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Lupus Erythematosus

Lupus Erythematosus

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SLE and Frequently asked questions..
SLE and Frequently asked questions..

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Published by: Lakshya J Basumatary on Feb 11, 2009
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01/28/2013

 
SYSTEMIC LUPUS ERYTHEMATOSUS
Q. What is SLE ?Ans: It is a multisystem disease characterized by the association of immunological abnormalities with pathologicalchanges affecting multiple organ systems particularly the skin, joints and vasculature.Q. What is the age of onset?Ans It tends to occur in EARLY ADULT LIFE. Peak age of onset is 38 years in female and 44.2 years in malesQ. What is the female:male ratio ?Ans:
8:1.
Q. Any familial cases?Ans: yes, in
10%.
Q. Discuss the ETIOPATHOGENESIS of SLE ?Ans: there are 4 theoretical sequential phases in the clinical expression of this disease---
(A)
INHERITANCE OF
SUSCEPTIBILITY 
GENE
(B)
INDUCTION
OF AUTOIMMUNITY
(C)
EXPANSION
OF AUTOIMMUNE PROCESS
(D)
IMMUNOLOGIC
INJURY 
.(A) SUSCEPTIBILITY PHASE: It involves the inheritance of genes that confer predisposition to SLE. There is atenfold difference in the heritability of SLE between dizygotic and monozygotic twins suggesting the presence of at least 4 genes:
MHC class II DR gene.
Genes encoding complement proteins and TNF.
Genes mediating apoptosis
Genes involved in cell signaling process.
Genes for clearance of immune complexes.(B) INDUCTION PHASE: It is C/B the appearance of autoreactive T cells that exhibit the loss of self-tolerence.Mechanisms involved are
Failure of central thymic or peripheral tolerance mechanism.
Aberrant expression of HLA-DR molecule
Molecular mimicry induced by infection
Presentation of cryptic peptides during apoptosis.
(C) EXPANSION OF AUTOIMMUNE PROCESS: During this phase, autoantibodies are produced by population of clonally expanded B cells which are directed against a limited number of nuclear and cytoplasmic antigens. 3major targets are ------
Nucleosome ( anti-DNA & anti-histone)
Spliceosome ( anti-Sm & anti –RNP antibodies)
Ro and La molecules (anti-Ro and anti-La).These autoantigens are clustered in distinct structures at the surface of apoptotic cells. Once the immunetolerance to the intact particles is broken down, the autoantibody response diversifies to include more and morecomponents of the complex. This phenomenon is called EPITOPE SPREADING.(E)IMMUNOLOGIC INJURY: The auto antibodies cause tissue injury by the following means :
Direct cell death
Opsonisation
Blocking of target molecule function
ADCC
1
 
IMMUNE COMPLEX DEPOSITION AND COMPLEMENT MEDIATED LYSIS.Q. Discuss the predispositions of SLE?Ans:
(1)
Role of genetics
: evidences are as follows ----
SLE is associated with HLA DR2 & DR3.
SCLE is associated with HLA B8, DR3.
DLE is associated with HLA B7, B8, DR2, DR3, DQA0102.
Genetic deficiencies of complement components
including C2,C3,C4,C5 & C1 esterase inhibitor------- are associated with DLE & SCLE.
TNF-α gene polymorphisms
are implicated ---- this promotes TNF-α production ---- photosensitivity.
(2)
Role of UVL
: it is the most important environmental factor in the induction of LE (UVB> UVA). Mechanismsinvolved are :
It
injures keratinocytes ----- autoantigen release
.( antigens expressed on the surface)
It
alters cellular DNA ---- immunogenic
Causes the
exaggerated release of immune mediators
such as ---- IL-2, TNF-a, PGE, proteases, freeradicals, histamine.
Induces the
expression of adhesion molecule--- ICAM 1
--- increased vascular permeability andexudation of inflammatory cells.
UVL
suppresses cutaneous
 
T cells and Langerhans cells
which suppress abnormal patterns of cutaneous inflammation.
(3)
Role of tobacco
: presence of LUPOGENIC AROMATIC AMINO AMINES
(4)
Role of drugs
: Induces T cell DNA hypomethylation ---- biological autoreactivity of lymphocytes. SKIN ISOFTEN SPARED IN DRUG INDUCED SLE.
(5)Role of viruses :
Induce virus induced apoptosis ---- expression of sequestered antigens on the cell surface.
EBV causes the overexpression of bcl-2 in the viral infected B cells ---- uncontrolled growth of B cells.
(6)
role of 
endocrine factors
: evidences are :
more in women
premenstrual flares
Precipitation of disease with OCP.
(7)
heavy metals
: CADMIUN, GOLD AND MERCURY are associated with autoimmunity
(8)
silicon breast implant
: associated with ---
scleroderma
lupus like syndrome
fibrositis
inflammatory myopathy
autoimmune thyroid disease
(9)
diet :
hemolytic anemia and high titers of ANA and anti – ds DNA antibodies are reported following ingestionof sprouts, alphalpha which contains the amino acid ------
L- canavanine
.Q. Discuss the ARA criteria for the diagnosis of SLEAns: There are 11 criteria of which 4 must be there to make a diagnosis. SENSITIVITY AND SPECIFICITY IS
96%
criteriadefination
1. MALAR RASH( 50%)
It is a fixed erythema, flat or raised over the malar area tendingto spare the nasolabial folds.
2. DISCIOD RASH(25%)
Erythematous raised patches with adherent keratotic scalingand follicular plugging; atrophic scarring may occur in olderlesions.
3. PHOTOSENSITIVE
Skin rash as a result of unusual reaction to sunlight by patienthistory or physician observation.
4. Oral ulcer (25%)
Usually painless, observed by a physician.
2
 
5. Arthritis (90%)
Nonerosive
arthritis involving
2 or more peripheral joints
C/B
tenderness
swelling
effusion
6. serositis
a.
 pleuritis
:
pleritic pain complained by patient
rub heard by physician
pleural effusion in X-ray or clinical finding
 b.
 pericarditis
:
rub heard by the physician
evidence of effusion
ECG.
7. RENAL DISORDER(67%)
a
.
 persistant proteinuria
> 0.5 gm/day or,> 3+ if quantification is performed.b. cellular
cast 
– red cell, hemoglobin, granular, tubular ormixed.
8. Neurologicdisorder (25%).
a.
Seizures
in the absence of DRUGS OR METABOLICDERANGEMENTS.b.
Psychosis
in the absence of DRUGS OR METABOLICDERANGEMENTS.
9. Hematologicdisorder
a.
HEMOLYTIC ANEMIA
with reticulocytosisb.
LEUCOPENIA
< 4000 / microlitre on two or more occasionsc
. LYMPHOPENIA
< 1500 / microlitre on two or more occasionsd.
THROMBOCYTOPENIA
< 100000 / microlitre in the absenceof offending drugs.
10. immunologicdisorder
a.
anti-ds DNAb. anti- Sm
c
. antiphospholipid antibody 
based on the finding of ---
Abnormal serum level of IgG or IgM anticardiolipinantibody.
Positive test for lupus anticoagulant ( see below)
BFPT for syphilis present for > 6 months andconfirmed by TPI or FTA- ABS.
11. ANA
An abnormal titer of ANA by IF at any point of time and in theabsence of drugs known to be associated with drug inducedlupus syndrome. Q. What are the immunological abnormalities of SLE?Ans:
1.Positive LE CELL TEST.2.ANA3.Anti cytoplasmic antibodies4.anti- neuronal , anti-phospholipid, anti-platelet and anti-RBC
5.
BFPT for syphilis.
3

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