Evidence and Consensus-Based Recommendations for Non-Pharmacological Treatment of Fatigue, Hand Function Loss, Raynaud's Phenomenon and Digital Ulcers in Patients With Systemic Sclerosis

Juliane K. Stöcker; Anne A. Schouffoer; Julia Spierings; Marisca R. Schriemer; Judith Potjewijd; Lian de Pundert; Frank H.J. van den Hoogen; Maria W.G. Nijhuis-van der Sanden; J. Bart Staal; Ton Satink; Madelon C. Vonk; Cornelia H.M. van den Ende

Disclosures

Rheumatology. 2022;61(4):1476-1486.

Abstract and Introduction

Abstract

Objective: SSc is a complex CTD affecting mental and physical health. Fatigue, hand function loss, and RP are the most prevalent disease-specific symptoms of systemic sclerosis. This study aimed to develop consensus and evidence-based recommendations for non-pharmacological treatment of these symptoms.

Methods: A multidisciplinary task force was installed comprising 20 Dutch experts. After agreeing on the method for formulating the recommendations, clinically relevant questions about patient education and treatments were inventoried. During a face-to-face task force meeting, draft recommendations were generated through a systematically structured discussion, following the nominal group technique. To support the recommendations, an extensive literature search was conducted in MEDLINE and six other databases until September 2020, and 20 key systematic reviews, randomized controlled trials, and published recommendations were selected. Moreover, 13 Dutch medical specialists were consulted on non-pharmacological advice regarding RP and digital ulcers. For each recommendation, the level of evidence and the level of agreement was determined.

Results: Forty-one evidence and consensus-based recommendations were developed, and 34, concerning treatments and patient education of fatigue, hand function loss, and RP/digital ulcers-related problems, were approved by the task force.

Conclusions: These 34 recommendations provide guidance on non-pharmacological treatment of three of the most frequently described symptoms in patients with systemic sclerosis. The proposed recommendations can guide referrals to health professionals, inform the content of non-pharmacological interventions, and can be used in the development of national and international postgraduate educational offerings.

Introduction

SSc is a complex, chronic and incurable CTD characterized by diffuse microangiopathy and immune dysregulation, ultimately leading to widespread skin and internal organ fibrosis.^[1] Its prevalence is estimated to be 23 per 100 000 people.^[2] The consequences of this complex disease significantly adversely affect both mental and physical health.^[3] Fatigue, hand function loss and RP, which often lead to digital ulcers (DU), are the most prevalent disease-specific symptoms of limited (lcSSc) and diffuse cutaneous SSc (dcSSc) according to SSc patients.^[4–6] All three symptoms interfere, to varying degrees, with the performance of everyday tasks and have a major impact on health-related quality of life (HRQoL).^[7–10] Fatigue significantly affects patients' ability to fulfil social roles, RP and DU are associated with significant pain and disability, and hand function loss worsens the ability to perform meaningful activities of daily living.^[10–15]

In the Netherlands, SSc care delivered by rheumatologists and nurses is offered in hospitals and rehabilitation centres; while care delivered by health professionals like physiotherapists, occupational therapists and psychologists is predominantly delivered in primary care settings. Depending on the nature of the patient's condition, his or her specific needs and the availability of caregivers at an institution or in the area, delivery of care includes, in addition to treatment by medical specialists, continuous or intermittent involvement of health professionals (HPRs).^[16] HPRs from different professions can be involved in the non-pharmacological treatment of patients with SSc-induced fatigue, hand function loss and RP/DU. In addition to rheumatologists and specialized nurses, there is a role for physiotherapists, occupational therapists, psychologists, dieticians and social workers. In clinical practice, the HPR treatment offer varies, as well as the content of the treatments, and there is little evidence available regarding HPR treatment for SSc-induced fatigue, hand function loss and RP/DU thus far.^[17–19] Although HPRs offer numerous treatments to satisfy the unmet care needs of patients with SSc, and these patients are satisfied with the content and results of HPR treatments, fatigue, hand function loss and RP are uncommon reasons for referral to HPRs.^[18,20,21] In a previous study, we found that rheumatologists are reluctant to refer their patients to HPRs due to a poor overview of HPR treatment options and a lack of published evidence.^[22] Existing SSc guidelines and recommendations do not include recommendations regarding non-pharmacological care or only superficially include them. Specific recommendations on non-pharmacological treatment approaches for patients with SSc are not yet available.^[23]

HPR recommendations not only could support HPRs in SSc treatment, but also could provide clinicians with guidance on timely referrals and access to adequate care for patients with SSc, fatigue, hand function loss and RP/DU. To address this need, this study aimed to develop HPR recommendations for the management and treatment of fatigue, hand function loss and RP/DU in patients with SSc. A multidisciplinary task force has been assembled to develop these recommendations based on evidence and consensus. These recommendations are targeted at all HPRs in the field of non-pharmacological SSc care and are potentially relevant to key stakeholders, namely SSc patients, as well as their patient organizations, rheumatologists and other (medical) care providers.

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Abstract and Introduction
Methods
Results
Discussion
Conclusion
References
Sidebar

Table 1. Recommendations on patient education and treatments for systemic sclerosis patients with fatigue
		Level of evidence	Reference	Level of Agreement (0–10) Mean (S.D.); Median (range)
PATIENT EDUCATION
Persistent fatigue can have far-reaching consequences for activities of daily living and social participation. It is therefore essential that all patients with SSc who report symptoms of fatigue are properly informed about measures that support self-management skills. All SSc patients who report fatigue should receive patient information about the following aspects.
1.	Maintaining good physical condition and regular exercise	I	[35–37]	9.3 (0.8) 9 (8–10)
2.	Principles of energy conservation and good sleep hygiene	I	[36, 38]	8.8 (1.0) 9 (7–10)
3.	Relaxation exercises	I	[38]	7.5 (1.7) 7 (4–10)
4.	A healthy diet	V	n/a	7.9 (1.8) 8 (2–10)
5.	The possible link between fatigue and drug side effects	V	n/a	7.2 (2.1) 8 (2–10)
TREATMENTS
a) Treatments for SSc patients with persistent fatigue and related restrictions in whose activities of daily living and who have an imbalance between mental load and mental resilience
6.	Psychoeducational interventions (individually or in a group) aimed at principles of goal setting, energy conservation, dealing with the social environment and relaxation should be offered to SSc patients with fatigue. These interventions can be performed by a skilled health professional, e.g. a nurse, social worker or occupational therapist.	I	[36, 38, 39]	8.7 (1.1) 9 (7–10)
7.	CBT under the supervision of a psychologist should be offered to SSc patients with fatigue, if there are severe impediments to activities of daily living.	I	[38–40]	8.2 (1.2) 8.0 (6–10)
8.	Participation in available online and face-to-face courses with fellow patients, provided by trained patient representatives: e.g. ReumaUitgedaagd! (self-management training for people with rheumatism) should be offered to patients with SSc with fatigue.	V	n/a	8 (1.3) 8 (5–10)
9.	In order to maintain the ability to work, SSc patients with fatigue should be guided in adapting the work environment or switching to different work by a skilled health professional, e.g. an occupational therapist or social worker.	V	n/a	8.3 (1.2) 8.0 (5–10)
b) Treatments for SSc patients with persistent fatigue where reduced physical resilience plays a role
10.	SSc patients with fatigue should receive support to improve exercise capacity and incorporate more physical activity into daily life with the guidance of health professionals such as physical therapists.	I	[35–38, 41–43]	9.1 (1.0) 9 (7–10)
11.	Advice about a healthy diet and preventing malnutrition offered by e.g. a dietician should be offered to SSc patients with fatigue.	V	n/a	7.9 (1.2) 8 (6–10)
c) Multidisciplinary treatments
12.	A multidisciplinary rehabilitation program should be offered to SSc patients with severe fatigue symptoms that lead to problems in several domains of activities of daily living.	V	n/a	8.3 (1.6) 9 (3–10)

Level of evidence (according to the standards of the Oxford Centre for Evidence Based Medicine), Level of Agreement for the recommendations, Numeric Rating Scale from 0 (total disagreement) to 10 (total agreement) reported as mean (range), n/a not applicable. CBT: cognitive behavioural therapy.

Table 2. Recommendations on patient education and treatments for systemic sclerosis patients with hand function loss
		Level of evidence	Reference	Level of Agreement (0–10) Mean (S.D.); Median (range)
PATIENT EDUCATION
Provide patient education and advice to all SSc patients with hand function loss to enhance their knowledge about treatment options and promote self-management. The patient education and advice should be tailored to the individual patient but should contain the following aspects.
13.	Independently and regularly doing hand exercises to maintain hand mobility and strength.	II	[19, 44–47]	9.1 (0.9) 9.0 (7–10)
14.	A continuous use of hands in activities of daily living to maintain hand functionality.	V	n/a	8.7 (1.3) 9 (5–10)
15.	Avoiding cold and keeping the hands warm.	IV	[48]	9.0 (0.8) 9 (8–10)
16.	Good hand care, for example by moisturizing the skin (especially with lanolin-based products) and wearing protective gloves.	IV	[48]	8.3 (1.2) 8 (5–10)
TREATMENTS
a) Treatments for SSc patients whose activities of daily living are restricted due to limitations in hand function
17.	Passive and active hand function exercises to promote hand mobility, functionality and strength, under the guidance of a skilled health professional (e.g. a hand therapist), should be offered to SSc patients who experience restrictions in the performance of daily activities due to hand function loss.	II	[19, 44–47]	8.5 (0.9) 8.0 (7–10)
18.	Learning ergonomic measures under the guidance of a health professional such as an occupational therapist should be offered to SSc patients who experience restrictions in the performance of daily activities due to hand function loss.	I	[45, 49]	8.4 (1.1) 8 (7–10)
19.	The adaption of hobbies and work (including volunteer work) to enable participation in meaningful activities of daily living, under the guidance of a health professional, e.g. a social worker or occupational therapist, should be offered to SSc patients who experience hand function loss.	II	[47]	7.9 (1.2) 8 (5–10)
b) Multidisciplinary treatments:
20.	A multidisciplinary rehabilitation should be offered to SSc patients with hand disabilities that lead to problems in multiple domains of activities of daily living.	II	[19, 47]	8.0 (1.4) 8 (3–10)

Table 3. Recommendations on patient education and treatments for systemic sclerosis patients with RP and/or digital ulcers
		Level of Evidence	Reference	Level of Agreement (0–10) Mean (S.D.); Median (range)
PATIENT EDUCATION
All SSc patients with problems due to Raynaud's phenomenon and/or digital ulcers should receive patient education about the following aspects.
21.	Quitting smoking.	IV	[50]	9.4 (0.9) 10 (7–10)
22.	Avoiding triggers that can elicit an attack of Raynaud's, such as sudden changes in temperature, drinking large amounts of coffee or energy drinks, and stress.	V	[50]	9.0 (1.2) 9.0 (5–10)
23.	Practical advice for protection against cold and avoiding temperature differences: use of special clothing, silver gloves, heated gloves, or heating pads; drying the skin thoroughly after showering or washing hands; (iii) avoiding contact with cold objects (wearing gloves to remove items from the fridge/freezer, using a heated keyboard and mouse).	IV	[50]	8.9 (1.1) 9 (6–10)
24.	Wearing fingertip protection (e.g. thermoplastic material or neoprene) to prevent pain when performing actions that cannot be avoided.	V	n/a	7.8 (1.9) 8 (3–10)
25.	Preventing infections and wounds through good hygiene, avoiding bruising and hazardous work, and using gloves for protection.	V	n/a	8.7 (1.7) 9 (3–10)
26.	Promoting good blood circulation through the use of a stress ball and having sufficient exercise throughout the day.	V	n/a	7.4 (2.3) 8 (1–10)
27.	Avoiding prolonged, static postures.	V	n/a	7.3 (2.2) 8 (1–10)
In SSc patients with digital ulcers, attention should also be paid to the following aspects.
28.	Hydration of the skin around the ulcers with products based on lanolin, petroleum jelly or cetomacrogol.	IV	[48]	8.0 (1.6) 8 (4–10)
29.	Avoiding frequent exposure to water with aggressive cleaning agents.	IV	[48]	8.1 (1.7) 8 (3–10)
30.	Avoiding finger punctures.	V	n/a	8.0 (1.8) 8 (4–10)
31.	Avoiding manipulation of ulcers (e.g. by squeezing out calcium deposits or cutting away hard skin).	V	n/a	8.3 (1.9) 9 (4–10)
TREATMENTS
Treatments for SSc patients whose activities of daily living are restricted due to Raynaud's phenomenon and/or digital ulcers.
32.	Exercise therapy (with an arm bicycle) to promote general blood circulation and support the integration of exercise activities in daily life, guided by a health professional (e.g. a physiotherapist), should be offered to SSc patients with Raynaud's phenomenon/digital ulcers.	III	[51]	7.4 (1.7) 8 (2–10)
33.	In case of vasculopathy of the feet, advice about suitable, non-restrictive footwear (for indoor and outdoor use), by a health professional such as, for example, a podiatrist, should be offered to SSc patients.	V	n/a	7.8 (1.9) 8 (1–10)
34.	Advice on the protection of the fingertips with special gloves or by using adaptive devices, provided by a health professional (e.g. an occupational therapist), should be offered to SSc patients whose activities of daily living are restricted due to Raynaud's phenomenon/digital ulcers.	V	n/a	8.2 (1.7) 9.0 (3–10)

Table 4. Draft recommendations excluded from the final recommendations through determination of the level of agreement
		Level of Evidence	Reference	Level of Agreement (0–10) Mean (S.D.); Median (range)
FATIGUE—Patient education
*1	The beneficial effect of yoga and tai chi on general physical condition and relaxation.	I	[35, 36]	6.6 (1.7) 7 (4–10)
HAND FUNCTION LOSS—Treatments
*2	Manual lymph drainage by a skilled health professional such as a physiotherapist, occupational therapist or hand therapist in SSc patients with finger or hand oedema.	II	[19]	6.4 (2.0) 7 (2–10)
*3	Connective tissue massage (possibly in combination with passive and active hand exercises).	II	[19, 52]	6.7 (1.6) 7 (3–10)
*4	Avoid wearing dynamic finger splints for the purpose of reducing contractures of the proximal interphalangeal joints by stretching the connective tissue, due to the potential lack of effect and adverse patient outcomes.	III	[53]	6.1 (2.6) 6 (1–10)
RP AND DIGITAL ULCERS—Patient education
*5	Avoiding exposure to vibrations, which can adversely affect blood flow.	IV	[50]	7.4 (2.0) 7 (1–10)
*6	The possibly beneficial effect of soda baths (no more than twice a week) to prevent dehydration and cracking.	IV	[48]	6.7 (2.3) 7 (1–10)
*7	The possible importance of a healthy diet with adequate fat intake.	V	n/a	6.7 (2.4) 7 (1–10)

Level of evidence according to the standards of the Oxford Centre for Evidence Based Medicine, Level of Agreement for the recommendations, Numeric Rating Scale from 0 (total disagreement) to 10 (total agreement) reported as mean (range), n/a not applicable, *1–7 excluded from the final recommendations.