Acquired activated protein C resistance is associated with lupus anticoagulants and thrombotic events in pediatric patients with systemic lupus erythematosus

Blood ◽  
2001 ◽  
Vol 97 (4) ◽  
pp. 844-849 ◽  
Author(s):  
Christoph Male ◽  
Lesley Mitchell ◽  
James Julian ◽  
Patricia Vegh ◽  
Penny Joshua ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Protein C ◽  
Pediatric Patients ◽  
Activated Protein C ◽  
Factor V Leiden ◽  
Protein S ◽  
Activated Protein C Resistance ◽  
Systemic Lupus ◽  
Lupus Anticoagulants

Abstract Acquired activated protein C resistance (APCR) has been hypothesized as a possible mechanism by which antiphospholipid antibodies (APLAs) cause thrombotic events (TEs). However, available evidence for an association of acquired APCR with APLAs is limited. More importantly, an association of acquired APCR with TEs has not been demonstrated. The objective of the study was to determine, in pediatric patients with systemic lupus erythematosus (SLE), whether (1) acquired APCR is associated with the presence of APLAs, (2) APCR is associated with TEs, and (3) there is an interaction between APCR and APLAs in association with TEs. A cross-sectional cohort study of 59 consecutive, nonselected children with SLE was conducted. Primary clinical outcomes were symptomatic TEs, confirmed by objective radiographic tests. Laboratory testing included lupus anticoagulants (LAs), anticardiolipin antibodies (ACLAs), APC ratio, protein S, protein C, and factor V Leiden. The results revealed that TEs occurred in 10 (17%) of 59 patients. Acquired APCR was present in 18 (31%) of 58 patients. Acquired APCR was significantly associated with the presence of LAs but not ACLAs. Acquired APCR was also significantly associated with TEs. There was significant interaction between APCR and LAs in the association with TEs. Presence of both APCR and LAs was associated with the highest risk of a TE. Protein S and protein C concentrations were not associated with the presence of APLAs, APCR, or TEs. Presence of acquired APCR is a marker identifying LA-positive patients at high risk of TEs. Acquired APCR may reflect interference of LAs with the protein C pathway that may represent a mechanism of LA-associated TEs.

Acquired activated protein C resistance is associated with IgG antibodies to protein S in patients with systemic lupus erythematosus

2009 ◽  
Vol 124 (1) ◽  
pp. 127-131 ◽  
Author(s):  
Junzo Nojima ◽  
Yoshinori Iwatani ◽  
Kiyoshi Ichihara ◽  
Hidehiro Tsuneoka ◽  
Toshizo Ishikawa ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Protein C ◽  
Activated Protein C ◽  
Protein S ◽  
Igg Antibodies ◽  
Activated Protein C Resistance ◽  
Systemic Lupus

scholarly journals Low levels of activated protein C in patients with systemic lupus erythematosus do not relate to lupus anticoagulants but to low levels of factor II

2002 ◽  
Vol 117 (3) ◽  
pp. 676-684 ◽  
Author(s):  
Marleen J. A. Simmelink ◽  
José A. Fernández ◽  
Ronald H. W. M. Derksen ◽  
John H. Griffin ◽  
Philip G. De Groot
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Protein C ◽  
Activated Protein C ◽  
Systemic Lupus ◽  
Lupus Anticoagulants ◽  
Factor Ii ◽  
Low Levels

Clinical significance of acquired activated protein C resistance in patients with systemic lupus erythematosus

Lupus ◽  
2002 ◽  
Vol 11 (11) ◽  
pp. 730-735 ◽  
Author(s):  
F-J Muñoz-Rodríguez ◽  
J-C Reverter ◽  
J Font ◽  
D Tàssies ◽  
G Espinosa ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Clinical Significance ◽  
Lupus Erythematosus ◽  
Protein C ◽  
Activated Protein C ◽  
Activated Protein C Resistance ◽  
Systemic Lupus

Resistance to activated protein C in systemic lupus erythematosus patients with antiphospholipid antibodies

2009 ◽  
Vol 53 (3) ◽  
pp. 188-189 ◽  
Author(s):  
Juzo Matsuda ◽  
Kengo Gohchi ◽  
Miyo Tsukamoto ◽  
Moritaka Gotoh ◽  
Noriko Saitoh ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Antiphospholipid Antibodies ◽  
Protein C ◽  
Activated Protein C ◽  
Systemic Lupus

scholarly journals Increased Thrombophilic Tendency in Pediatric Cystic Fibrosis Patients

2009 ◽  
Vol 16 (1) ◽  
pp. 71-76 ◽  
Author(s):  
Vaughan Williams ◽  
Adrian B. M. Griffiths ◽  
Zen L. Yap ◽  
James Martin ◽  
Gregory Smith ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
General Population ◽  
Protein C ◽  
Activated Protein C ◽  
Protein S ◽  
Activated Protein C Resistance ◽  
Antithrombin Deficiency ◽  
Indwelling Catheters ◽  
C Protein ◽  
Lupus Anticoagulants

Thrombophilia has recently been reported to be increased in patients with cystic fibrosis (CF). We wanted to determine whether this was applicable to our population with CF and how our patients compared to the previously reported groups. Seventy one pediatric CF patients were assessed for a thrombophilic tendency, using a lupus anticoagulant screen, protein C, protein S, antithrombin assay, and activated protein C resistance (APCR) screen. The incidence of activate protein C resistance (4.2%) was within expected limits for the general population as was the incidence of antithrombin deficiency. However there was a marked increase in the incidence of lupus anticoagulants (18%) and 14% and 19.7% of the patients showed a reduced protein C and protein S, respectively, far in excess of the general population. This increased incidence of thrombophilia was not related to any specific CF phenotype and while perturbed liver function cannot be entirely ruled out, it appeared unlikely to be responsible for all the abnormal coagulation findings. Despite the apparent thrombophilic tendency, no clinically evident thrombotic episodes were noted during the study period. Thrombophilia is of concern because of the increasingly frequent placement of indwelling catheters in CF patients. The precise cause for the thrombophilic tendency in CF patients is unknown at this stage.

scholarly journals Comparison of Acquired Activated Protein C Resistance, Using the CAT and ST-Genesia® Analysers and Three Thrombin Generation Methods, in APS and SLE Patients

2021 ◽  
Vol 11 (1) ◽  
pp. 69
Author(s):  
Maria Efthymiou ◽  
Philip J. Lane ◽  
David Isenberg ◽  
Hannah Cohen ◽  
Ian J. Mackie
Keyword(s):  
Lupus Erythematosus ◽  
Protein C ◽  
Thrombin Generation ◽  
Activated Protein C ◽  
Kappa Coefficient ◽  
Antiphospholipid Antibody ◽  
Data Handling ◽  
Activated Protein C Resistance ◽  
Systemic Lupus ◽  
Pregnancy Morbidity

Background: Acquired activated protein C resistance (APCr) has been identified in antiphospholipid syndrome (APS) and systemic lupus erythematosus (SLE). Objective: To assess agreement between the ST-Genesia® and CAT analysers in identifying APCr prevalence in APS/SLE patients, using three thrombin generation (TG) methods. Methods: APCr was assessed with the ST-Genesia using STG-ThromboScreen and with the CAT using recombinant human activated protein C and Protac® in 105 APS, 53 SLE patients and 36 thrombotic controls. Agreement was expressed in % and by Cohen's kappa coefficient. Results: APCr values were consistently lower with the ST-Genesia® compared to the CAT, using either method, in both APS and SLE patients. Agreement between the two analysers in identifying APS and SLE patients with APCr was poor (≤65.9%, ≤0.20) or fair (≤68.5%, ≥0.29), regardless of TG method, respectively; no agreement was observed in thrombotic controls. APCr with both the ST Genesia and the CAT using Protac®, but not the CAT using rhAPC, was significantly greater in triple antiphospholipid antibody (aPL) APS patients compared to double/single aPL patients (p < 0.04) and in thrombotic SLE patients compared to non-thrombotic SLE patients (p < 0.05). Notably, the ST-Genesia®, unlike the CAT, with either method, identified significantly greater APCr in pregnancy morbidity (median, confidence intervals; 36.9%, 21.9–49.0%) compared to thrombotic (45.7%, 39.6–55.5%) APS patients (p = 0.03). Conclusion: Despite the broadly similar methodology used by CAT and ST-Genesia®, agreement in APCr was poor/fair, with results not being interchangeable. This may reflect differences in the TG method, use of different reagents, and analyser data handling.

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