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UPDATES IN SLE AND APS FINAL.pdf
1. What’s new in SLE &
APS?
• Dr. Khuloud Saleh
• MD,FRCPC
• CONSULTANT RHEUMATOLOGY
2. Agenda
2019 EULAR/ACR new Lupus Classification
Criteria
EULAR 2019 non-renal Lupus management
Updates in Lupus nephritis management
(KDIGO 2021)
2019 EULAR /ACR Management for thrombosis
in APS
2022 ACR New classification criteria for APS
3. Systemic lupus
erythematosus (SLE)
• SLE is a systemic autoimmune disease with
multisystem involvement
• It is associated with significant morbidity and
mortality
• There are recent evolutions on diagnosis and
management of lupus in last couple years
15. Lupus nephritis
• Lupus nephritis (LN) is associated with increased morbidity and
mortality
• Renal biopsy is essential and the gold standard to diagnose LN
• Extra-glomerular involvement is seen in up to 60% of patients with
LN and is associated with poor outcomes
.
2020 Jun 4;22(7):30. doi: 10.1007/s11926-020-00906-7.
16. Lupus Nephritis subtypes
(LN)
• Class I: 1% Minimal change
• Class II: 10% Mesangial proliferative
• Class III: 20% Focal proliferative
• Class IV: 37% Diffuse proliferative
• Class V: 29% Membranous
• Class VI: 3% Advanced sclerosis
18. • Belimumab Approved in late December 2020, the U.S. Food & drug
administration (FDA) approved belimumab (Benlysta) to treat adults with active
lupus nephritis who are receiving standard therapy.
• An unmet need for lupus nephritis patients and is the result of an FDA
breakthrough therapy designation and priority review of findings from the
BLISS-LN study.
*N Engl J Med. 2020 Sep 17;383(12):1117–1128.
19. • Voclosporin, a novel calcineurin inhibitor, was approved in
combination with immunosuppressive therapy to treat adults with
active lupus nephritis by the FDA in January/2021.
21. Kidney biopsy
• The diagnosis of lupus nephritis ideally confirmed by a kidney biopsy
• Indications for kidney biopsy in SLE patients:
1) Urine protein > 500mg/day.
2) Rising serum creatinine/ decrease GFR that is not clearly attributable to
another mechanism
3) Hematuria(any level proteinuria) / active sediment
22. EULAR research agenda
• It is recommended that patients with SLE, including
those with lupus nephritis, be treated with
hydroxychloroquine or an equivalent anti-malarial
unless contraindicated,
• Lowers flares, including kidney
• Higher response rate to therapy
• Lower incidence of CV and thrombotic events in patients
with APS
• Less organ damage, improved lipid profile and better
preservation of bone mass
October 6, 2021
23. Lupus Science & Medicine 2019;6:e000310.doi:10.1136/lupus-2018-000310
Prevent disease flares
Protects from infections
Prevents autoimmune neonatal
heart
block
Correction of lipid
abnormalities
Prevention of metabolic
syndrome
Halting of
damage accrual
29. Class III and IV LN: maintenance therapy
• Glucocorticoids should be tapered to the lowest possible dose during
maintenance, except when glucocorticoids are required for extra-renal lupus
manifestations.
• Discontinuation can be considered after patients have maintained a complete
clinical response for ≥ 12 months.
• The total duration of immunosuppression plus combination maintenance
immunosuppression for proliferative LN should not be < 36 months.
31. Class VI / ESRD
Hemodialysis, peritoneal
dialysis or kidney
transplantation (kidney
transplantation is preferred
to long term dialysis)
Transplantation can be
carried out as soon as
disease in remission
35. Agenda
• New ACR 2022 APS Classification criteria
• Is there a role for primary prophylaxis?
• Is there a role for DOAC in APS?
• CAPS treatment
• Management of APS in pregnancy
36. Definite APS: 1 Clinical + 1 Lab criteria
Clinical criteria
Vascular thrombosis-arterial or
venous
Pregnancy morbidity:
• 1 or more death of normal fetus at ≥
10WK
• 1 or more premature birth at ≤ 34
weeks due to severe preeclampsia or
placental insufficiency
• ≥ 3 consecutive abortions at < 10
weeks with other causes being rules
out
Laboratory criteria
• Anti-cardiolipin IgG and IgM
• Lupus anticoagulant (LAC)
• Antiß2-glycoprotein IgG and IgM
Medium – high titer (40 GPL or MPL or
higher than 99th percentile) at least 12
weeks from the clinical manifestation
2019 Revised SAPPORO classification criteria for APS
37. • Hematological features:
• Thrombocytopenia
• Hemolytic anemia
• Renal
• Acute thrombotic
microangiopathy
• Chronic vaso-occlusive lesions
• Cardiac
• Valvular heart disease
• Neurological features
• Dermatologic
• Livedo reticularis
• Livedoid vasculopathy
• Neurological features
• Cognitive dysfunction
• White matter lesions
Major APS features not included in the criteria
Garcia D, Erkan D. Diagnosis and Management of the Antiphospholipid Syndrome. N Engl J Med. 2018 May 24;378(21):2010-2021.
Chaturvedi S, McCrae KR. Diagnosis and management of the antiphospholipid syndrome. Blood Rev. 2017 Nov;31(6):406-417.
38. • Sneddon's syndrome
• Pulmonary hypertension
• Splenic artery hemorrhage
• Addison's syndrome(adrenal
hemorrhage)
• Chorea
• Transverse myelopathy
• ARDS
• Renal thrombotic
microangiopathy,
• Budd-chiari syndrome
• Nodular regenerative
hyperplasia of the liver
• Avascular necrosis of the bone
• Cutaneous necrosis or
subungual splinter
hemorrhages
• Intracardiac thrombus
Rare APS manifestations
39.
40. • aPL without clinical criteria for APS
• No strong evidence to support the use of Low Dose Aspirin
(LDA) EXCEPT:
• Cardiovascular risk factors
• SLE
• High risk aPL profile
Is there a role for primary prophylaxis in aPL?
41. EULAR Definitions of medium-high antiphospholipid
antibody (aPL) titres, and of high-risk and low-risk APL
profile
42. • ASA (80-100mg/day)
• ASA allergy : LMWH
• General measures: Avoid smoking and sedentary life, control HTN and
dyslipidemia
• Other drugs: hydroxychloroquine, statins and folic acid
Primary prophylaxis
(high risk)
44. Patients who developed a thrombotic event while INR is therapeutic
(failure) :
1. Switch to LMWH
2. Oral anti coagulation + ASA or hydroxychloroquine
3. Increase the target INR 3.1-4
4. DOAC??
Treatment of Recurrent thrombosis
in spite of a therapeutic INR in patients with APS
45. Can the Direct Oral
Anti-coagulant
(DOIC) be used in
APS?
Can Direct Oral Anti-coagulant
(DOIC) be used in APS?
46. NO…
§ Trial of rivaroxaban in antiphospholipid syndrome (TRAPS)
§ Apixaban for the secondary prevention of thromboembolism among
patients with the antiphospholipid syndrome (ASTRO-APS)
§ Rivaroxaban for antiphospholipid antibody syndrome (RAPS)
No, especially in high risk patients ( triple positive aPL, arterial thrombosis,
recurrent events)
Trials of DOIC in APS?
47.
48. Catastrophic Antiphospholipid Syndrome (CAPS)
A rare variant of APS (1%)
Systemic inflammatory response
syndrome (SIRS)
Characterized by thrombosis in
multiple organs and a Cytokine storm
50. •Multiple simultaneous or evolving thrombotic events involving
microvasculature in < 1 week
•Malignant HTN (renal)
•ARDS (rapidly progressive pulmonary micro thrombosis, DAH)
•DIC
•Microangiopathic hemolytic anemia
•Thrombocytopenia
• Can be presenting manifestation of APS
Catastrophic Antiphospholipid Syndrome
signs and symptoms
51. CAPS criteria
• Definite (all 4)
• probable (all 4 with 2 organs involved, all but not 4 ( without 6 week
confirmation), or 1/2/4, 1/3/4 within 1 month despite anticoagulation)
52. • Early diagnosis and management are recommended.
• Based on the recently published clinical practice guidelines for CAPS
management:
• Combination therapy with :
• Glucocorticoids
• Heparin
• Plasma exchange or IVIG is recommended
• Concurrent treatment of precipitating factors is also recommended
• For refractory CAPS, B cell depletion (eg, Rituximab) or C5 inhibition (eg,
Eculizumab) therapies may be considered based on data from case reports.
Catastrophic antiphospholipid syndrome (CAPS)
53. • ASA 81 to 100mg daily before conception
• Once conception, add either heparin or LMWH until 34 weeks
• Resume LMWH at prophylactic dose one week post-partum for
6-12 weeks
Treatment of Pregnancy with APS