BSR and BHPR guideline for the treatment of systemic sclerosis

Abstract

This is a repository copy of BSR and BHPR guideline for the treatment of systemic sclerosis.

Early diagnosis and management of systemic sclerosis

“…First-and second-line treatment options for systemic sclerosis. 9 Some of these treatments can potentially be initiated in primary care (eg drug therapy for Raynaud's phenomenon and proton-pump inhibitors for reflux disease). Scleroderma renal crisis is a medical emergency that requires immediate specialist input.…”

“…Up to 80% of patients with SSc may develop lung fibrosis, and this is clinically significant in around one-third. 9 Around 10% of patients with SSc may develop PAH, 10 and this has a significant impact on survival. Three-year survival in SSc patients with PAH has been reported to be 56% compared to 94% in those without PAH.…”

“…27 Patients with both abnormalities were 60 times more likely to develop SSc. Figure 3 presents an overview of the British Society of Rheumatology/British Health Professionals in Rheumatology (BSR/BHPR) guideline for the treatment of SSc, 9 which is a useful reference tool for clinicians in both primary and secondary care alike. A key point to highlight is the importance of distinguishing between the limited and diffuse subsets of SSc, as this has an important impact on the likely disease course including prognosis, and in making treatment decisions.…”

Dissecting the enigma of scleroderma: possible involvement of the kynurenine pathway

“…Current recommended management is via nontargeted immunosuppression with the best evidence for corticosteroids, methotrexate and mycophenylate mofetil [81]. Targeting the KP may provide additional and more targeted therapy for this group of disorders.…”

Diagnosis and treatment of interstitial lung disease in scleroderma systematica

“…Уже после выхода обновленных рекомендаций появились результаты закончившихся клинических испытаний имму-носупрессивной терапии, а также новые данные об анти-фиброзных препаратах. В 2016 г. были опубликованы реко-мендации Британской ассоциации ревматологов [18], а в 2017 г. -отечественные клинические рекомендации по ревматологии, которые составлены с учетом международ-ных рекомендаций [19].…”