systemic lupus

erythematosus

(sle)

*A rash across the bridge of the

nose and the upper cheek bone.

*Systemic- Can affect any

organ or tissue in body.

*Lupus- Wolf

*Erythymatosus- Colour of the

rash.

*SLE- systemic autoimmune disease or

auto immune connective tissue disease.

*Can affect any part of the body.

*Characterised by acute or chronic

inflammation in various tissues of the

body.

*Type III hypersensitivity reactions in

which Ab immune complexes

precipitate and cause further immune

response.

*Skin manifestation- Butterfly eruption

on face and photosensitivity.

*SLE most commonly harms heart,

kidney, joints, lungs, blood vessels,

liver and nervous system

EPIDEMIOLOGY

*Women are more affected, especially women in

child bearing age are more affected. Because

XX chromosomes in females, X carries

immunological related genes which can mutate.

*Common in Canada and Europe.

*Children about 3 years are affected, majority in the

age of 15-44 years.

ETIOLOGY

*Environmental factors- cold, fatigue, stress,

sunlight. Sunlight contain UV rays- UVA and

UVB(Shorter wavelength causes more harm).

*Genetic factors – not caused by a single gene, but

multiple gene appear to influence chance of

develop lupus. HLA region on chromosome 6

where mutation occur randomly.

*Virus- Epstein bar virus.

*Chemicals- silica, mercury.

*Drug reactions- occur in people treated for

long term infections.

Eg. Procainamide

Hydrolazine

phenytoin.

PATHOPHYSIOLOGY

It is mainly related to

The deposition of immune complexes in

various organs which trigger complement and

other mediator of inflammation.

Auto-antibodies in SLE are directed against

wide variety of self antigens, most commonly

against Nuclear self antigen(ANA).

Deactivate the bacteria, virus and allergen.

APC’s activate the immune system

NORMAL MECHANISM

Bacteria, virus, allergens enter the body

Aggrevatedby env.

stimulantants

UV light or drug rxn

Destruct other cells in the body and

exposure of DNA histones

& other proteins mainly

a part of cell nucleus

Body’s sensitised B-cells produce Ab

against nuclear reacted proteins

Abnormalities in apoptosis

Ab-protein complex

Which will stick to surface of blood

vessel

Deficiency in clearance

Deposition of dead cells and other cellular waste

SLE

TYPES

Discoid lupus- A form of lupus that can be

isolated only to skin without affecting

internal organs is called discoid lupus

When internal organs are involved it is

called SLE.

COMPLICATIONS

*Anaemia- Haemolytic and iron deficiency.

*Antiphospholipid syndrome:

• disorder of blood coagulation related to

the presence of auto antibodies called

lupus anticoagulation and anti cardiolipin.

•APS causes blood clot in deep veins.

*Vasculitis- inflammation of blood vessel.

*Thrombocytopenia- Ab attack platelets and

cause their destruction- causes bleeding

from nose, gums and intestine.

*Blood cancer

*Heart complications- myocarditis, heart

attack, stroke, endocarditis, plaque build

up in arteries.

*Lung complications: chest pain, coughing,

pain in breathing, pulmonary hypertension.

*Kidney : early fluid build up which causes

overall weight gain, kidney failure.

*CNS complications: psychologic and

neurological disorder, anxiety, depression,

amnesia and migrane.

COMPLICATIONS OF HEART AND

LUNG

*Infection: fungal and parasitic infection, people

with weak immune system have increased risk

of UTI and yeast infection.

*GI complications: nausea, diarrhoea, weight

loss, heart burn(GERD).

*Joint, muscle and bone marrow complications:

osteoporosis, muscle ache, swelling of joints.

DIAGNOSIS

*SLE affects various organs, so they have wide

variety of symptoms and different combination of

organ involved.

There are 11 criteria mainly determined as

diagnosis…..

Butterfly rash.

Discoid skin rash/patchy redness with

hypo/hyper pigmentation that causes scarring.

*Photo sensitivity.

*Mucous membrane ulcers- nose mouth or

throat.

*Arthritis- 2 or more joints.

*Kidney abnormalities- abnormal amounts of

urine protein, casts are detectable.

*Anti- nuclear antibodies(ANA antibody)

*Antiphospholipid syndrome.

*Lungs – inflammation of lining around

the lungs.

*Brain irritation- by seizures,

convulsions- reffered as lupus

cerebritis.

*Low blood count abnormalities-

reduced WBC/RBC/Platelets.

TREATMENT

Management aims:

* To reduce inflammation.

*To reduce pain.

*To prevent organ damage.

*To improve the quality of life.

NON PHARMACOLOGICAL TREATMENT

*Sun protection

*Wear hats and clothing made of tightly

woven fabrics

PHARMACOLOGICAL

TREATMENTCATEGORY EXAMPLES

SALICYLATES IBUPROFEN, NAPROXEN,

ASPIRIN

ANTIMALARIALS HYDROXYCHLOROQUINE,

CHLOROQUINE, QUINACRINE

TOPICAL CORTICOSTEROIDS BETAMETHASONE,

DIPROPIONATE,

CYCLOPHOSPHAMIDE,

METHYL PREDNISOLONE

IMMUNOSUPPRESANTS CYCLOPHOSPHAMIDE,

AZATHIOPRINE,

MYCOPHENOLATE MOFETIL,

DHEA

INTRAVENOUS

IMMUNOGLOBULINS

BELIMUMAB

SALICYLATES

*Indication: To treat arthritis.

*Dose : 400-800 mg 3-4 times/day

initial dose- 2.4-3.6 g/day in divided doses

maintainence dose – 3.6-5.6 g/day.

*ADR: Intolerance,

salicylism (HA, dizziness, vertigo, difficulty in

hearing).

ANTIMALARIALS

*Indication: To treat flares,

To protect blood clot in people with anti-

phospholipid syndrome.

*Dose : initial dose-400 mg OD

Maintainence dose: 200-400 mg

*ADR: GI problems, HA, hair loss, yellow

colour skin(Quinacrine), retinal damage.

TOPICAL CORTICOSTEROIDS

*INDICATION: Reduce inflammatory process,

symptoms involving anaemia and kidney. IV

methyl prednisolone can reduce flares in 3

days.

*Mechanism : acts as alkylating agents, it acts

by adding an alkyl group to base of DNA which

reduces immune system response to various

diseaes(AID’s)

*Dose : mild potency for face

mid potency for other areas

Apply thin films to affected areas once or twice

daily.

Maximum dose 45 g / week

Prednisolone, methyl prednisolone given as IV

in a dose of < 1 mg/kg/day.

*ADR: Osteoporosis, weight loss, excess hair

growth, insomnia, irritability, infertility.

IMMUNOSUPPRESSANTS

*Indication: to treat kidney disease.

*Mechanism: acts by blocking an early stage in

activation of cytotoxic T-lymphocytes after the

recepient is exposed to Ag.

*Dose: initial dose- 2-3 g/day

Maintainence dose- 0.5-3 g/day

Azathioprim: 1 mg/kg/day for 6-8 weeks

increases by 0.5 mg/kg every 4 weeks.

*Methotrexate- 7.5-15 mg / week with folic

acid.

*Mycofenolate mofetil: It inhibits an enzyma

neede for the growth of B & T cells.

*ADR: nausea, vommiting, hair loss,

infertility, diarrhoea(mycofenolate mofetil)

INTRAVENOUS IMMUNGLOBULINS

*Indication: In pain & fever , to reduce Ab

production, promote clearance of immune

complexes.

*It is a biological monoclonal antibody which

inhibits a protein called B- lymphocyte stimulator

protein and reduces the production of B - cells

and thereby reduces the immune response.

LIFE STYLE MODIFICATIONS

• Use topical sunscreens.

• Avoid triggers

- Exposure to sunlight.

- Avoid UV rays

- Use hypo-allergic cosmetics and hair

products.

- Don’t smoke or avoid exposure to passive

smoking.

Prevent infections:

-Reduce exposure to crowds.

-Reduce exposure to people with contagious

illness.

-Proper hygiene.