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ERYTHEMATOSUS
June,2014
SYSTEMIC LUPUS ERYTHEMATOSUS
Receptor ligation
ex: TNF, Fas
DNA fragmentation
Chromatin condensation
Cytoplasmic
blebbing
Clearance by phagocytes
Y
Apoptotic bodies
AUTOREACTIVITY Y
Immune complex formation
RBC Y C’
Y
Endo
BM
Intima
Complement fixation
Release of inflammatory,
vasoactive and chemotactic
RBC
C’ mediators
Disruption of endothelium
Y
C’ C’ Thickening of BM
Y Y
Infiltration of
inflammatory
cells
Tissue damage
EPIDEMIOLOGY
Age : peak 20s and 30s but any age can be affected
before 8 yrs unusual
Sex :more women affected ,10:1 during childbearing
age
Prevalence:1/2500
Severe fatigue
Fever
Weight loss
Anorexia
Lymphadenopathy
Mucocutaneous manifestation
Frequency: 76%
Malar rash
Discoid lupus
Vasculitis (purpura, petechiae)
Raynaud’s phenomenon
Nail involvement
Alopecia
Photosensitivity
Oral/ nasal ulcers
MALAR RASH
Fig 65-10
MALAR RASH
Photosensitivity
Erythematous raised
patches with adherent
keratotic scaling and
follicular plugging
Atrophic scarring may
occur in older lesions
Oral lesions of SLE
Erythema of hard
and soft palate,
papules ,vesicles
and petechiae
Erythematous rash
of the tongue.
Oral Ulcers
Oral or
nasopharyngeal
ulceration,
usually painless,
observed by
physician
Joint
Pleuritis :
convincing history of pleuritic pain ,pleural
rub heard by a physician or evidence of
pleural effusion
or
Pericarditis:
documented by ECG ,pericardial rub or
evidence of pericardial effusion
Pulmonary Findings In SLE
Incidence: 5-67%
May be subclinical (abnormal PFTs)
Pleuritis
Pleural effusion
Pneumonitis
Pulmonary hemorrhage
Pulmonary hypertension
Restrictive pulmonary disease & diffusion
defects most commonly observed
abnormalities on PFTs
Cardiovascular Findings
In SLE
Pericarditis
Myocarditis
Sterile valvular vegetations (rarely
clinically significant except for risk of
bacterial endocarditis)
Arrhythmias
Cor pulmonale
Vasculitis (small vessels)
Atherosclerosis/ Coronary Heart disease
Dyslipoproteinemias
Renal Findings In SLE
Most common cause of morbidity & mortality
Hypocoagulable states:
Anti-platelet antibodies--decreased numbers of
platelets or decreased function (increased
bleeding time)
Other platelet dysfunction and thrombocytopenia
Anti-clotting factor antibodies
Hypercoagulable states:
Antiphospholipid Antibody Syndrome (APS): more
later
Protein C and S deficiencies
Conjunctivitis
Photophobia
Monocular blindness
transient or permanent
Blurred vision
Photosensitivity:
Rash as a result of unusual reaction to sunlight, by
patient history or physician observation
Pleuritis or pericarditis
Pleuritis—convincing history of pleuritic pain or rub heard by a
physician or evidence of pleural effusion, or
Pericarditis—documented by electrocardiogram or rub or evidence of
pericardial effusion
Cytopenias:
Hemolytic anemia with reticulosis or
leukopenia (<4,000/mm3) or
lymphopenia (<1,500/mm3) or
thrombocytopenia (<100,000/mm3)
Nephritis:
Proteinuria >0.5 gm/dL or >3+ or
Cellular casts
1997 CRITERIA FOR THE
CLASSIFICATION OF SLE (cont)
Encephalopathy:
Seizures
Psychosis
Positive ANA
Positive immunoserology:
Anti-DNA antibody to native DNA in abnormal titer, or
Anti-Sm, or
Positive finding of antiphospholipid antibodies based on (1)
an abnormal serum level of IgG or IgM anticardiolipin
antibodies, (2) a positive test for lupus anticoagulant using
a standard test, or (3) a false-positive serologic test for
syphilis known to be positive for at least 6 months and
confirmed by negative Treponema pallidum immobilization
or fluorescent treponemal antibody absorption test
The revised criteria for the classification
of systemic lupus erythematosus
1. Serositis
8. Immunologic
2. Oral Ulcers abnormalities:
3. Arthritis
-Anti-ds- DNA
4. Photosensitivity
-Anti- Sm
5. Blood disorders:
-Hemolytic anemia -Antiphospholipid
-Leukopenia -False +ve VDRL
(Lymphopenia) 9. Neurologic
-Thrombocytopenia abnormalities
6. Renal disorders 10. Malar rash
7. ANA positive 11. Discoid rash –
rimmed with scaling,
follicular plugging
CLASSIFICATION CRITERIA