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


Rheumatology. 2022;61(4):1476-1486. 

In This Article

Abstract and Introduction


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.


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.