Systemic sclerosis: state of the art on clinical practice guidelines

Abstract

Systemic sclerosis (SSc) is an orphan disease characterised by autoimmunity, fibrosis of the skin and internal organs, and vasculopathy. SSc may be associated with high morbidity and mortality. In this narrative review we summarise the results of a systematic literature research, which was performed as part of the European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases project, aimed at evaluating existing clinical practice guidelines or recommendations. Only in the domains ‘Vascular & Ulcers’ (ie, non-pharmacological approach to digital ulcer), ‘PAH’ (ie, screening and treatment), ‘Treatment’ and ‘Juveniles’ (ie, evaluation of juveniles with Raynaud’s phenomenon) evidence-based and consensus-based guidelines could be included. Hence there is a preponderance of unmet needs in SSc referring to the diagnosis and (non-)pharmacological treatment of several SSc-specific complications. Patients with SSc experience significant uncertainty concerning SSc-related taxonomy, management (both pharmacological and non-pharmacological) and education. Day-to-day impact of the disease (loss of self-esteem, fatigue, sexual dysfunction, and occupational, nutritional and relational problems) is underestimated and needs evaluation.

Clinical practice guidelines adherence, knowledge and awareness in rare and complex connective tissue diseases across Europe: results from the first ERN ReCONNET survey

“…Since ERN ReCONNET aims at improving the level of adherence to CPGs across European HCPs, by providing practical tools and training to healthcare professionals, many of the initiatives emerging from this survey are being realised under the scope of the ERN ReCONNET, following the suggestions, expectations and points of view of the member HCPs and ePAGs. The ERN ReCONNET has already reviewed the landscape of the existing CPGs in rCTDs [10][11][12][13][14][15][16][17][18][19] and the existence of partially sufficient CPGs for some rCTDs was highlighted, while demonstrating the absence of evidence-based CPGs for other rCTDs. [10][11][12][13][14][15][16][17][18][19] Among the different unmet needs on CPGs, the results raised the issue that more attention should be devoted to the assessment of CPGs adherence in routine clinical practice.…”

Monitoring the microcirculation in the diagnosis and follow‐up of systemic sclerosis patients: Focus on pulmonary and peripheral vascular manifestations

“…8,9 Therefore, primary RP patients should have a careful capillaroscopic analysis every 6-12 months, in order detect the first signs of any transition to the secondary form of RP, in the most reliable manner. 7,8,12 Nailfold videocapillaroscopy images in secondary RP are characterized by morphological signs that represent the microvascular damage. These include giant capillaries, microhemorrhages, capillary loss, the presence of avascular areas, and angiogenesis.…”

Digital ulcers: should debridement be a standard of care in systemic sclerosis?

“…A major challenge is that at present there is no strong SScspecific evidence base for DU local wound care. 33 However, valuable guidance on the management of CTD/vasculitis-associated skin ulcers including local wound care have been proposed. 34 In general, current management is largely based on expert opinion/experience and revolves around lessons learned from ulcer wound care in other common ulcer patient groups (e.g.…”