Download 1. Malar rash

Systemic Lupus Erythermatosus(SLE)
DR: DRGEHAN MOHAMED
DR. GEHAN MOHAMED
• Definition: chronic multisystem,
autoimmune disease most commonly
affecting young women.
• any organ of the body may be involved .
•
course of the disease is remissions and
exacerbations.
•
With good management, the ten years
survival may be over 90%.
Etiology and Pathogenesis of SLE
A) Genetic factor
•
•
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Certain genes permit a little immune
overresponse, or presentation of high
quantities of target antigens in certain tissues.
Many studies have described familial
aggregation of SLE.
lupus have at least one first or second degree
relative with lupus.
B). Environmental factors
1.
UltraViolet light, flares SLE in most patients .There is good
evidence that exposure of skin to UV light alters the location
and chemistry of DNA.
2. Drug-induced lupus. Drugs ( hydralazine, procainamide,
beta-blokers, isoniazid, penicillamine) can induce lupus.
Generally, lupus that is caused by a drug exposure goes
away once the drug is stopped.
3. Infection: There has been continuing interest in the
possibility that infectious agents might initiate or flare SLE.
c) Sex hormones
Female : Male
9
: 1
D)Abnormal immune system
1- molecular mimicry between external Ag and a self-Ag,
2- Defective regulatory mechanism.
3- nonspecific activation of T or B cells.
4- Autoantibodies to DNA, RNA:Circulating immune complexes
(Ag &Ab complexes are frequently observed and these may
deposit in the kidney, skin, brain, lung, and other tissues. It causes
inflammation and tissue damage by a number of mechanisms,
notably fixation and activation of the complement system which
cause damage in the tissues.
Clinical manifestations of SLE
General symptoms
The most common symptoms listed as initial
complaints are fatigue, fever, and weight loss.
• Fatigue
Low grade fever, anemia, or any source of
inflammation can result in fatigue.
• Raynaud’s phenomenon
it is commonly found in
lupus. It lack specificity.
(a triphasic reaction of distal
digits to cold or emotion, in
which the skin colour
changes from white to blue
to red)
Dermatological involvement
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•
•
•
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Up to 85% of SLE
Butterfly rash
Maculopapular eruption
Discoid lupus
Relapsing nodular non-suppurative
panniculitis
• Purpuric lesions
• Alopecia(loss of hair )
• Oral ulcer
Malar rash: This is a "butterfly-shaped" red rash
over the cheeks below the eyes and across the
bridge of the nose. It may be a flat or a raised
rash.
The rashes are made worse by sun exposure
Maculopapular eruption
Discoid lupus
These are red, raised patches with scaling of
the overlying skin.
Vasculitic skin lesin
Alopecia
Oral ulcer:
• Painless sores in the nose
or mouth .
Musculoskeletal system
• The arthritis of lupus is usually found on both
sides of the body and does not cause deformity of
the joints. Swelling and tenderness must be
present.
• The most frequently involved joints are those of
the hand, wrists and knees,.
• People with lupus can suffer from a certain type
of low blood flow injury to a joint causing death
of the bone in the joint.
• The muscle involvement was reported in 30-50%
of lupus patients
Kidney system
• Haematuria
• Proteinure (>0.5g protein/day )
R.B.Cs Casts in urine
Nervous system
• The brain , nerve problems and psychiatric syndromes
are common in lupus affecting up to two-thirds of
people.
• Potential disorders include seizures, nerve paralysis,
severe depression, and even psychosis.
• Spinal cord involvement in lupus is rare and occurs
primarily when there is clot formation in a critical
vessel that supplies blood to the spinal cord.
Hematological abnormalities
• Red blood cells
a normochromic, normocytic anemia is
frequently found in SLE. They appears to be
related to chronic inflammation, drug-related,
haemorrhage.
haemolytic anemia due to presence of a serum
antibody which attack red blood cells.
• White blood cell
leucopenia :its cause is probably a combination
of destruction of white cells by autoantibodies,
decreased marrow production, increased or
marginal splenic pooling for W.B.Cs.
it should also noted that the immunosuppressive
drugs used in the treatment of SLE may cause a
marked leucopenia.
Vasculitis
• Vasculitis in arteries
throughout the body
can account for signs
and symptoms from a
variety of organ
involvements. Seen
here is an artery with
extensive vasculitis
with chronic
inflammatory cells.
Pulmonary manifestations
• Pleurisy: inflammation of the pleura
• Pleural effusion:
Pleural effusions may occur in SLE patients either due to
direct pleural injury or secondary to nephrotic
syndrome, infection, cardiac failure.
Lung
• 1) acute lupus pneumonia:
• 2) pulmonary hemorrhage
3) chronic diffuse interstitial lung disease.
the diagnosis should not be made until infectious processes
such as viral pneumonia, tuberculosis, and other bacterial,
fungal and pneumocystis carinii infection have been
completely excluded.
Cardiovascular manifestations
• Pericarditis is the most common cardiac
manifestation of SLE.
• Myocarditis (Libman-Sacks endocarditis )and
valvular disease
• Hypertension, cardiac failure
SLE can be associated with endocarditis. Shown here is Libman-Sacks
endocarditis in which there are many flat, reddish-tan vegetations
spreading over the mitral valve and chordae
• .
Gastrointestinal and hepatic manifestation
• Esophagitis, dysphagia, nausea, vomiting: (drug related
in most cases).
• Chronic intestinal pseudo-obstruction, mesenteric
vasculitis, protein-losing enteropathy.
• Pancreatitis
• Lupus hepatitis
Eyes
• The eyes are rarely involved in lupus
except for the retina.
Secondary sjogren’s syndrome
• Dry eyes
• Dry mouth
exocrine glands were infiltrated with
lymphocytes
Secondary Antiphospholipid syndrome
• characterized by recurrent arterial and /or
venous thrombosis.
Deep venous thrombosis
• Notice the contrast
between the involved left
leg and the normal right
leg. Redness, swelling,
and warmth combined
with discomfort in the
involved leg are cardinal
manifestations of a deep
venous thrombosis.
Diagnosis
Criteria for diagnosing lupus
• The diagnosis of lupus is a clinical one
made by observing symptoms. Lab tests
provide only a part of the picture. To
receive the diagnosis of lupus, a person
must have 4 or more of these criteria:
Criteria for the diagnosis of SLE
1. Malar rash:
Fixed erythema over malar areas
2. Discoid rash: Erythematous raised patches with keratotic scaling .
3. Photosensitivity: Skin rash after exposure to sunlight.
4. Oral ulcers: Oral or nasopharyngeal, painless.
5. Arthritis:Tenderness, swelling, effusion in 2 or more peripheral joints
6. Serositis: A) pleurisy or B) pericarditis
7. Renal disorder A) proteinuria >0.5g/24hour
8. Neurological disorder: A) seizures or B) psychiatric disorder.
9. Haematological disorder: A) haemolytic anaemia or B) leucopenia or C)
thrombocytopenia
10. Immunologic disorder: A) positive LE cells or B) raised anti-native DNA antibdy
binding or C) anti-Sm antibody
11. Positive antinuclear antibody
Laboratory investigation
Autoantibodies in SLE
• Antibodies to cell nucleus component
anti-DNA, antibodies to nuclear antigen (anti-Sm)
• Antibodies to cytoplasmic antigens
anti-SSA, anti-SSB
• Cell-specific autoantibodies
lymphocytotoxic antibodies, anti-neuron antibodies,
anti-erythrocyte antibodies, anti-platelet antibodies
• Antibodies to serum components
antiphospholipid antibody
Lupus band test
• Immunofluorescence of skin
with antibody to IgG
demonstrates a band-like
deposition of immune
complexes that is bright green
at the dermal epidermal
junction in this skin biopsy
taken from an area with a
visible rash. With Systemic
Lupus Erythermatosus such
deposition can be found in both
involved and uninvolved skin
by a rash, whereas with Discoid
Lupus Erythermatosus the
immune complexes are found
only in involved skin.
Anti-nuclear antibodies
• The lupus
erythermatosus (LE) cell:
Is a mature neutrophilic
polymorphonuclear
leukocyte, which has
phagocytized a spherical,
homogeneous-appearing
inclusion, itself derived
from nuclear material of
degenerating leukocytes
and coated with antinuclear
antibody; a characterisitic
of lupus erythematosus