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By Maureen Charagu
Facilitator Dr. Daniel Zuriel-Dermatopathologist
University of Nairobi
systemic lupus erythematosus by Maureen
Charagu 1
 History of X
 28 years female
 1 month history of
intermittent fever
 joint pains
 puffiness of her
face, hands and feet
 malar rash which
was more severe on
exposure to sunlight.
 intermittent
abdominal pain
 chest pain.
 bleeding of her
gums after
brushing her
teeth
 bruising of her
skin
systemic lupus erythematosus by Maureen Charagu 2
 enlarged non-tender lymph nodes and
spleen.
 A faint pericardial friction rub was present.
 Patient was on Prednisolone for treatment.
systemic lupus erythematosus by Maureen Charagu 3
 urine revealed moderate to severe protenuria &red and
white cells in the sediment
 serum hypoprotenemia, a reversed albumin/ globulin
ratio, hypocomplementemia, and antinuclear antibodies
(ANAs) to double stranded DNA
 CBC- low numbers platelet, neutrophil and normochromic
normocytic anaemia. LE cells were seen in a special
preparation
systemic lupus erythematosus by Maureen Charagu 4
systemic lupus erythematosus by Maureen Charagu 5
 Definition of SLE- autoimmune dx
 Causes and pathogenesis
 Morphology and Clinical Picture
 Investigations
 Treatment
6systemic lupus erythematosus by Maureen Charagu
 a multi-system auto-immune disease
that is caused by tissue damage
resulting from autoantibodies against
selfantigens and formation of
immunecomplexes
7systemic lupus erythematosus by Maureen Charagu
 Geography.
 Race.
 Sex. Affects 1 in
700 women of the
child bearing age.
 Age- high incidence 20-29 years
 Family history
 Blood group –
studies by Baart
De La Faille
systemic lupus erythematosus by Maureen Charagu 8
It’s a disease of the young and old
1. Infectious agents
2. Drugs
3. UV
4. Trauma
5. Toxins and diet
6. Immune regulation
dysfunctiontolerance $
ANAs
7. Hormonal
imbalance
8. Genetic
susceptibility- HLA-DQ
locus
9systemic lupus erythematosus by Maureen Charagu
systemic lupus erythematosus by Maureen Charagu 10
pathogenesis
Inherited gene
$
Environmental
triggers
Nuclear
proteins, self
antigens
Activation of T$
B cells to self
antigens
IgG
autoantibody
pxn
I. Complex $
Autoantibody
mediated
tissue injury
Skin:
 Malar Rash
 Discoid rash
 Oral ulcers
 Photo sensitive rash
Systemic Criteria
 Arthritis
 Serositis
 Kidney Disorder
 Neurologic Disorder
 Hematolic Disorder
Laboratory Criteria
 Immunologic Disorder
systemic lupus erythematosus by Maureen Charagu 11
systemic lupus erythematosus by Maureen Charagu 12
photosensitivity
Oral ulcer
Bulous eruption
LE cell
a neutrophil or macrophage that has
phagocytized (engulfed) the
denatured nuclear material of
another cell
 Kidney WHO
Classification
 Class
I. Normal on LM
II. Mesangial lupus
GN
III. Focal
proliferative GN
IV. Diffuse
Proliferative GN
V. Mebranous GN
systemic lupus erythematosus by Maureen Charagu 13
Mesangial lupus
Slight increase in mesangial
matrix and cellularity
systemic lupus erythematosus by Maureen Charagu 14
Focal proliferative GN Diffuse proliferative GN Membranous GN
Entire glomerulus has hypercellularity Increased deposition of
basement membrane
Localised proliferation of the
endothelium
Characterised by hematuria and protenuria- severity depends on class
 Endocardial– Libmanns and sacks verrucous
vegetations on both surfaces of any of the
valves, May be superimposed with bacterial
endocarditis
 Myocarditis
 Pericardium – fibrinoid deposition with LE
bodies
systemic lupus erythematosus by Maureen Charagu 15
16systemic lupus erythematosus by Maureen Charagu
systemic lupus erythematosus by Maureen Charagu 17
Superficial and deep perivascular
lymphocyte infiltrate
Injury to the blood vessels that
supply the retina.
Retinal hemorrhages= reduced blood
supply in:
•Macula= loss of central vision
•Optic nerve= blindness
 CNS- Vascular lesion= ischaemia/ multifocal
cerebral microinfarcts
 Lung- pleuritis & Pleural Effussion
 Spleen – moderately enlarged
 Lymph nodes –enlarged(edema &sinus hyperplasia) are
nontender
 Joints- synovitis
 GIT- Ulceration & arteritis (bleeding gums, Abd pain)
 Acute necritizing vasculitis
systemic lupus erythematosus by Maureen Charagu 18
 Screening- ANAs (antibodies to DNA,histones
etc)
 CBC (RBC, WBC, platelets)
 Urinalysis
 Sedimentation Rate (ESR)
 Rheumatoid Factor
 Skin biopsy
 Kidney Biopsy
systemic lupus erythematosus by Maureen Charagu 19
h C reactive protein (CRP)
h Sedimentation rate (ESR)
h Anti DNA
h Liver and Kidney
Function tests
h CPK
h Urine protein or cell
casts
i CBC (WBC, RBC,
platelets)
i Complement
i Serum albumin
20systemic lupus erythematosus by Maureen Charagu
 NSAIDs-ASA, ibuprofen, indomethacin
 Antimalarials-Chloroquine
 Corticosteroids-Hydrocortisone,
prednosolone, Prednisone, Methylprednisolone
 Immunosuppressants
cyclphosphamide, Azathioprine
21systemic lupus erythematosus by Maureen Charagu
systemic lupus erythematosus by Maureen Charagu 22
Toni Braxton
Nick Canon
Michael Jackson
Lady Gaga has borberline Lupus
People living with lupus… lets find a cure.
systemic lupus erythematosus by Maureen Charagu 23
systemic lupus erythematosus by Maureen Charagu 24

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Systemic lupus erythematosus.

  • 1. By Maureen Charagu Facilitator Dr. Daniel Zuriel-Dermatopathologist University of Nairobi systemic lupus erythematosus by Maureen Charagu 1
  • 2.  History of X  28 years female  1 month history of intermittent fever  joint pains  puffiness of her face, hands and feet  malar rash which was more severe on exposure to sunlight.  intermittent abdominal pain  chest pain.  bleeding of her gums after brushing her teeth  bruising of her skin systemic lupus erythematosus by Maureen Charagu 2
  • 3.  enlarged non-tender lymph nodes and spleen.  A faint pericardial friction rub was present.  Patient was on Prednisolone for treatment. systemic lupus erythematosus by Maureen Charagu 3
  • 4.  urine revealed moderate to severe protenuria &red and white cells in the sediment  serum hypoprotenemia, a reversed albumin/ globulin ratio, hypocomplementemia, and antinuclear antibodies (ANAs) to double stranded DNA  CBC- low numbers platelet, neutrophil and normochromic normocytic anaemia. LE cells were seen in a special preparation systemic lupus erythematosus by Maureen Charagu 4
  • 5. systemic lupus erythematosus by Maureen Charagu 5
  • 6.  Definition of SLE- autoimmune dx  Causes and pathogenesis  Morphology and Clinical Picture  Investigations  Treatment 6systemic lupus erythematosus by Maureen Charagu
  • 7.  a multi-system auto-immune disease that is caused by tissue damage resulting from autoantibodies against selfantigens and formation of immunecomplexes 7systemic lupus erythematosus by Maureen Charagu
  • 8.  Geography.  Race.  Sex. Affects 1 in 700 women of the child bearing age.  Age- high incidence 20-29 years  Family history  Blood group – studies by Baart De La Faille systemic lupus erythematosus by Maureen Charagu 8 It’s a disease of the young and old
  • 9. 1. Infectious agents 2. Drugs 3. UV 4. Trauma 5. Toxins and diet 6. Immune regulation dysfunctiontolerance $ ANAs 7. Hormonal imbalance 8. Genetic susceptibility- HLA-DQ locus 9systemic lupus erythematosus by Maureen Charagu
  • 10. systemic lupus erythematosus by Maureen Charagu 10 pathogenesis Inherited gene $ Environmental triggers Nuclear proteins, self antigens Activation of T$ B cells to self antigens IgG autoantibody pxn I. Complex $ Autoantibody mediated tissue injury
  • 11. Skin:  Malar Rash  Discoid rash  Oral ulcers  Photo sensitive rash Systemic Criteria  Arthritis  Serositis  Kidney Disorder  Neurologic Disorder  Hematolic Disorder Laboratory Criteria  Immunologic Disorder systemic lupus erythematosus by Maureen Charagu 11
  • 12. systemic lupus erythematosus by Maureen Charagu 12 photosensitivity Oral ulcer Bulous eruption LE cell a neutrophil or macrophage that has phagocytized (engulfed) the denatured nuclear material of another cell
  • 13.  Kidney WHO Classification  Class I. Normal on LM II. Mesangial lupus GN III. Focal proliferative GN IV. Diffuse Proliferative GN V. Mebranous GN systemic lupus erythematosus by Maureen Charagu 13 Mesangial lupus Slight increase in mesangial matrix and cellularity
  • 14. systemic lupus erythematosus by Maureen Charagu 14 Focal proliferative GN Diffuse proliferative GN Membranous GN Entire glomerulus has hypercellularity Increased deposition of basement membrane Localised proliferation of the endothelium Characterised by hematuria and protenuria- severity depends on class
  • 15.  Endocardial– Libmanns and sacks verrucous vegetations on both surfaces of any of the valves, May be superimposed with bacterial endocarditis  Myocarditis  Pericardium – fibrinoid deposition with LE bodies systemic lupus erythematosus by Maureen Charagu 15
  • 16. 16systemic lupus erythematosus by Maureen Charagu
  • 17. systemic lupus erythematosus by Maureen Charagu 17 Superficial and deep perivascular lymphocyte infiltrate Injury to the blood vessels that supply the retina. Retinal hemorrhages= reduced blood supply in: •Macula= loss of central vision •Optic nerve= blindness
  • 18.  CNS- Vascular lesion= ischaemia/ multifocal cerebral microinfarcts  Lung- pleuritis & Pleural Effussion  Spleen – moderately enlarged  Lymph nodes –enlarged(edema &sinus hyperplasia) are nontender  Joints- synovitis  GIT- Ulceration & arteritis (bleeding gums, Abd pain)  Acute necritizing vasculitis systemic lupus erythematosus by Maureen Charagu 18
  • 19.  Screening- ANAs (antibodies to DNA,histones etc)  CBC (RBC, WBC, platelets)  Urinalysis  Sedimentation Rate (ESR)  Rheumatoid Factor  Skin biopsy  Kidney Biopsy systemic lupus erythematosus by Maureen Charagu 19
  • 20. h C reactive protein (CRP) h Sedimentation rate (ESR) h Anti DNA h Liver and Kidney Function tests h CPK h Urine protein or cell casts i CBC (WBC, RBC, platelets) i Complement i Serum albumin 20systemic lupus erythematosus by Maureen Charagu
  • 21.  NSAIDs-ASA, ibuprofen, indomethacin  Antimalarials-Chloroquine  Corticosteroids-Hydrocortisone, prednosolone, Prednisone, Methylprednisolone  Immunosuppressants cyclphosphamide, Azathioprine 21systemic lupus erythematosus by Maureen Charagu
  • 22. systemic lupus erythematosus by Maureen Charagu 22 Toni Braxton Nick Canon Michael Jackson Lady Gaga has borberline Lupus People living with lupus… lets find a cure.
  • 23. systemic lupus erythematosus by Maureen Charagu 23
  • 24. systemic lupus erythematosus by Maureen Charagu 24