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Anti-phospholipid syndrome.pptx
- 1. Anti-phospholipid syndrome (APS) by Dr. Arwa Mohammed Othman Associate professor of Immunology and Microbiology Sana’a University
- 2. APS (Hughes syndrome) Outlines Definition of APS Epidemiology Pathogenesis Diagnosis Management Graham Hughes (1984)
- 3. Antiphospholipid syndrome (APS) Definition APS is systemic autoimmune disease
- 4. Antiphospholipid syndrome (APS) APS is characterized by:
- 5. Antiphospholipid syndrome (APS) Antiphospholipid Abs (aPL)
- 6. Antiphospholipid syndrome (APS) Antiphospholipid antibodies (aPL) are a heterogeneous family of autoAbs 1- Lupus anticoagulant (LA)* 2- Anti𝛽2glycoprotein-1 antibodies (anti𝛽 2GP1 Abs)* 3- Anticardiolipin antibodies (aCL)
- 7. APS could be: Antiphospholipid syndrome (APS)
- 8. Antiphospholipid syndrome (APS) Epidemiology Incidence: 2.1/100 000 per year Prevalence: 50/100,000 population. Andreoli et al, 2013; Chighizola et al, 2015
- 10. Antiphospholipid syndrome (APS) ~ 20% of patients with a cerebrovascular clots < 60 years old Berkman et al, 2021
- 11. Antiphospholipid syndrome (APS) Epidemiology 6-50% of females with recurrent miscarriages Rodríguez et al, 2021
- 13. Antiphospholipid syndrome (APS) The prevalence of aPL is estimated to be 1- 5% of the general population. Only a minority of these individuals develop APS. Berkman, Song, 2021
- 14. Linnemann, 2018; Knight and Kanthi, 2022 Paraoxonase Antiphospholipid syndrome (APS)
- 16. Antiphospholipid syndrome (APS) Lab diagnosis
- 17. Diagnosis of APS Clinical criteria (at least one) Laboratory criteria (at least one) Miyakis et al, 2006
- 18. Clinical criteria of APS 1- Confirmed thrombosis Arterial thrombosis: more frequent in males Deep veins thrombosis: more frequent in females Giannakopoulos B and Krilis, 2013
- 19. Clinical criteria of APS 2- Pregnancy morbidity 1≤ unexplained deaths of a morphologically normal fetus (after 10th WOG) 1≤ premature delivery of a morphologically normal neonate (before 34th WOG) 3 ≤ unexplained consecutive spontaneous abortions (before 10th WOG) Giannakopoulos B and Krilis, 2013
- 20. Clinical criteria of APS 3- Others Livedo reticularis Gangrene
- 21. Laboratory criteria Persistent +ve aPL (12 weeks apart) Clot-based assays Serology (ELISA) Plasma; poor platelets Serum, Plasma LA Anti𝛽2GP1, aCL Abs Triple positive has strong association with clots
- 22. Laboratory diagnosis 1- Lupus anticoagulant (LA) Patient does Not to have lupus It is Ab Not anti-coagulant
- 23. Laboratory diagnosis 1- Lupus anticoagulant testing Activated partial thromboplastin time (aPTT) Dilute Russell viper venom test (dRVVT) Screen Mix Confirm
- 24. Antiphospholipid syndrome (APS) 1- Lupus anticoagulant testing Stop warfarin for 2 ws; heparin for 2 days
- 25. Antiphospholipid syndrome (APS) 1- Lupus anticoagulant testing If aPTT test is normal? Patient has normal PT but prolonged aPTT
- 26. Antiphospholipid syndrome (APS) Other laboratory findings Hb: may be low Occasionally: thrombocytopenia (< 100000)
- 27. Antiphospholipid syndrome (APS) Catastrophic APS Rare complication of APS Widespread clotting in small blood vessels
- 28. Antiphospholipid syndrome (APS) Management Only Aspirin, LMWH or Warfarin New anticoagulant drugs are not used
- 29. Antiphospholipid syndrome (APS) Summary APS is an autoimmune disease Patients have: thrombosis, pregnancy morbidity, persistent aPL Diagnosis: depends on clinical criteria and lab criteria
- 30. Antiphospholipid syndrome (APS) References Andreoli L, Chighizola CB, Banzato A, Pons-Estel GJ, Ramire de Jesus G, Erkan D. Estimated frequency of antiphospholipid antibodies in patients with pregnancy morbidity, stroke, myocardial infarction, and deep vein thrombosis: a critical review of the literature. Arthritis Care Res (Hoboken). 2013;65(11):1869-1873. Berkman SA, Song SS. Ischemic Stroke in the Young. Clin Appl Thromb Hemost. 2021;27:10760296211002274. Knight JS, Kanthi Y. Mechanisms of immunothrombosis and vasculopathy in antiphospholipid syndrome. Semin Immunopathol. 2022;1-16. Linnemann B. Antiphospholipid syndrome - an update. Vasa. 2018;47(6):451-464.
- 31. Antiphospholipid syndrome (APS) References Miyakis S, Lockshin MD, Atsumi T, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost. 2006;4(2):295-306. Petri M. Antiphospholipid syndrome. Transl Res. 2020;225:70-81. Rodríguez CM, Velásquez-Berrío M, Rúa C, et al. Antiphospholipid Antibodies From Women With Pregnancy Morbidity and Vascular Thrombosis Induce Endothelial Mitochondrial Dysfunction, mTOR Activation, and Autophagy. Front Physiol. 2021;12:706743. Published 2021 Nov 29.