Abstract Number: 0085 • ACR Convergence 2020
Classifications of Inflammatory Myopathies: Differentially Expressed Membrane-Bound Complement Regulators Allow Specific Patterns of Membrane Attack Complex Deposition
Background/Purpose: Idiopathic inflammatory myopathies (IIM) are a group of heterogeneous disorders that typically present with proximal muscle weakness. According to the classification criteria, IMs are…Abstract Number: 0262 • ACR Convergence 2020
SLE Pregnancies: C4 as Predictor of Flares and Adverse Pregnancy Outcomes
Background/Purpose: SLE pregnancies have an increased risk of Adverse Pregnancy Outcomes (APO). In clinical practice, low C3 and C4 levels are associated with active disease…Abstract Number: 0528 • ACR Convergence 2020
The Complex Relationship Between C4b Binding Protein, Warfarin and Antiphospholipid Antibodies
Background/Purpose: C4b Binding Protein (C4BP) is the main inhibitor of the classical complement pathway. Both β2glycoprotein-I (β2GPI), the main antigen in the antiphospholipid syndrome (APS),…Abstract Number: 0938 • ACR Convergence 2020
Low Preconceptional Complement Level Is Related with an Adverse Obstetric Outcome in a Multicentric Cohort of Pregnancy in Patients with APS and aPL Positivity
Background/Purpose: The role of complement in the aPL related pathology has been widely studied in animal models. aPL can induce fetal loss in experimental animals but…Abstract Number: 1510 • ACR Convergence 2020
Platelet-bound C4d Is Associated with Platelet Activation and Arterial Thrombotic Events
Background/Purpose: Platelets have a well-defined role in arterial thrombosis, and platelet-bound complement activation products (PC4d) correlate with vascular thromboses in patients with Systemic Lupus Erythematosus…Abstract Number: 1636 • ACR Convergence 2020
8 Years Follow-Up of a Novel Autoinflammatory Disease: CD59 Malfunction Causes Hemolytic Anemia, Recurrent Guillain-Barre Syndrome, and Strokes in Pediatric Populations and Respond Well to Eculizumab and Pozelimab
Background/Purpose: In 2013 we have described the first patients with a novel autoinflammatory disease manifested in 4 children with recurrent Guillain-Barre syndrome and hemolytic anemia…Abstract Number: 1670 • ACR Convergence 2020
Low Copy Number of Long C4 Genes Is a Genetic Risk Factor for Childhood Onset SLE (cSLE) but Is Associated with Higher Age of Disease Onset
Background/Purpose: Hypocomplementemia is a marked feature of systemic lupus erythematosus (SLE), which may be a result of consumption initiated by immune complexes between self-nuclear antigens…Abstract Number: 1791 • ACR Convergence 2020
Renal Tubular Complement C9 Deposition Is Associated with Renal Tubular Damage and Fibrosis in Lupus Nephritis
Background/Purpose: Tubulointerstitial damage in lupus nephritis (LN) is a strong predictor of progression to chronic kidney disease (CKD) and end stage renal disease (ESRD). While…Abstract Number: 642 • 2019 ACR/ARP Annual Meeting
Complement Deposition C4d on Platelets Is Associated with Vascular Events in Systemic Lupus Erythematosus
Background/Purpose: Complement components, including C4d, can be detected on the surface of activated platelets and they have been associated with vascular disease in systemic lupus…Abstract Number: 669 • 2019 ACR/ARP Annual Meeting
Complement Activation in Probable Systemic Lupus Erythematosus (pSLE) May Predict Progression to SLE Defined by Fulfillment of ACR Classification Criteria
Background/Purpose: We reported (Ramsey-Goldman et al., Arthritis Rheumatol 2018: 70 [suppl 10]) that cell-bound complement activation products (CB-CAPs) and a multi-analyte assay panel with algorithm…Abstract Number: 686 • 2019 ACR/ARP Annual Meeting
Association Between the Soluble Terminal Complement Complex C5b-9 (sC5b-9) and Signs of Active Kidney Disease in a Swiss SLE Cohort
Background/Purpose: There is a lack of reliable biomarkers for disease activity in SLE. While C3a, an anaphylatoxin generated during of complement activation, could be predictive…Abstract Number: 1266 • 2019 ACR/ARP Annual Meeting
AGBL3 as a Novel Gene Associated with Hereditary Hypocomplementemic Urticarial Vasculitis and Favorable Response to Rituximab
Background/Purpose: Urticarial skin lesions are well-known features of autoinflammatory disorders associated with NLRP3 and NLRP12 variants. However hereditary forms of hypocomplementemic urticarial vasculitis (HUV) with…Abstract Number: 1825 • 2019 ACR/ARP Annual Meeting
Comparison of the Thrombosis Risk Score with Triple Positivity in SLE Thrombosis
Background/Purpose: We previously developed a Thrombosis Risk Score, a sum of three factors: lupus anticoagulant (by RVVT confirm); low C3; and C4d bound to platelets.…Abstract Number: 2887 • 2019 ACR/ARP Annual Meeting
Cell-bound Complement Activation Products in Combination with Low Complement C3 or C4 Have Superior Diagnostic Performance in Systemic Lupus Erythematosus
Background/Purpose: Cell-bound complement activation products (CB-CAPs) are stable forms of classical complement activation ex-vivo, with high sensitivity and specificity for systemic lupus erythematosus (SLE). We…Abstract Number: 2030 • 2018 ACR/ARHP Annual Meeting
Patients with Childhood-Onset SLE (cSLE) and Hypertension Have Consistently Higher Serum Concentrations of C3 and C4 Than Those without Hypertension
Background/Purpose: Chronic systemic inflammation, mediated in part by complement, and inadequate vascular repair mechanisms affect patients with cSLE from a young age and are associated…
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