Autoimmune Disorders During Pregnancy -Lupus -Antiphospholipid syndrome Rovnat Babazade, MD Obstetrical Anesthesia-2016

SYSTEMIC LUPUS ERYTHEMATOSUS An extremely complicated and multifactorial interaction among various genetic and environmental factors The loss of immune tolerance Increased antigenic load, excess T cell help Defective B cell suppression Shifting of T helper 1 (Th1) to Th2 immune responses Leads to B cell hyperactivity and the production of pathogenic autoantibodies Finally, certain environmental factors are probably required to trigger the disease

SYSTEMIC LUPUS ERYTHEMATOSUS 1997 ACR Classification Criteria Malar rash Discoid rash Photosensitivity Oral ulcers Arthritis Serositis Renal disorder Neurologic disorder Hematologic disorder Immunologic disorder Anti-nuclear antibodies

SYSTEMIC LUPUS ERYTHEMATOSUS 2012 SLICC Classification Criteria CLINICAL Acute cutaneous Chronic cutaneous Oral ulcers Non-scarring alopecia Synovitis Serositis Renal Neurologic Hemolytic anemia Leucopenia / Lymphopenia Thrombocytopenia IMMUNOLOGIC ANA Anti-dsDNA Anti-Sm Anti-Phospholipid Low complement Direct Coomb’s test Petri M et al. Derivation and vallidation of the SLICC Classification Criteria for SLE. Arth & Rheum 2012

Clark CA et al. Decrease in pregnancy loss rates in patients with SLE over a 40-year period. J Rheumatol 2005

Smyth A et al. A systematic review and metaanalysis of pregnancy outcomes in patients with SLE and lupus nephritis. Am Soc Nephrol 2010

Clark CA et al. Decrease in pregnancy loss rates in patients with SLE over a 40-year period. J Rheumatol 2005

Yang H et al. Pregnancy-related SLE: clinical features, outcome and risk factors of disease flares – a case control study. PLoS ONE 2014

Flares During Anesthesia/Surgery? SLE significantly increased the risks of surgical patients for overall major complications and mortality after major surgery. Lin J-A, et al. Ann Rheum Dis 2014

Flares During Anesthesia/Surgery?   Summary of complications among N=141,345 matched discharges SLE (N = 28,269) Control (N = 113,076) OR (99% CI) P-Value RENAL COMPLICATIONS 584.6 Acute renal failure with cortical (renal) 0 (0.00) 584.7 Acute renal papillary necrosis with medullary (renal) (papillary) 584.5 Acute renal papillary necrosis with tubular 218 (0.77) 623 (0.55) 584.8 Acute renal failure with specified pathology NEC 3 (0.01) 7 (0.01) 584.9 Acute kidney injury (nontraumatic) 768 (2.72) 2,292 (2.03) 997.5 Acute postoperative renal failure 93 (0.33) 393 (0.35) - Any of the above renal complications - 1,047 (3.70) 3,179 (2.81) 1.33 (1.21, 1.46) < 0.001 CARDIOVASCULAR COMPLICATIONS 997.1 Cardiac Arrest/insufficiency during or resulting from a procedure 177 (0.63) 830 (0.73) 411.81 Acute coronary occlusion without myocardial infarction 2 (0.007) 4 (0.004) 518.4 Pulmonary edema, postoperative 37 (0.13) 112 (0.10) 410 Myocardial infarction 111 (0.39) 403 (0.36) - Any of the above cardiovascular complications - 304 (1.08) 1,237 (1.09) 0.98 (0.83, 1.16) 0.79 IN-HOSPITAL MORTALITY 454 (1.61) 1,430 (1.26) 1.27 (1.11, 1.47) SLE was associated with higher risk of renal complications and in-hospital mortality but not cardiac events after major surgery Babazade et al. The Association between Systemic Lupus Erythematosus and Adverse Postoperative Outcomes. Anesthesia and Analgesia. 2016

Anti-Phospholipid Syndrome (APS) β2-glycoprotein I (β2GPI) recognized as a major target for autoantibodies in APS In situ association between β2GPI and HLA class II was observed in placental tissues of APS patients but not in healthy controls

Anti-Phospholipid Syndrome (APS) 2–4% in the general population Raising awareness of antiphospholipid antibody syndrome .The Lancet 2010

Anti-Phospholipid Syndrome (APS) 2006-modified Sapporo Lockshin MD. Anticoagulation in Management of Anti-Phospholipid Antibody Syndrome in Pregnancy. Clin Lab Med 2013

Anti-Phospholipid Syndrome (APS)

Anti-Phospholipid Syndrome (APS)

Anti-Phospholipid Syndrome (APS)

Anti-Phospholipid Syndrome (APS)