Introduction
Systemic lupus erythematosus: diagnostic criteria
(2012 – SLICC)
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Cutaneous manifestation – 4 Items | Acute Cutaneous Lupus Erythematosus/Subacute Cutaneous Lupus Erythematosus |
Chronic Cutaneous Lupus Erythematosus | |
Oral ulcers | |
Non-scarring alopecia | |
Joints – 1 Item | Synovitis > 2 peripheral joints (pain, tenderness, swelling or morning stiffness > 30 min) |
Serositis – 1 item | Pleuritis, typical pleurisy ≥ 1 day, history, rub, evidence of pleural effusion, pericarditis, typical pericardial pain ≥ 1 day, EKG evidence of pericardial fusion) |
Renal disorder – 1 Item | Urine protein/creatinine ratio or urinary protein concentration of 0.5 g of protein/24 h, Red blood cell casts |
Haematological disorder – 3 Items | Haemolytic anaemia |
Leukopenia (<4000/mm3) or lymphopenia (<1000/mm3) separately at least once | |
Thrombocytopenia (<100,000/mm3) at least once | |
Immunologic abnormal – 6 Items | Positive ANA |
Positive anti-dsDNA (except ELISA) on ≥ 2 occasions | |
Anti-Sm | |
Antiphospholipid antibody (including lupus anticoagulant, false-positive RPR, anti-cardiolipin, anti-beta2glycoprotein1) | |
Low complement (C3, C4 or CH50) | |
Direct Coombs test in the absence of haemolytic anaemia | |
Diagnosis | Fulfil 4 items (at least one clinical and one immunologic item) |
Classification criteria for the APS (modified from [17])
Clinical criteria
Laboratory criteria
Search strategy
Physiopathology of atherosclerosis and arterial thrombosis in SLE
SLE-related factors
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Effects
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TNF-alpha, MCP-1, IL-6, kidney disease | Dyslipidemia worsening, dysfunctional piHDL production |
ICAM, VEGF, vWF, VCAM overexpression | Pro-aggregation/pro-thrombotic behaviour |
Acute phase reactants elevation | Tissue damage |
INF-alpha elevation | Endothelial damage, pro-thrombotic behaviour |
Neutrophil extra-cellular traps | Vascular damage/pro-thrombotic behaviour |
IL-17, IL-12, IL-18 elevation, IgG overexpression | Pro-inflammatory |
Cystatin C | Early kidney damage marker, pro-inflammatory |
SS-A/SS-B positivity profile, photosensibility | Higher risk of CVD |
Medications
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Effects
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Corticosteroids | Higher risk of CVD |
Antimalarial (OH-chloroquine) | Anti-thrombotic, anti-inflammatory, anti-dyslipidemic |
Mycophenolate mofetil | Lower immune activity in carotid plaque, protective |
HMG-CoA reductase inhibitors (atorvastatin) | Fewer autoantibodies and improvement of kidney function in animal models, to be confirmed in humans |
NSAIDs | Higher risk of CVD (less with naproxene) |
Clinical risk factors for thrombosis in SLE: prevention and treatment
The clinical risk factors for thrombosis
Primary prevention of venous thromboembolism (VTE)
VTE and arterial thrombosis treatment
Clinical scenario
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Recommendations
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Patients with definite APS (+/− SLE) and first venous event | aVK, INR range of 2.0-3.0 |
Patients with definite APS (+/− SLE) and venous recurrent thromboembolism during aVK or arterial thrombosis* | aVK, target INR > 3.0a
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or | |
combination of aspirin (100 mg daily) and aVK, INR range 2.0-3.0a
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APS patients (without SLE) with a first non cardioembolic cerebral arterial event, low-risk aPL profile, and reversible predisposing factors | Antiplatelet agents |
SLE patients with venous or arterial thrombosis who do not fulfil APS laboratory classification criteria | Same as patients from the general population who develop venous or arterial thrombosis |
Primary prevention of acute arterial diseases
Pregnancy, contraception and thrombosis
Catastrophic APS
Criteria for the classification of catastrophic APS (modified from [79])
Definite catastrophic APS: All 4 criteria
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Evidence of involvement of three or more organs, systems and/or tissues.a
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Development of manifestations simultaneously or in less than a week.
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Confirmation by histopathology of small vessel occlusion in at least one organ or tissue.b
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Laboratory confirmation of the presence of antiphospholipid antibodies (lupus anticoagulant and/or anti-cardiolipin antibodies).
Probable catastrophic APS:
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All 4 criteria except for the involvement of only two organs, systems and/or tissues
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All 4 criteria except for the absence of laboratory confirmation at least 6 weeks apart due to the early death of a patient never tested for aPLs before catastrophic APS
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1, 2 and 4
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1, 3 and 4 and the development of a third event in more than a week but less than a month, despite anticoagulation