Objectives To examine the treatment decision-making process of patients with dcSSc in the context of haematopoietic stem cell transplantation (HSCT). Methods A qualitative semi-structured interview study was done in patients before or after HSCT, or patients who chose another treatment than HSCT. Thematic analysis was used. Shared decision-making (SDM) was assessed with the 9-item Shared Decision Making Questionnaire (SDM-Q-9). Results Twenty-five patients [16 male/nine female, median age 47 (range 27–68) years] were interviewed: five pre-HSCT, 16 post-HSCT and four following other treatment. Whereas the SDM-Q-9 showed the decision-making process was perceived as shared [median score 81/100 (range 49–100)], we learned from the interviews that the decision was predominantly made by the rheumatologist, and patients were often steered towards a treatment option. Strong guidance of the rheumatologist was appreciated because of a lack of accessible, reliable and SSc-specific information, due to the approach of the decision-making process of the rheumatologist, the large consequence of the decision and the trust in their doctor. Expectations of outcomes and risks also differed between patients. Furthermore, more than half of patients felt they had no choice but to go for HSCT, due to rapid deterioration of health and the perception of HSCT as ‘the holy grail’. Conclusion This is the first study that provides insight into the decision-making process in dcSSc. This process is negatively impacted by a lack of disease-specific education about treatment options. Additionally, we recommend exploring patients’ preferences and understanding of the illness to optimally guide decision-making and to provide tailor-made information.

Systemic sclerosis (SSc) is a highly heterogeneous disease caused by a complex molecular circuitry. For decades, clinical and molecular research focused on understanding the primary process of fibrosis. More recently, the inflammatory, immunological and vascular components that precede the actual onset of fibrosis, have become a matter of increasing scientific scrutiny. As a consequence, the field has started to realize that the early identification of this syndrome is crucial for optimal clinical care as well as for understanding its pathology. The cause of SSc cannot be appointed to a single molecular pathway but to a multitude of molecular aberrances in a spatial and temporal matter and on the backbone of the patient’s genetic predisposition. These alterations underlie the plethora of signs and symptoms which patients experience and clinicians look for, ultimately culminating in fibrotic features. To solve this complexity, a close interaction among the patient throughout its “journey,” the clinician through its clinical assessments and the researcher with its experimental design, seems to be required. In this review, we aimed to highlight the features of SSc through the eyes of these three professionals, all with their own expertise and opinions. With this unique setup, we underscore the importance of investigating the role of environmental factors in the onset and perpetuation of SSc, of focusing on the earliest signs and symptoms preceding fibrosis and on the application of holistic research approaches that include a multitude of potential molecular alterations in time in an unbiased fashion, in the search for a patient-tailored cure.

BackgroundSystematic Lupus Erythematosus (SLE) is a systemic inflammatory autoimmune disease with a heterogeneous presentation in which almost every organ can be affected1. SLE patients experience a lower health-related quality of life, due to variation of disease severity over time, arthritis, arthralgia, skin abnormalities, myalgia and general fatigue2. The treatment and support of patients with SLE is carried out by a multidisciplinary team, composed of rheumatologists, clinical immunologists, nurse practitioners and rheumatology nurses. Although nurses have a major task concerning providing information, support, and education in SLE care3, little is known about how patients experience nursing care and what needs and expectations they have.ObjectivesTo investigate the patients’ needs for nursing support in order to optimise and standardise nursing care in a SLE clinical pathway.MethodsTo identify specific factors regarding nursing care for SLE patients, a literature search was performed. Subsequently, semi-structured interviews were held among patients with SLE from the department Rheumatology and Clinical Immunology of the University Medical Centre Utrecht (tertiary care referral centre with approx. 300 SLE pts/year). The interviews focused on patients’ needs, quality of life and nursing care. The questions regarding quality of life were partially derived of the SLE QoL Questionnaire4 and focused on; daily-, social- or occupational activities, symptoms, medical treatment, and negative emotions. Interviews were recorded, transcribed and analysed with thematic analysis by the researchers and patient partners.ResultsSeveral tools to explore individual needs among patients with SLE, such as the Dutch version of the Educational Needs Assessment Tool (D-ENAT), were identified5. Eight SLE patients were interviewed (female n=6, average age 37.5 years). All were using a DMARD and/or biological. Patients indicated a need for help with problems in daily life, information regarding SLE, peer support, and psychosocial help. Pain and fatigue were the most commonly reported symptoms. Most patients saw their nurse on an irregular basis. They appreciated the accessibility, accuracy, clarity, and patience of nurses.ConclusionsThere is a need for individualised nursing support in dealing with SLE. To assess individual needs among patients, a needs assessment tool could be used. Further research on the usefulness and effectiveness of a needs assessment tool in daily clinical practice is needed.References[1] Rahman A, Isenberg DA. (2008) Systemic lupus erythematosus. N Engl J Med. 358(9):929–39.[2] Morgan C, Bland AR, Maker C, Dunnage J, Bruce IN. (2018) Individuals living with lupus: findings from the LUPUS UK Members Survey 2014. Lupus, 0,1–7.[3] Ferenkeh-Koroma A. (2012) Systemic Lupus Erythematosus: nurse and patient education. Nursing Standard, 50–59.[4] Leong KP, Kong KO, Thong B, Koh E, Lian T, et al. (2005) Development and preliminary validation of a systemic lupus erythematosus-specific quality-of-life instrument (SLEQOL)...