ANCA in systemic sclerosis, when vasculitis overlaps with vasculopathy: a devastating combination of pathologies.
Rheumatology (Oxford)
; 60(12): 5509-5516, 2021 12 01.
Article
em En
| MEDLINE
| ID: mdl-33744916
ABSTRACT
In patients with 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-myeloperoxidase 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. due to 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.
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Base de dados:
MEDLINE
Assunto principal:
Escleroderma Sistêmico
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Doenças Vasculares
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Anticorpos Anticitoplasma de Neutrófilos
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Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos
Tipo de estudo:
Diagnostic_studies
Limite:
Humans
Idioma:
En
Ano de publicação:
2021
Tipo de documento:
Article