-QoL) were other PROMs illustrated in the reviews.106
In the series of the reviews, for measuring hand function in SSc; the Cochin
Hand Functional Scale107 was described, and for general function; the HAQ, the
Scleroderma-HAQ (S-HAQ), the UK Scleroderma Functional Score, and the
Scleroderma Assessment Questionnaire (UKFS) were described.108 To measure
disability in SSc; the World Health Organization Disability Assessment
Schedule II (WHODASII) was described. For assessment of general health and
HRQL in SSc; the SF-6D,109 the Symptom Burden Index (SBI), the University
of California, Los Angeles Scleroderma Clinical Trials Consortium,
Gastrointestinal Scale (UCLA SCTC GIT) 2.0, the Gastrointestinal Quality of
Life Index, the Frequency Sclaes for Symptoms of Gastroesophagel Reflux Disease (FSSG), the Baseline Dyspnea Index and Transition Dyspnea Index
(Mahler’s Index), St. George’s Respiratory Questionnaire (SGRQ), Cambridge
Pulmonary Hypertension Outcome Review (CAMPHOR), Raynaud’s Condition
Score (RCS) and the Mouth Handicap Scale in Systemic Sclerosis were
described.108
These lists may however not be a complete description of PROMS in SSc and SLE as there may be other PROMs tested for reliability and validity in these diagnoses. Further, PROMs covering contextual factors of interest for person-centred care and physiotherapy is still not fully explored.
Measuring fatigue in SLE
Using a diversity of PROMs measuring fatigue in SLE in research has made it
more difficult to compare the results of different interventions.110 However, the
FSS is the most commonly used and recommended PROM for clinical trials and
observational studies of SLE.40 The FSS is a one-dimensional questionnaire that
has been developed for SLE, with valid psychometric properties, available in
several languages.40 The FSS was originally developed for patients with SLE
and multiple sclerosis (MS)111 measuring the impact of fatigue on daily life.40, 102
The questionnaire consists of nine items that have been shown to be common for SLE and MS. Each item is rated from one to seven and the FSS total score is calculated by the mean value of the nine items. A higher score indicates more
fatigue.111 At the time of this thesis, no existing study had translated and tested a
RATIONALE
I have worked for many years at a rheumatology clinic as a physiotherapist and met persons with different rheumatic diseases. I realised that there was a few studies within physiotherapy covering SLE and SSc, and that many patients were troubled with HRQL and fatigue. As a physiotherapist I wanted to know more about patients’ experiences of their functioning and health in order to more effectively support them in their self-management of the disease, physical activity and exercise, as well as to individualise their physiotherapy.
To gain insights about patients’ perspectives, individuals can be interviewed or PROMs can be used. In clinical practice PROMs can serve as a tool to measure functioning and health. PROMs can also be used to stimulate interaction between the patient and the health professional when discussing the patient’s completed PROM. In order to develop and evaluate non-pharmacological treatment and physiotherapy interventions, reliable and valid measurement instruments are needed. An invisible yet, common symptom, experienced by
patients with rheumatic diseases is fatigue.112-115 The FSS is a PROM that has
been recommended to measure fatigue in SLE.40 A Swedish translation of the
FSS tested for reliability, and validity in SLE is lacking.
To develop person-centred care, increased knowledge about experiences of functioning and health in patients with rheumatic systemic connective tissue disease such as SLE and SSc is needed. Such knowledge can support the physiotherapist in assessment and tailoring interventions for each patient. Qualitative studies of everyday life experiences are of special interest, since these studies can support the understanding of fatigue in its context rather than
as an isolated entity.116 Few qualitative studies were presented where the
diagnosis was confirmed explicitly using the criteria specified by the ACR for SLE and/or have included both women and men focusing on both positive and negative aspects of SLE.
Due to the rarity of SSc, cooperation among research centres is especially valuable, since this can contribute to larger study populations. Multi-centre studies with centres in different countries can also provide broader knowledge and understanding of patients’ experiences and thus contribute to development of an outcome measurement core set. The ICF is a framework that can be used to describe and study functioning and health from a biopsychosocial perspective. Qualitative European multi-centre studies of experiences of functioning and health in SSc from such a biopsychosocial perspective are lacking.
To gain a deeper understanding of patients’ experiences of functioning and health in SSc, it is important to learn more about personal factors from a patient perspective. Personal factors cover information about the influence on
functioning related to the individual.13, 15 Although not classified in the ICF, the use of a personal factor structure can provide a conceptualisation and category specification that could contribute to a further understanding of personal factors. To have access to PROMs, including personal factors, is needed to improve person-centred care. Qualitative European multi-centre studies with special focus on personal factors in SSc and their coverage in PROMs are lacking.
AIMS Overall aim
The overall aim of this thesis is to describe experiences of persons with systemic lupus erythematosus (SLE) and systemic sclerosis (SSc) with a focus on
functioning and health and to evaluate patient reported outcome measures (PROMs).
Specific aims
To translate, test, and describe aspects of reliability and validity of Fatigue
Severity Scale (FSS) in Swedish (FSS-Swe) in patients with SLE (Study I)
To describe how patients with established SLE experience their illness in
everyday life, including both negative and positive aspects (Study II)
To describe how persons with SSc in different European countries experience
their functioning and health and to link these experiences to the WHO ICF framework to develop a common understanding from a biopsychosocial perspective (Study III)
To identify and describe personal factors in the experiences of functioning and
health of persons with SSc and to examine if and to what extent PROMs in SSc research cover these personal factors (Study IV)
METHODOLOGICAL FRAMEWORK Reliability and validity of PROMs
In order to operationalise and quantify the phenomena under study is it essential that questionnaires have good reliability and validity. When translating a questionnaire from an original language into a new language, agreement concerning the content and meaning of the items between the languages is
important.117 Cultural adaptation and translation is performed in a stepwise
procedure, where the items are translated and back-translated by experts. Further the translation is analysed and the language expressions are harmonised during
the process.118, 119 In a last step, the questionnaire needs to be tested for its
psychometric properties.119
The range of the scale in a questionnaire must be able to detect the current status
as well as changes in status; otherwise, a ceiling or floor effect occurs.120
Reliability is defined as the degree to which a result of a measure is free from
errors.121 In classic test theory, the basic assumptions are that every measure
consists of a true value and an error component,121 and that items in the
instrument are comparable indicators of the constructs to be measured.122 Test-
retest reliability can be estimated by studying the stability of the questionnaires
over time by calculating the agreement between test-occasions.120 The level of
agreement between test occasions indicates whether the questionnaire is reliable.
Reliability also includes internal consistency122, 123 which concerns the
relationship of the items to each other, i.e., the items must be homogeneous and
measure the same thing.122, 124
Validity describes whether an instrument adequately measures what it is
intended to measure.121, 122 Several validity concepts exist, such as content
validity, criterion validity, construct validity, and discriminal validity.117 Content
validity is defined as the extent to which the items cover all aspects, the whole
content, of the phenomena the questionnaire aims to measure.121, 122, 125 To assure
and test content validity, the content of the items in the questionnaire can be
critically reviewed by relevant persons.121 Criterion validity refers to whether the
instrument relates to another instrument measuring the same phenomenon.125
This is possible to study if there is a “gold standard”, e.g., an established and
defined unit of measure with which to compare the assessed measure.117, 125
When it comes to measuring abstract concepts where no instrument can serve as a “gold standard”, construct validity can be studied. Assessment is conducted by comparing a measurement instrument result with results from other
instruments.122 Construct validity is more clearly verified if a hypothesis exists
concerning the relationship with other instruments, e.g. the assessed instrument
correlates to certain instruments but not to others.122 Another aspect of validity is
discriminal validity, and this assesses if the measurement instrument has the
The Consensus-based standards for the selection of health status measurement Instruments (COSMIN) checklist is a tool for evaluating the quality of studies
on measurement properties of health-related, patient-reported outcomes.126, 127
The checklist in general suggests to report missing items and to describe how missing items are handled. For internal consistency Cronbach’s alpha is suggested to be calculated and to control whether the instrument is uni-dimensional (e.g., factor analysis is calculated). When assessing test-retest reliability, the test must offer at least two test occasions, be administered independently in similar conditions, and employ stable test persons who are concerned with the construct of interest during the test period. When assessing test-retest reliability in ordinal scales, weighted kappa can be calculated. For assessment of content validity, the items in the questionnaire have to be relevant and reflect the construct of interest. To obtain construct validity a clear
description of the other instruments used for comparison is necessary, and, if possible, a hypothesis concerning the magnitude and directions of the correlations between the questionnaire and the other instruments should be
provided.127 For cross-cultural validity when translating an instrument, both the
original and the new translated language is suggested to be reported with description of how differences between languages were handled. Further
suggested is that translators work separately from each other, that the translation is reviewed by for example the original developer and that the translation is pre-tested (e.g. is it easy to understand and cultural relevant) in a similar sample as
the original.126 The COSMIN checklist has been updated with a rule of thumb
for sample size, 100 persons is described as excellent, 50 as good, 30 as fair, and
less than 30 as poor.128 No recommendation is described in the checklist
concerning time intervals between test occasions in test-retest procedure, as this
is related to the construct to be measured.126, 127 However, others have described
that an appropriate time interval between test occasions when assessing
test-retest reliability can be one to two weeks.129
Reliability and validity of the FSS in SLE
The original English version of the FSS has been reported as reliable in SLE.47,
111 The test-retest reliability has been described as acceptable or excellent,47, 111
and internal consistency is acceptable.111 The FSS has also been considered
valid to measure fatigue in SLE.40, 111 Previous studies that have described
associations between the FSS and disease activity, organ damage, HRQL, oxygen uptake (aerobic capacity), age, and disease duration have made it
possible to determine and find support for construct validity of the FSS.36, 44, 45,
47-50, 52, 111
The FSS can discriminate between fatigue related to SLE and the
fatigue that a healthy person may experience.111 A total score of four or higher
(maximum is seven) has been considered as a cutoff level, e.g., the level of fatigue that discriminates between fatigue in SLE and fatigue reported by
recently been ensured by qualitative interviews with persons with SLE when
translating the FSS into Danish.130 In this thesis feasibility, ceiling-floor effects,
test-retest reliability, content and construct validity have been studied. Trustworthiness in qualitative studies
A common overarching concept for qualitative studies is trustworthiness, which
includes the concepts of credibility, dependability, confirmabilityand
transferability.131 Credibility covers all parts of the research process and deals
with the truthfulness of the study findings, that is the findings cover the multiple
realities of the study participants.132 Credibility includes a description of the data
collection method and the selection of participants.133 The selection of
participants in qualitative studies is usually purposive and strategic,132 which can
provide the researcher with a diversity of experiences concerning the study
question.132-134 The amount of data that needs to be collected depends on the
complexity of the research question; complex questions need greater amounts of
data.134 The setting for data collection should also be considered, since this can
influence the interview outcome.133 Further, credibility includes a well described
analysis process of the collected data.133 It also comprises how well the findings
cover the collected data.134 This can be illustrated by the use of representative
quotations from the interviews, to demonstrate the findings and interpretation of
the collected data.133, 134 Further, checking for agreement within the research
team, among experts and participants are other options that support
credibility.132, 134 Researchers with different professional backgrounds can
provide the study with different perspectives, which can increase the
credibility.132 Dependability concerns whether the data collection or analysis is
stable during the study process.134 Qualitative studies are associated with a view
of the reality as multiple and a study process which involves new insights
among the researchers during the course of the study.132 Discussions within the
research team concerning the consistency in sorting the data in similarities and
differences are one way to address changes of consistency over time.134
Confirmabilitycan be supported by participants’ recognition of study findings as well as by describing the researchers. The researchers can describe their
relationship with informants and their personal characteristics, such as occupation, gender and training to make it possible for the reader to judge the
potential influence of the researcher on the findings.133 Transferability refers to
the possibility of transferring the findings to others outside the study group. To be able to transfer results of new insights and understanding, the selection of informants, data collection, methods of analysis and context need to be well
Focus group interviews
People’s experiences can be highlighted in focus groups.114, 135-137
The focus group method is based on the belief that humans are influenced by their context
and those with whom they interact.138, 139 Focus groups can contribute to a social
context,140 where interaction and group dynamics can contribute to increase the
richness and amount of the data created.139, 141, 142 Another advantage of the use
of focus groups is the strengthening of participants’ roles in the interview situation, thereby reducing the influence of the researcher, since focus groups
are built on interaction among the informants.139 The presence of other
informants can also be supportive when discussing sensitive issues.141, 142 On the
other hand, the presence of others can also reduce the informants’ willingness to
reveal experiences when sensitive topics are discussed.141 Since focus groups are
known to potentially contribute to comprehensive information on functioning
and health from a patient perspective,143 focus group interviews were used in
Study II, III, and IV in this thesis. Content analysis
Data collected with qualitative methods can be analysed with different methods and qualitative approaches. Content analysis is one common method to analyse
focus group data.114, 144, 145 It is a flexible method which can provide broad and
condensed descriptions of the study phenomena.146 There are several forms of
content analysis,134, 146, 147 and one is qualitative content analysis. Qualitative
content analysis can be performed in an inductive or deductive manner. In the inductive analysis the categories are created from the data; the process starts in
the specific and items are combined into larger descriptions.146 The analysis
process in deductive analysis starts from an existing theory or model; in other
words, it starts as a whole and moves to the more specific.146 Further, the
analysis can be manifest and/or latent; both forms include interpretation but on
varying levels. Latent analysis involves a higher degree of interpretation.134, 148
Qualitative content analysis is related to communication theory.134 The data,
e.g., the interview text, is created in a communication between the informant and the researcher. There is also communication between the researcher and the text during the analysis process. Manifest analysis illustrates the content of the collected data and answers the question what, a description of the visible and
obvious content in the data.134, 148 The latent analysis answers the question how
and illustrates the relation in the data, the underlying meaning in the interview
text.134, 148 In this thesis inductive latent qualitative content analysis was used in
Study II. This is a common analysis method in interview studies in
Meaning condensation
Meaning condensation is another method that can be used to analyse interview data from focus groups. It is a method that can be used to condense interview transcripts to shorter formulations, taking the essential meaning of what was said
and rephrasing it into fewer words.149 A modified form of meaning condensation
has earlier been used in qualitative studies when analysing qualitative data from
focus groups.150-152 This analysis method has also been used in qualitative
multi-centre studies involving different countries.153, 154 This form of condensation has
been described by Stamm et al.154 as a modified form of content analysis. In this
thesis, modified meaning condensation was used in Study III.
Linking qualitative data to the ICF and literature review
Qualitative results, concepts obtained in focus groups, can be translated into the
unified ICF language by using the ICF linking rules developed by Cieza et al.155
These rules have been used in several qualitative studies with focus group when
linking qualitative results to the ICF framework.150-154 These linking rules were
used in Study III to describe the qualitative result to get an increased
understanding from a biopsychosocial perspective. Scientific knowledge can also be obtained by analysis of previously performed studies using a new
research question.156 Reviews can contribute to making knowledge available in a
more accessible form.156 In this thesis a literature review was performed to
METHODS Design
This thesis is based on four studies (Table 2). To answer the thesis aim, a method study (Study I) and qualitative studies with focus group interviews were performed (Study II, III and IV). Study IV used the same data collection as Study III. The data was re-analysed and a literature review was conducted according to a new aim. Study III and IV were included in a European research collaboration project entitled “Evaluation and Development of Clinical Outcome Measures and Instruments in Systemic Sclerosis from the Perspective of
Patients”.
Table 2. Participants, design and analysis in the included studies (n=63 persons with systemic lupus erythematosus, n=63 persons with systemic sclerosis)
Study Participants Design Analysis
I Sample 1, n=32
Sample 2, n=23
Method study - Statistics in line with classic test theory
and nonparametric statistics -Categorisation of patients’ written statements on the items and the questionnaire
II n=19a Qualitative
study with focus group interviews
-Inductive latent qualitative content
analysisb III n=20 Austria n=10 Romania n=17 Sweden n=16 Switzerland Multi-centre study with qualitative focus group interviews
-Meaning condensation (modified)c
-Meaning categorisation(modified)c -Qualitative result data were linked to the
ICF by the use of ICF linking rules
IV n=20 Austriad n=10 Romaniad n=17 Swedend n=16 Switzerlandd Multi-centre study with qualitative focus group interviews; Literature review
-Meaning condensation (modified)c
-Qualitative results data were sorted into a structure for personal factors by Geyh et al. -PROMs were analysed whether they covered personal factors found in the qualitative results data
a
11 of the participants in Study II were also included in Sample 2 in Study I.
b
According to Graneheim and Lundman.134
c
According to Kvale.149
d
Participants
Study I. Participants with SLE were recruited from outpatient clinics at
rheumatology departments at hospitals in the middle of Sweden (Sample 1) and in northern Sweden (Sample 2).
Sample 1 was examined at baseline, before participation in an evaluation of a
physical activity programme.157 Main inclusion criteria were SLE according to
ACR;29 women aged 18–70 years; stable; low to moderate disease activity; and
low organ damage, according to a rheumatologist’s evaluation. Main exclusion criteria were cardiovascular, pulmonary, or cerebrovascular problems or other conditions that could influence the physical activity programme. One hundred twenty-eight women were invited to participate in the physical activity programme; 40 accepted; 34 of them fulfilled study criteria; and, finally, 32 participated in Study I (Table 3). The construct validity of the FSS-Swe was tested in Sample 1.
In Sample 2, the main inclusion criteria were SLE, according to the ACR
criteria;29 ages between 18 and 70 years; stable; and low disease activity and
organ damage, according to a rheumatologist’s evaluation. The main exclusion criteria were the Systemic Lupus International Collaborating Clinics (ACR) – Damage Index (SLICC) score >6, coexisting diseases in an active phase or in a chronic symptom-giving stage, and a diagnosis of depression. Forty-four
patients were invited to the study and 27 of them agreed to participate. However, two persons did not meet the study criteria based on the rheumatologist’s
examination, and two withdrew later for personal reasons. Finally, 23 persons with SLE were included in the second sample of Study I (Table 3). Feasibility, ceiling and floor effects, and test-retest reliability, internal consistency, and the content validity of the FSS-Swe were tested in Sample 2.
Study II. Patients with established SLE attending an outpatient clinic at a rheumatology department in northern Sweden were recruited for Study II.
Patients with SLE, according to the ACR criteria,29 confirmed by a
rheumatologist, ≥18 years of ageand able to understand and speak Swedish were
included. Patients with acute-stage SLE, very severe SLE (e.g. end stage organ impairment), or severe cognitive impairment according to a rheumatologist’s examination were excluded. Fifty-two patients fulfilled inclusion criteria and 20 persons agreed to participate. One of these withdrew before the interviews for personal reasons, leaving 19 participants in Study II (Table 3).
Study III and IV. Patients with SSc according to ACR criteria (Preliminary criteria, 1980) were recruited from the outpatient clinics at rheumatology departments of the participating centres in Austria, Romania, Sweden and Switzerland. The recruitment of participants in each country followed the
maximum variation strategy158 with variations in age, sex, disease duration and diffuse/limited SSc. In total, 63 persons with SSc participated in Study III and IV (Table 3). In Sweden, 17 persons participated, these were recruited from two rheumatology departments, one in the southern part and one in the northern part of the country. In Sweden 30 patients met the criteria and were invited and 17 of them agreed to participate. In the other countries, Austria, Switzerland, and Romania, the participants were recruited in one centre in each county and eligible patients were invited to participate.
Table 3. Demographic data of participants in the studies
Study I Study II Study III
and IV
Gender women/men Sample 1, 32/0
Sample 2, 19/4 16/3 53/10 Age, years Median (min-max) Sample 1, 52.5 (30-69) Sample 2, 50 (24-68) 55 (27-80) 56a (30-85) Disease duration, years Median (min-max) Sample 1, 18.5 (2-53) Sample 2, 25 (4-40) 27 (7-34) 10a (0.5-48) Overall disease activity, SLEDAIb Median (min-max) (0-105) Sample 1, 1 (0-16)* Sample 2, 0 (0-2)* 0 (0-16) Not applicable Overall organ damage, SLICCc Median (min-max) (0-49) Sample 1, 0 (0-4) Sample 2, 1 (0-4) 2 (0-6) Not applicable a
Mean, bSLEDAI=Systemic Lupus Erythematosus Disease Activity Index, higher score indicates higher disease activity. *Modified SLEDAI, complement levels and anti-DNA antibody measurement were excluded (0-101), cSLICC=Systemic Lupus International Collaborating Clinics (American College of Rheumatology) Damage Index, higher score indicates more severe organ damage (0-49).
Data collection and procedure
Study I. The FSS was first translated into Swedish at the Karolinska University
Hospital/Karolinska Institutetby one physiotherapist (CB) with approval from
the original author, Dr. Lauren Krupp. The translation was evaluated by two rheumatologists, two physiotherapists, and one nurse, and minor revisions were made. Next, a bilingual translator back-translated the FSS from Swedish to English, and showed no important changes of content in the original English
FSS. In the next step, this Swedish version of the FSS was tested for content validity from the patients’ perspective in a pilot study (n=40). This pilot study was performed within the previously mentioned study (see page 23) concerning
evaluation of a physical activity programme.157 Participants with SLE completed
the Swedish translated FSS, and afterward they answered questions about whether items in the FSS were understandable and relevant and if there were any items they wanted to include or exclude. The comments were analysed and the FSS was revised accordingly. This version was recognised as the FSS-Swe 1.
During the course of the study, others developed and tested other Swedish translations of the FSS for other diagnoses. The FSS-Swe 1 was harmonised with these versions into one final version of the Swedish FSS (FSS-Swe 2). This involved minor changes in wording between the FSS-Swe 1 and the FSS-Swe 2. Two physiotherapists (MM, CB) and one language teacher conducted the analyses. Again, a bilingual translator (not the one who back-translated the FSS-Swe 1) back-translated the FSS-FSS-Swe 2, and no important changes in the content of the English version could be detected.
The construct validity of the FSS-Swe was tested in Sample 1. Possible
correlations between the FSS-Swe 1 and the following variables were examined:
The overall ongoing disease activity was assessed according to the Systemic
Lupus Erythematosus Disease Activity Index (SLEDAI) and the Systemic
Lupus Activity Measure (SLAM).159 A modified SLEDAI was used, from which
complement levels and anti-DNA antibody measurements were excluded. The participants rated their global disease activity on a Visual Analogue Scale (0-100 mm), which is included in the SLAM instrument (SLAM-VAS). Higher scores indicate higher disease activity. SLEDAI and SLAM are considered
reliable and valid in measuring disease activity in SLE.159
Overall organ damage was assessed according to SLICC.159 Higher scores
indicate more severe organ damage. SLICC was described as reliable and valid
in measuring organ damage in SLE.159
HRQL was assessed by the Swedish SF-36. The SF-36 covers eight domains:
physical function, physical role function, bodily pain, general health, vitality, social function, emotional role function, and mental health. A higher score indicates better function. The SF-36 in English is described as reliable and valid
in patients with SLE.160
Aerobic capacity (maximal voluntary oxygen uptake, VO2 max) was measured
in a maximal symptom-limited, bicycle ergometer exercise test, a test which is
considered to give reproducible results.161, 162
