complication in SLE is diverse, how to deal with?

1. Lupus complication2
Lupus cerebritis, cutaneous SLE
Marwa Abo Elmaaty Besar
Lecturer Of Internal Medicine
(Rheumatology & immunology Unit)
(Pediatric Rheumatologist)

3. Neuropsychiatric lupus
Rule out mimic (infections, malignancy, others).
Pathological mechanism; Inflammation/ Thrombosis/ Embolism/ Ischemia.
Risk factors:
◦ Type and timing of manifestation
◦ Presence of generalised
◦ Non-neurological disease activity
Abnormal neuroimaging and cerebrospinal fluid analysis
◦ Positive antiphospholipid antibodies [apl].
Clinical assessment + neuroimaging + CSF studies.
Distinction between the two pathophysiological processes may not be easy in clinical practice, or the
two processes may coexist in the same patient.

5. Management:
◦ Correction of aggravating factors.
◦ Symptomatic therapy when appropriate.
◦ Specific therapy: Depend on whether inflammation or thrombosis.
Inflammation:
Mild to moderate: Glucocorticoids in combination immunosuppression (AZA,
Cyclophosphamide).
Sever/ refractory cases: plasma exchanges, IVIG, RTX.
Thrombosis:
Antiplatelet/ Anticoagulant in NPSLE related to APLs.

9. Cutaneous Lupus:

10. ACLE

11. SCLE

12. DLE

13. LEP
LET

16. Best Pract Res Clin Rheumatol. Author manuscript; available in PMC 2014 June 01

17. © 2016 European Academy of Dermatology and Venereology

18. © 2016 European Academy of Dermatology and Venereology

19. © 2016 European Academy of Dermatology and Venereology

21. Antiphospholipid syndrome
Presence of aPL is associated with thrombotic and obstetric complications and
increased risk of damage accrual.
Primary prophylaxis: Low dose aspirin (aPL carrier who had SLE), risky.
Patients with SLE with aPL may also receive additional anticoagulant treatment,
such as low-molecular weight heparin, during high-risk periods for thrombosis
(pregnancy or postoperatively).
Anticoagulant: ???to patients with lupus with any aPL antibodies or
only to those carrying a high risk aPL profile (ie, triple aPL positivity, lupus
anticoagulant or high titres of anticardiolipin antibodies)

22. APLs
New oral anticoagulant:
◦ use of novel oral anticoagulants for secondary prevention should be avoided.
◦ Can be used for :
• low-risk aPL profile.
• no history of arterial thrombotic events
• with difficult to control international normalised ratio on warfarin, after balancing possible risks.

23. Moderate to high :
• Asymptomatic
• No treatment
• Aspirin ( 81 mg / day )
Equivocal thrombosis
APLA titer

24. For absent or low titer :
• Recurrent pregnancy loss
• Evaluate for :
• Other coagulopathies
• Other causes of
pregnancy loss
Consider :
Heparin … 5000 IU bid
OR
LMWH throughout pregnancy
Discontinue 6 to 12 weeks postpartum
APLA titer

25. Start with the standard manner with
Heparin
Followed by long term maintenance
with Warfarin
• Usually at INR of 2.5
( International Normalized Ratio )
• Do not exceed 2-3 times than normal
• Warfarin needs 2-3 days
• So , do not withdraw Heparin rapidly
but within 3 days
Anticoagulationfor thrombosis

26. Pitfall
In some patients
LA cause the INR to be unreliable
OR , with unfractionated or LMWH
Monitored by measurement of Anti-factor
Xa activity or other appropriate assay
• Such patients may be treated
with warfarin, monitored by
special assays

27. Forwell–anticoagulatedpatients,whocontinuetohavethromboses
Aspirin
HQ
A statin drug
IvIG&
Plasmapheresis
Have theoretical bases for efficacy & all been used

28. Catastrophic Vascular Occlusion
It is usually :
Sudden
Confusing &
Immediately life threatening
• The most effective treatment :
• Combines full dose anticoagulation
• High dose CCs
• Plasmapheresis &
• IV IG
• Cyclophosphamide ( some authors even without SLE )
• Pulse therapy

29. Hematological:
Thrombocytopenia:
Thrombocytopenia: <100,000/mm3 at least once (in the absence of other known causes such as
drugs, portal hypertension, TTP)
Significant thrombocytopenia <30.000/mm3.
Aim: platelet count >50.000/ mm3.
o Initial therapy; IV pulse steroid for 1-3 days or IVIG
o IVIG in cases of inadequate response to pulse, acute phase, to avoid steroid side effect.
o Moderate/high glucocorticoids + immunosuppression (AZA, MMF, Cyclosporine).
o Failure or relapse : RTX or Cyclophosphamide.
o splenectomy is last options.
o Thrombopoietin agonist????

30. Autoimmune hemolytic anemia
Less common than thrombocytopenia.
Moderate/high glucocorticoids + immunosuppression (AZA, MMF, Cyclosporine).
Resistant: RTX, cyclophosphamide.
Leukopenia
<4000/ mm3 at least once in absence other known causes as (Felly's, drugs, portal hypertension).
Lymphopenia: < 1000/mm3 at least once (in the absence of other known causes such as corticosteroids, drugs
and infection)
Exclude drug induced.
Autoimmune leukopenia is common in SLE rare need treatment.

31. Infection:
Risk factors:
◦ Disease related; sever leukopenia, high disease activity, renal affected.
◦ Treatment related ( CYC, RTX, MMF, High dose steroid).
Primary prevention:
o Early recognition antimanagement.
o Vaccination: against seasonal influenza, pneumococcal infection (PCV12, PPSV23), stable disease.
o Vaccination against herpes zoster not yet study in SLE.
o Score for sepsis: SOFA score
o (SBP <100mmhg, Respiratory rate >22/min, altered mental status with GCS <15)
o 2 point = indicate patient at onset of infection, associated with greatest risk of death, need ICU.
Seymour CW, Liu VX, Iwashyna TJ, et al. Assessment of clinical criteria for sepsis: for the third International consensus
definitions for sepsis and septic shock (Sepsis-3). JAMA 2016;315:762–74.

32. CVS:
Risk factor:
◦ Active disease.
◦ Increase disease duration.
◦ Medium /high titres of apl.
◦ Renal involvement (persistent proteinuria, gfr < 60ml/min)
◦ Chronic use of glucocorticoids.
Pericarditis: dry or effusion.
Myocarditis; asymptomatic in most case, indolent course or unexplained tachycardia till global
hypokinesia on ECHO.
Endocarditis; Libman-sacks lesion; verrucous , thickening of mitral and aortic valve
Valvular damage: more with APL syndrome.

35. Marker for assessment:
◦ Surrogate measures of atherosclerosis such as carotid plaques, carotid intima
media thickness (cITMT).
◦ Coronary artery calcium; subclinical CVD.
Primary prevention;
low dose aspirin.
Statin; failed to show clear benefit.
Treatment:
Corticosteroid + Immunosuppression according to SLE activity.