Update of EULAR recommendations for the treatment of systemic sclerosis

Abstract

The aim was to update the 2009 European League against Rheumatism (EULAR) recommendations for the treatment of systemic sclerosis (SSc), with attention to new therapeutic questions. Update of the previous treatment recommendations was performed according to EULAR standard operating procedures. The task force consisted of 32 SSc clinical experts from Europe and the USA, 2 patients nominated by the pan-European patient association for SSc (Federation of European Scleroderma Associations (FESCA)), a clinical epidemiologist and 2 research fellows. All centres from the EULAR Scleroderma Trials and Research (EUSTAR) group were invited to submit and select clinical questions concerning SSc treatment using a Delphi approach. Accordingly, 46 clinical questions addressing 26 different interventions were selected for systematic literature review. The new recommendations were based on the available evidence and developed in a consensus meeting with clinical experts and patients. The procedure resulted in 16 recommendations being developed (instead of 14 in 2009) that address treatment of several SSc-related organ complications: Raynaud's phenomenon (RP), digital ulcers (DUs), pulmonary arterial hypertension (PAH), skin and lung disease, scleroderma renal crisis, and gastrointestinal involvement. Compared with the 2009 recommendations, the 2015 recommendations include phosphodiesterase type 5 (PDE-5) inhibitors for the treatment of SSc-related RP and DUs, riociguat, new aspects for endothelin receptor antagonists, prostacyclin analogues and PDE-5 inhibitors for SSc-related PAH. New recommendations regarding the use of fluoxetine for SSc-related RP and haematopoietic stem cell transplantation for selected patients with rapidly progressing SSc were also added. In addition, several comments regarding other treatments addressed in clinical questions and suggestions for the SSc research agenda were formulated. These updated data-derived and consensus-derived recommendations will help rheumatologists to manage patients with SSc in an evidence-based way. These recommendations also give directions for future clinical research in SSc.

Diagnosis and treatment of interstitial lung disease in scleroderma systematica

“…[17]. Как и в версии EULAR 2009 г., эти рекомендации сгруппированы по системам органов или наиболее значи-мым клиническим синдромам, включая ИПЛ.…”

Systemic sclerosis – diagnostic and therapeutic recommendations of the Polish Dermatological Society. Part 1: diagnosis and monitoring

“…The complex and diverse clinical features seen in patients require case-by-case approach and cooperation of multiple specialists both at the stage of diagnosis and treatment. Critical factors in SSc include early diagnosis, assessment of internal organ involvement, identification of patients at potential risk of organ complications, assessment of disease dynamics and activity, and implementation of optimal therapy [2,3]. The study presents current recommendations for the diagnostics and monitoring of patients.…”

“…Złożo-ny i zróżnicowany obraz kliniczny wymaga indywidualnego podejścia i współpracy wielospecjalistycznej zarówno na etapie diagnozowania, jak i leczenia chorych. W SSc ważne są wczesne rozpoznanie, ocena zajęcia procesem chorobowym narządów wewnętrznych, identyfikacja chorych z ryzykiem wystąpienia powikłań narządowych, ocena dynamiki i aktywności choroby oraz wdrożenie optymalnej terapii [2,3]. W pracy przedstawiono aktualne rekomendacje dotyczące rozpoznawania, diagnostyki i monitorowania chorych.…”

Systemic sclerosis – diagnostic and therapeutic recommendations of the Polish Dermatological Society. Part 2: treatment

“…Twardzina układowa (systemic sclerosis -SSc) jest chorobą mediowaną immunologicznie o przewlekłym, postępującym przebiegu, która prowadzi do niewydolności wielonarządowej, niepełnosprawności i przyczynia się do znacznego obniżenia jakości życia [1]. Procesem chorobowym zajęta jest skóra, tkanka podskórna, mięśnie, układ kostno-stawowy oraz narządy wewnętrzne.…”

“…introduction Systemic sclerosis (SSc) is an immune-mediated disease with a chronic progressive course leading to multiorgan failure and patient disability, and contributing to a significant impairment in the quality of life [1]. The disease process involves the skin, subcutaneous tissue, muscles, osteoarticular system and internal organs.…”