Granulomatosis with polyangiitis (GPA) is a small vessel vasculitis associated with anti-neutrophil cytoplasmic antibodies (C-ANCA), specifically proteinase 3 (PR3) ANCA. GPA classically presents with multi-organ involvement. The first description of GPA defined a triad of features: 1) systemic necrotizing angiitis, 2) necrotizing inflammation of the respiratory tract and 3) necrotizing glomerulonephritis.1 Few cases of digital…
Defined by the presence of antiphospholipid antibodies (aPL) in individuals with clinical evidence of thrombosis or pregnancy morbidity, antiphospholipid syndrome (APS) is a systemic autoimmune thrombophilia. Clinical thrombosis, which should be confirmed by objective validated criteria (e.g., imaging studies or histopathology), can occur in the arterial, venous or small vessel vasculature and is not limited…
Patients in intensive care may experience dangerous manifestations of rheumatic disease, such as digital ischemia, multi-system organ failure and more. In a session at ACR Convergence 2021, experts addressed how to manage and treat these critically ill patients.