Hughes, M; Kahaleh, B; Denton, CP; Mason, JC; Matucci-Cerinic, M; (2021) ANCA in systemic sclerosis, when vasculitis overlaps with vasculopathy: a devastating combination of pathologies. Rheumatology , 60 (12) pp. 5509-5516. 10.1093/rheumatology/keab278.

Preview

Text Rheumatology-ANCA Review-accepted-UCL.pdf - Accepted Version Download (209kB) | Preview

Abstract

In patients with systemic sclerosis (SSc), the coexistence of ANCA-associated vasculitis (SSc-AAV) has been reported to be associated with a severe disease course, including significant pulmonary and renal involvement. The presence of ANCA is not uncommon in patients with SSc and therefore clinicians must maintain a high index of clinical suspicion about SSc-AAV. p-ANCA and anti-MPO antibodies are the most common antibodies observed. Patients typically present with clinical features of microscopic polyangiitis or renal-limited vasculitis There are multiple areas of potential interaction in the pathogenesis of SSc and AAV which can exacerbate/compound vascular disease. In addition, similar patterns of major internal organ involvement (e.g., lung and kidneys) are seen in both conditions. We highlight a diagnostic approach to SSc-AAV and the paucity of data to inform management. As such, SSc-AAV is typically treated as per isolated AAV which can potentially be hazardous in patients with SSc (e.g., the association between high-dose steroid and scleroderma renal crisis). We propose that this rare clinical entity warrants rigorous investigation including definition of a therapeutic strategy to ameliorate the potentially devastating combination of pathologies in SSc-AAV.

Download activity - last month

Export as JSONXMLCSV

Download activity - last 12 months

Export as CSVXMLJSON

Downloads by country - last 12 months

Export as JSONCSVXML

Archive Staff Only

View Item