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

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 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

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

Immunosuppressantscyclphosphamide, Azathioprine

21systemic lupus erythematosus by Maureen Charagu

systemic lupus erythematosus by Maureen Charagu 22

Toni Braxton

Nick CanonMichael 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