SLE is an autoimmune disorder the above mentioned slide is a real case presentation

1. Systemic Lupus Erythematosis
Dr Mehak Ali
Peads

2. CONTENTS :
● Introduction
●Etiology
● Pathogenesis
● Clinical manifestations
● Lab investigations
● Diagnostic criteria
● Treatment
● Reference

3. ● Sle is a chronic autoimmune disease characterised
by multi system inflammation and the presence of
circulating autoantibodies directed against self
antigens
● Nearly every organ maybe affected

4. ETIOLOGY
● Genetic
● Environmental
● Hormonal

5. GENETIC
● Congenital deficiencies of C1q , C2 , and C4
● Polymorphism (e,g interferon regulatory factor)
● Familial clustering
● Human leukocyte antigen (HLA) types HLA B8 , HLA DR2 ,
HLA DR3

6. ENVIRONMENTAL
● UV light exposure
● Viral infections EBV
● Environmental factors influence epi genetic
modification to DNA

7. HORMONAL
● Estrogen (promotes B cell auto reactivity
● Hence affects females more especially during their
reproductive years

8. ● In children 1-
6/100,000
● Childhood SLE is rare
before 5yrs
● Median age 11-16yrs
EPIDEMIOLOGY

9. PATHOGENESIS :

10. UV light exposure / Inc level of Apopotosis ➡️ cell death
hence release of cell contents nucleic antigens ➡️ Sle
cells have impaired ability to clear cell debris ➡️
prolonged exposure to nucleic antigens in bloodstream
➡️ B cell stimulation ➡️ autoantibody production ➡️
circulating autoantibodies form immune complexes and
deposit in tissues ➡️ local complement and
proinflammatory cascade activation ➡️ ultimately tissue
damage

11. Antibodies to double stranded (ds) DNA can form
immune complexes deposit in glomeruli and initiate
inflammation leading to glomerulonephritis (not every
patient having circulating ds DNA antibodies have
nephritis )

12. Type 1 interferon signature
High levels of interferon (IFN-alpha) production by
plasmacytoid dendritic cells (DC’s) promote
expression of proinflammatory cytokines and
chemokines , maturation into myeloid DC’s hence
promotion of B and T cells

13. Other chemokines include
IL-1 , IL-2 , IL-6 , IL-10 , IL-12 , IL-17 , IL-21 , anti tumor
necrosis factor alpha , B lymphocyte stimulator (BLys)
also known as B cell activating factor (BAFF)

15. CLINICAL
MANIFESTATION :

23. What clinical
manifestation were
positive in our recent
case???

24. LABORTAROY
INVESTIGATIONS
● ANA (sensitive but not
specific positive in 95%
cases
● Antibodies to ds DNA
specific for SLE
● Serum levels of CH50 ,
C3 , C4 shows flares
decreased in diseases
improve with treatment
● Antiphopholipid
antibodies (inc in
venous & arterial
thrombotic event)

26. DIAGNOSIS
● ACR (American
college of
rheumatology
● SLICC (Systemic
lupus international
collaborating clinics

29. ACUTE CUTANEOUS LUPUS :
● Malar rash
● Bulluos lupus
● TEN
● Photosensitive lupus
rash
● Maculopapular lupus
rash
CHRONIC CUTANEOUS
LUPUs :
● Discoid rash
● Lupus panniculitis
● Lupus erthematous
tumidus
● Lichen planus overlap

30. TREATMENT :

31. General :
● Avoid exposure to UV light (avoid prolonged direct sun
exposure ) , use of sunscreen (reinforced at every visit)
● Calcium and vit d supplements to avoid osteoporosis is
such patients
● HYDROXYXHOLOROQUINE recommended when tolerated
by patients helps in mild sle (rashes and arthritis also
prevent sle flares (side effect retinal deposition)
maintanence dose 4-5mg/kg

32. Corticosteroids :
● Mainstay of treatment regarding many
manifestations of sle
● Oral prednisone given 1-2mg/kg/day
● In severe cases IV methylprednisolome can be given
from 30-1000mg/kg/ day for 3 days and sometimes
followed by weekly pulses

33. Steroid sparing immunosuppressive agents :
● Used to treat persistent moderate disease (arthritis
cutaneous hematalogical and pleural disease
● Examples : METHOTREXATE, LEFLUNOMIDE ,
AZATHIOPRINE , RITUXIMAB
● CYCLOPHOSPHAMIDE is used to treat severe cases
with maninfestations involving renal , neurological ,
cardiopulmonary disease

34. Childhood Arthrtis Rheumatology Research
Alliance CARRA :
● Proliferator lupus nephritis grade IV is associated
with Peadiatrics SLE
● 6 month induction therapy for cyclophosphamide and
MMF
● Belimumab (monoclonal antibody treatment of lupus
in adult

35. Atherosclerosis prevention in pediatric lupus
erythematosud (APPLE) :
● Statins should be in prevention of cardiovascular
manifestations

36. References
Nelson textbook of
Peadiatrics
Harriet lane

37. THANK YOUUU