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By Maureen Charagu
Facilitator Dr. Daniel Zuriel-Dermatopathologist
University of Nairobisystemic 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
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 BaartDe 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 dysfunction tolerance $
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
Positive ANA Testsystemic lupus erythematosus by Maureen Charagu 11
systemic lupus erythematosus by Maureen Charagu 12
photosensitivityOral 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 membraneLocalised 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
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
Immunosuppressantscyclphosphamide, Azathioprine
21systemic lupus erythematosus by Maureen Charagu
systemic lupus erythematosus by Maureen Charagu 22
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