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SYSTEMIC LUPUS ERYTHEMATOSUS

Q. What is SLE ?
Ans: It is a multisystem disease characterized by the association of immunological abnormalities with pathological
changes 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 males

Q. 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 a
tenfold 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. 3
major 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 immune
tolerance to the intact particles is broken down, the autoantibody response diversifies to include more and more
components 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
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 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). Mechanisms
involved 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, free
radicals, histamine.
 Induces the expression of adhesion molecule--- ICAM 1 --- increased vascular permeability and
exudation 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 IS
OFTEN 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 ingestion
of sprouts, alphalpha which contains the amino acid ------ L- canavanine.

Q. Discuss the ARA criteria for the diagnosis of SLE


Ans: There are 11 criteria of which 4 must be there to make a diagnosis. SENSITIVITY AND SPECIFICITY IS 96%

criteria defination
1. MALAR RASH It is a fixed erythema, flat or raised over the malar area tending
( 50%) to spare the nasolabial folds.

2. DISCIOD RASH Erythematous raised patches with adherent keratotic scaling


(25%) and follicular plugging; atrophic scarring may occur in older
lesions.

3. PHOTOSENSITIVE Skin rash as a result of unusual reaction to sunlight by patient


history or physician observation.

4. Oral ulcer (25%) Usually painless, observed by a physician.


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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 a. persistant proteinuria


(67%) > 0.5 gm/day or,
> 3+ if quantification is performed.

b. cellular cast – red cell, hemoglobin, granular, tubular or


mixed.

8. Neurologic a. Seizures in the absence of DRUGS OR METABOLIC


disorder (25%). DERANGEMENTS.

b. Psychosis in the absence of DRUGS OR METABOLIC


DERANGEMENTS.
9. Hematologic a. HEMOLYTIC ANEMIA with reticulocytosis
disorder
b. LEUCOPENIA < 4000 / microlitre on two or more occasions

c. LYMPHOPENIA < 1500 / microlitre on two or more occasions

d. THROMBOCYTOPENIA < 100000 / microlitre in the absence


of offending drugs.
10. immunologic a. anti-ds DNA
disorder
b. anti- Sm

c. antiphospholipid antibody based on the finding of ---


 Abnormal serum level of IgG or IgM anticardiolipin
antibody.
 Positive test for lupus anticoagulant ( see below)
 BFPT for syphilis present for > 6 months and
confirmed by TPI or FTA- ABS.
11. ANA An abnormal titer of ANA by IF at any point of time and in the
absence of drugs known to be associated with drug induced
lupus syndrome.

Q. What are the immunological abnormalities of SLE?


Ans:
1. Positive LE CELL TEST.
2. ANA
3. Anti cytoplasmic antibodies
4. anti- neuronal , anti-phospholipid, anti-platelet and anti-RBC
5. BFPT for syphilis.
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Q. What is LE cell ?
Ans: LE cell is a PMN leukocyte (neutrophil or macrophage) which has ingested basophilic staining nuclear
material from a degenerative white cell in presence of LE cell factor (anti – DNA Ab). Polymorphs containing
homogenous pink purple inclusion showing no residual chromatin pattern can be accepted as LE cell. Nucleus
(purple) of the polymorph is pushed to the periphery with a thin rim of cytoplasm (sky blue).

Sometimes large masses of nuclear material are found extracellularly and these are surrounded
by leucocytes forming ROSETTES.
LE cell is present in 80% cases of SLE. If LE cells are present in large numbers, it suggests SLE.

Occasional LE cell in -------


 Chronic DLE
 Drug induced LE
 Systemic sclerosis
 Rheumatoid arthritis.
Q. What is the mechanism of LE cell formation?
Ans: circulating ANA cannot penetrate intact cells. If cell nuclei are exposed, the ANA can bind to them ---- this
denatures the nuclei( nuclei loses its chromatin pattern and becomes homogenous) and subsequent fixation of
complement renders the antibody coated nuclei strongly chemotactic for the phagocytic cells.

Q. What is LE cell phenomenon?


Ans. It is the demonstration of LE cell in normal blood when a serum of a patient with LE cell factor has been
added to it.

Q. Is ANA specific for SLE? Why?


Ans: No. ANA are not specific but are sensitive. Using human cells e.g. Hep2 cells as substrate, of ANA are
positive in 95% cases SLE. ANA also occur in:

 Normal individuals (5-15% of normal individuals have low titres of ANA).


 Other autoimmune diseases.
 Chronic inflammatory conditions.
 Drugs
 Viral infections.
ANAs are directed against several nuclear antigens and can be grouped into 4 catagories :
1. Antibodies to DNA
2. Antibodies to histones.
3. Antibodies to nonhistone proteins bound to RNA
4. Antibodies to nucleolar antigens.

Clinically the most commonly used method to detect ANA is INDIRECT IMMUNOFLOURESCENCE. THE PATTERN
OF NUCLEAR FLOURESCENCE SUGGESTS THE TYPE OF ANTIBODY PRESENT IN THE PATEINTS SERUM. 4 BASIC
PATTERNS ARE RECOGNISED -------

 HOMOGENOUS or diffuse nuclear staining: Reflects antibodies to CHROMATIN, HISTONES and occasionally
DOUBLE STRANDED DNA.
 RIM or peripheral pattern: Reflects antibodies to double stranded DNA. Specific for SLE.
 SPECKLED PATTERN refers to the presence of uniform or variable sized speckles. It reflects the presence of
antibodies to non-DNA nuclear constituents. These components are easily extractable with buffered salt
solution and hence they are called EXTRACTABLE NUCLEAR ANTIGEN. Examples of ENAs are Sm antigen,
RNP, and SS-A and SS-B reactive antigens. THIS IS ONE OF THE MOST COMMONLY OBSERVED PATTERNS OF
FLOURESCENCE AND THEREFORE THE LEAST SPECIFIC.
 Anti-Sm, Anti- U1RNP, Anti- SS-A, Anti- SS-B, Anti- topoisomerase 1 (Scl-70) ----- fine
speckled.
 Anti-centromere: CREST syndrome.
 NUCLEOLAR PATTERN: refers to the presence of few discrete spots of fluorescence within the nucleus and
represents antibodies to nucleolar RNA. Anti- U3RNP, Anti- PmScl, Anti- RNA polymerase.

THE SO CALLED “SHRUNKEN PERIPHERAL PATTERN” IS THOUGHT TO BE ASSOCIATED WITH POOR


PROGNOSIS AND HIGH INCIDENCE OF RENAL DISEASE.
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A titer of <1:16 ANA can be ignored. A TITRE OF 1: 64 IS SIGNIFICANT.

Q. How many patients are ANA negative and how do they present?
Ans: 5-10 % patients are ANA negative but anti cytoplasmic antibodies positive i.e.
60% have anti- Ro
30% have anti-La
25% have anti- ss DNA.

THEY HAVE MANY SIMILARITIES LIKE SCLE like they have prominent malar rash, oral ulcers, photosensitivity
and SCLE like skin lesions. Systemic manifestations of SLE are rare.

Q. What are the markers of disease activity?


Ans:
1. High serum levels of ANA
2. Anti- ds DNA
3. Low levels of serum complement.
4. DNA binding activity: Radiolabelled DNA is incubated in test serum and DNA- antiDNA complex are
precipitated by 50% ammonium sulphate. Next the radioactivity of the supernatant and precipitate is
compared. >30% is considered abnormal. HIGH BINDING IS ASSOCIATED WITH POOR PROGNOSIS AND
RENAL DISORDER.

Q. What are the biologically false positive tests for syphilis?


Ans: Biologically false positive test are encountered in the standard nonspecific tests for syphilis where cardiolipin
(purified extract of beef heart) is used as antigen and the antibodies are detected in the serum of patients. These
tests include:
1. WASSERMANN CFT
2. KAHN TEST
3. VDRL SLIDE FLOCCULATION TEST
4. RAPID PLASMA REAGIN TEST
5. Automated RPR
6. VDRL- ELISA test.

Conditions where we get these tests false positive:


(A) ACUTE FALSE POSITIVE REACTION ( < 6 months)
 Recent viral infection or immunisation
 Genital herpes
 Malaria
 HIV infection
 Parenteral drug use.
(B) CHRONIC FALSE POSITIVE REACTION ( > 6 months)
 Aging
 SLE
 AI disorders
 RA
 Parenteral drug use.

Q. Discuss the significance of different autoantibodies in SLE?


Ans:
 Anti- ds DNA ( 70%): SPECIFIC FOR SLE . High titres associated with renal lupus and clinical activity.

 Anti- Sm (30%): SPECIFIC FOR SLE. Sm is a protein complexed to species of small nuclear RNA.
A/W renal, CNS disease and vasculitis.

 Anti – RNP (40%): characteristic of MCTD. It is a protein complexed to U1RNA.

 Anti- Ro (30%): It is a protein complexed to y1-y5 RNA. A/W increased photosensitivity, renal disease,
neonatal LE, Sjogren’s syndrome, SCLE.

 Anti- histone (70%): Low incidence of renal and CNS disease. IT IS PRESENT IN 95% CASES OF DRUG
INDUCED SLE.
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 Anti – ribosomal P (20%): A/W CNS lupus. The antigen is ribosomal P protein.

 Antineuronal antibody (60%): A/W diffuse CNS lupus. The antigen is present in the neuronal
surface.

 Anti – phospholipids antibody (50%): Thrombosis, Thrombocytopenia and Recurrent fetal loss.

 Other antibodies are anti-erythrocyte, anti- platelet, anti lymphocyte.

Q. What is antiphospholipid antibody ?


Ans:
This is a antibody directed against PLASMA PROTEINS ( prothrombin, annexin V, beta 2 glycoprotein,
protein C and Protein S) COMPLEXED WITH PHOSPHOLIPIDS .

The antibody is an acquired immunoglobulin (IgG/IgM). Since phospholipids are needed for normal
clotting process, these antibodies interfere with the normal clotting process. Hence they are also called as LUPUS
ANTICOAGULANT. It is identified in vitro clotting tests like prolonged APTT and kaolin clotting time
which is not corrected by normal plasma . Also by Russel Viper venom test.

IT IS PRESENT IN 14% CASES OF SLE.


It is also found in other conditions like-----
 Drug induced SLE
 Other CTD
 Carcinoma and lymphoma
Despite the circulating anticoagulant, half of the cases with antiphospholipid antibody have a
procoagulant effect. The possible mechanisms for this are:
 Decreased prostacyclin release
 Inhibition the anticoagulant effect of prostacyclin
 LE anticoagulant + phospholipid fraction of platelets ---- activation of platelets
----- THROMBOSIS.
 Direct endothelial injury.

Q. What is antiphospholipid syndrome ?


Ans: It is characterized by:
 THROMBOTIC EPISODES in
 legs ---- leg ulcer, gangrene, cutaneous necrosis
 renal
 cerebral
 pulmonary
 hepatic
 Placenta ---- fetal death ---- recur in subsequent pregnancies.
 LIBMAN SACKS ENDOCARDITIS
 THROMBOPHLEBITIS
 THROMBOCYTOPENIA ------- PURPURA, ECCHYMOSES.
 BFPT for syphilis.
All women with SLE, THROMBOSIS, and RECURRENT ABORTIONS AND BFPT for syphilis should be
screened for LUPUS ANTICOAGULANT. Treatment with Prednisolone 40-80 mg daily with Aspirin 75mg daily -------
RESULTS IN SUCCESSFUL PREGNANCY.

Q. What are the poor prognostic factors in SLE ? 50% mortality in 10 years.
Ans:
(A) Serious renal involvement in the form of
 High serum creatinine ( > 1.4 mg/dl)
 Nephrotic syndrome ( 24 hr urinary protein > 2.6 gm)
 Hypertension
(B) Serious CNS involvement
(C) Anemia. Thrombocytopenia
(D) Hypocomplementemia suggesting hightened disease activity
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(E) Antiphospholipid syndrome
(F) Low socio economic status

Leading causes of death in the 1st decade = INFECTION and RENAL FAILURE.
Leading causes of death in the 2nd decade = THROMBOEMBOLIC EVENTS.

Q. Discuss the features of DRUG INDUCED SLE ?


Ans.
 Uncommon in blacks ( SLE is most common in American blacks)
 Occurs in older age group (SLE occurs in the 2nd and 3rd decade mostly).
 F:M = 4:1 (SLE = 8:1)
 Increased incidence of HLA-DR4.
 Occurs more in slow acetylators.
 Renal and CNS involvement is uncommon. Most common systemic complaints are arthralgia.
 Anti-histone antibodies are frequent – 95% (in SLE = 70%)
 Anti DNA antibodies are absent
 Serum complements are normal.
 Typical skin manifestations of SLE are rare. They are mostly VASCULITIC, PYODERMA GANGRENOSUM like,
BULLOUS, ERYTHEMA MULTIFORME like.
 Resolves within weeks of stopping the drug. Others need a short course of steroids (2-10 weeks).
 Symptoms rarely persist beyond 6 months.

Q. What drugs are implicated in drug induced lupus?


Ans.
 Hydralazine
 Procainamide
 INH
 Penicillamine
 Griseofulvin
 OCP
 Beta blockers
 Captopril
 Gold
 Minoxidil
 Streptomycin
 Penicillin
 Tetracycline
 Sulfonamides
 Anticonvulsants
 Chlorpromazine
 Ibuprofen

Q. Discuss the relation of pregnancy with SLE


Ans. FERTILITY IS NORMAL IF RENAL FUNCTION IS GOOD.
It is ideal for a girl to contemplate pregnancy when there HAS BEEN CLINICAL REMISSION FOR 6 MONTHS.
This should indicate an uncomplicated pregnancy and live birth. Inactive disease is not associated with a disease
recurrence.
WORSENING OF SLE IS UNCOMMON IN PREGNANCY ESPECIALLY IN THOSE ON IMMUNOSUPPRESIVE
THERAPY.
IN PREGNANCY, IF THERE ARE
 NO FLARES
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 NO NEPHROPATHY
 NO PSYCHOSIS
Then, safely continue pregnancy, otherwise institute steroids to prevent postpartum flares just
before delivery.

In less than 10% cases permanent detoriaration of renal function can occur.
IN ALL PATIENTS , THERE IS A HIGHER RISK OF

 Premature delivery This may be because of immune complex


 Fetal loss : deposition on the trophoblast basement membrane
 Perinatal mortality or the transplacental passage of antiphospholipid
 Abortion. Antibodies ( anticardiolipin antibodies especially of
IgG type or the lupus anticoagulant.
In a case with recurrent abortions, it is ideal to screen for antiphospholipid antibody. If positive they
should be treated with daily prednisolone and aspirin. HOWEVER, THE PRESENCE OF THESE ANTIBODIES
WITHOUT RECURRENT ABORTIONS IS A NOT AN INDICATION FOR TREATMENT.
A patient of SLE on low dose steroids can be maintained thus during pregnancy also (sometimes,
high dose steroid during early pregnancy can cause cleft palate). Steroids can assist in the growth and
pulmonary maturity of a child. THE DOSE OF STEROIDS HAS TO BE INCREASED DURING DELIVERY AND
POSTPARTUM (STRESS). Also therapeutic abortion and caesarian section is contraindicated because of
increased stress.
For contraception, estrogen containing contraceptives are contraindicated. BARRIER CONTRACEPTIVES
ARE INDICATED.
Breast feeding is probably safe if the patient is on aspirin or low dose steroid but should probably be
avoided if other immunosuppressives are used.
Q. Compare SLE of childhood vs. elderly
Ans.

CHILDHOOD ELDERLY
 Earliest age of onset is 3 months  Increased incidence of lung disease
 The clinical picture, course and and Sjogren’s syndrome and lower
treatment are similar to the disorder in incidence of CNS disease and
the adults but CHILDREN USUALLY mesenteric vasculitis.
HAVE A MORE SEVERE DISEASE.  Anti-Ro and Anti- La are frequent.
 Liver and lymph nodes are most  Association with HLA – DR3.
commonly enlarged.
 CNS & KIDNEY may be involved.

Q. What is NEONATAL LUPUS ERYTHEMATOSUS?


Ans. It is a disorder thought to be caused by the transplacental passage of maternal antibodies. The most
frequent manifestations are (A) CUTANEOUS LESIONS & (B) CONGENITAL HEART BLOCK. It might be related to
HLA-DR3 haplotype.

The antibodies mostly implicated are


 Ro/SS-A antibody (100% cases of NLE): ANTI-Ro Ab is the MARKER FOR THE CONDITION . Used for
prenatal diagnosis.
 La/SS-B antibody( 50% cases of NLE)
 U1RNP.

These antibodies are not specific for NLE but are also seen in
 Sjogren’s syndrome
 SCLE
 Normal population (0.5-2%).

Approximately half of NLE patients manifest skin lesions which may be present at birth or occur in the first
few weeks of life. The most common finding is an ERYTHEMATOUS SLIGHTLY SCALY ERUPTION ON THE FACE AND
PERIORBITAL SKIN (raccoon sign/ owl-eye/ eye-mask) with the SCALP, TRUNK, EXTREMITIES, NECK AND
INTERTRIGINOUS AREAS involved in the decreasing order of frequency. The eruption can be exacerbated by UV
exposure and PHOTOTHERAPY. The rash improves by 4-6 months of life and has usually completely resolved
without scarring BY 12 MONTHS.
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COMPLETE HEART BLOCK occurs due to fibrosis of the conducting tissue of the heart. There may be
associated pericardial and pleural effusion, ascitis, cardiomyopathy, and IUGR and hydrops fetalis.

Other features are ----


 Hepatosplenomegaly
 Thrombocytopenia
 Coomb’s positive hemolytic anemia
 Neutropenia
 Aplastic anemia
 SPECKLED ANA AND ANTI-Ro ARE ALMOST ALWAYS PRESENT.
 ARA criteria are not fulfilled
 Involvement of joints, kidneys and CNS are rare.

Treatment:
1. sun avoidance
2. topical steroid
3. pacing

Regarding future pregnancies -----


 ladies with anti-Ro/anti-La antibodies should be made aware of the possible problems.
 The risk is low if such a lady does not suffer of CTD (SLE/ Sjogren) or gives birth to a child with NLE.
 THE RISK RISES WITH
 The presence of NLE in a previous child ( 25-50% risk)
 PRESENCE OF MATERNAL LUPUS ( 1:60 risk)
 PRESENCE OF HLA-DR3 in mother

Prognosis : some children can develop SLE in later life and some mothers can develop CTD in later life.

Q. what is the GILLIAM CLASSIFICATION OF SKIN LESIONS ASSOCIATED WITH LUPUS


ERYTHEMATOSUS?
Ans.
Skin lesions are classified as
1. LE specific
2. LE non specific skin lesions.

(1) LE specific skin lesions


(A) ACUTE CUTANEOUS LE ( ACLE) : 80% of SLE cases have these and 20% present with these.
 Localised ACLE ( malar rash) : butterfly blush or discrete maculopapular eruption with fine
scaling on the “butterfly” distribution of the face.
 Generalised ACLE

(B) SUBACUTE CUTANEOUS LE ( SCLE)


 Annular LE
 Papulosquamous SCLE

(C ) CHRONIC CUTANEOUS LE ( CCLE) : DLE lesions occur in SLE patients. In 10% they are the presenting
lesion and in 33% they occur during the course of the disease.
 Classic discoid LE
 Hypertrophic / verrucous LE
 LUPUS PROFUNDUS
 Mucosal LE
 Lupus tumidus
 Chilblain LE
 Lichenoid DLE
 Bullous DLE
 Rosacea like DLE
 Annular atrophic DLE
 LE gyratum repens
 Rowell’s syndrome
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(2) LE nonspecific skin lesions
(A) Cutaneous vascular diseases
(a) VASCULITIS
---- LEUCOCYTOCLASTIC (palpable purpura, urticarial vasculitis)
---- PERIARTERITIS NODOSA.

(b) VASCULOPATHY
----- Degos disease like lesions
----- Secondary atrophie blanche
(c ) PERIUNGUAL TELANGIECTASIA
(d) LIVEDO RETICULARIS---- COMMON IN PATIENTS WITH CNS LUPUS.
(e) THROMBOPHLEBITIS
(f) RAYNAUD’S DISEASE
(g) ERYTHROMELALGIA
(h) PURPURA ---- causes are : thrombocytopenia, cutaneous arteritis, steroid therapy.

(B) Non scarring alopecia : mostly in active stage ( 50% of patients with active stage have this). Hair
recovers once the disease becomes inactive but the lupus hair tends to persist longer.
 Lupus hair: occurs in 30% patients. These are coarse, dry, brittle, broken off shortened unruly hair
especially on the frontal margin. They have slowed anagen growth. They usually persist longer.
 Telogen effluvium
 Alopecia areata.

(C ) Sclerodactyly, digital pitted scar

(D ) Sclerodermatous skin changes in face and limbs although mat like telangiectasia are rare.

(F) Panniculitis

(G) Urticaria

(H) Bullous lesion

(I) Rheumatoid nodule

(J) Papulonodular mucinosis

(K) Calcinosis cutis

(L) Erythema multiforme

(M) Leg ulcer

(N) Widespread icthyosis

(O) Occlusion of large and medium sized arteries also occur suddenly---- may need amputation of the limb.

Q. What are the changes of SLE in hand ?


(A) Reticulate telangiectatic erythema on the palms ( thenar and hypothenar eminence), pulp and back of the
fngers.
(B) Small vascular necrosis on the tips of fingers and alongside the nails.
(C) Ragged cuticle
(D) Digital tip gangrene
(E) Dilated nail fold capillaries
(F) Over nails ---- splinter hemorrhages, pitting, ridging, onycholysis, striate leukonychia, clubbing
(G) Recurrent osler’s nodes ---- painful erythematous papules on the pulp of the finger due to arteritis or
embolism.

Q. What are the vesicobullous skin changes in LE ?


Ans.
(A) LE specific
 TEN like ACLE.
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 TEN LIKE SCLE.
 Vescicobullous SCLE.
 Bullous DLE.

(B) LE non specific


 Bullous SLE
 Bullous pemphigoid
 Dermatitis herpetiformis
 Pemphigus erythematosus
 Porphyria cutanea tarda.

Q. What is BULLOUS SLE ?


Ans.
It is a separate subset of SLE with subepidermal blisters and neutrophilic microabscess, nuclear dust and
fibrin at the tips of the dermal papillae (like DH). It is a transient autoimmune blistering condition that occurs in
the setting of SLE.

IMMUNOFLOURESCENCE shows the linear deposition of IgA, G, M , C3 at DEJ ( unlike DH but like BP).
However electron microscopy shows that the reactants are in the sublamina densa and not in the lamina lucida as
in BP.

TARGET ANTIGEN IS TYPE VII collagen (in some).

Bulla predominantly occur in the face, neck and upper trunk but maybe widespread. Heal with scar and milia
formation. Also, photosensitivity +.

Nephritis is common. Also hypocomplementemia and anti-ds DNA .

HYDRALAZINE can precipitate this.

Treatment of choice is Dapsone alone or in combination with steroid. In resistant cases, thalidomide is used.

Q. What is SENEAR – USHER SYNDROME ?


Ans.
It is also known as PEMPHIGUS ERYTHEMATOSUS. Here patients have immunological features of 2 diseases---
pemphigus foliaceous and lupus erythematosus namely----
 Granular IgG and C3 in the DEJ.
 Intercellular IgG and C3 in the epidermis.
 Circulating ANA.
 CIRCULATING ANTIBODY AGAINST DG1 (160kD).

But anti- ds DNA and ENA are absent. Progression to SLE is rare.

It is characterized by RED SCALY LESIONS IN A BUTTERFLY DISTRIBUTION SIMULATING LE or SEBORRHOEIC


DERMATITIS. PHOTOSENSITIVITY IS PRESENT. Lesions elsewhere like pemphigus foliaceous.

Condition can be brought about by PENICILLAMINE, PROPANOLOL, CAPTOPRIL.

It can be associated with thymoma and myasthenia gravis.

Q. discuss the mucous membrane lesions of SLE ?


Ans.
 These occur in 26%.
 They usually occur in the PALATE ( 82%), BUCCAL MUCOSA AND GUMS in the active phase of the disease.
 Lesions start as small reddish or purplish areas which break to shallow ulcers with dirty yellowish base and
a red halo.
 Histology and immunology is like the skin --- this differentiates it from LP.
12
 LIPS : cracked, oedematous and crusted.
 Tongue infarction may occur --- A/W anticardiolipin antibodies.
 Ulceration of the mucous membrane of the nose may lead to bleeding and perforation.
 Erythema can occur in other mucosal surface like the vulva and perianal area.
 HYDRALAZINE CAUSES OROGENITAL ULCERATION.

Q. Discuss the joint involvement of SLE?


Ans.
 Occurs in 90%
 Manifestations are :
 Symmetric small joint arthralgia, arthritis ( arthralgia > arthritis, erosions are less
common, deformity is less than RA).

DIFFERENCE BETWEEN JOINT INVOLVEMENT OF SLE AND RA.

FEATURES SLE RA
Deforming arthritis 25% Common
Sc nodules 5% 25%
X-Ray erosions rare Common
Kidney involvement common Rare
LE cell test 80% 15%
ANA 90% 20%
R-factor 40% 80%
 Other musculoskeletal manifestations include --- myalgia, myositis, tendonitis, avascular necrosis of
bones.

Q. Discuss the pulmonary involvement in SLE?


Ans.
 Most common feature is TRANSIENT PLEURISY. In two third some effusion develops.
 Pulmonary function tests may show diffusion abnormalities even when no changes may occur in the X-
ray.
 Lupus pneumonitis occurs with fever, dyspnoea, and cough. X-Ray shows fleeting lung infiltrates. This
responds to steroids (the most common cause of infiltrates in SLE is INFECTION). HIGH INCIDENCE OF
anti-Ro in pneumonitis.
 Later, interstitial pneumonitis and pulmonary hypertension may develop.
 In X- Ray: BILATERAL ELEVATION OF DIAPHRAGM WITH LINEAR SHADOWS OVER THE LOWER LUNG
ZONES is characteristic of SLE.

Q. Discuss about the renal changes in SLE?


Ans.
 Renal involvement is present in 67%.
 The involvement is significant in assessing prognosis.
 Most patients have Ig deposits in the glomeruli but only one half have clinical nephritis defined by
proteinuria.
 Classification of lupus nephritis ( WHO)

CLASS I: normal glomeruli by light microscopy, electron microscopy and immunoflorescence.


CLASS II: mesangial glomerulonephritis : there is
CLASS III : focal glomerulonephritis
A: focal segmental GN.
mild proteinuria but the patients maintain good renal function.
B: focal proliferative GN.
Characterized by proteinuria (occasionally nephritic syndrome) and hematuria.

CLASS IV: diffuse GN. : there is proteinuria, hematuria, RBC cast, nephrotic syndrome,renal insufficiency and
hypertension. IMMEDIATE TREATMENT SHOULD BE STARTED AT THIS STAGE OTHERWISE PROGRESS TO RENAL
FAILURE.
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CLASS V: diffuse membranous GN: severe proteinuria and nephritic syndrome.
CLASS VI: Advanced sclerosing GN: ESRD.

 INDICATIONS OF RENAL BIOPSY :


 Presence of any active urine sediment.
 Proteinuria > 500-1000 mg/day
 Creatinine > 1.1 mg/dl
 Prior to initiating cytotoxic agents
 Restaging, prognosis and adjustment of treatment.

 PATIENTS AT RISK OF SEVERE NEPHRITIS:


 With persistently abnormal urianalysis
 High titres of anti-ds DNA
 Hypocomplementemia.
 PROGNOSIS:
 Without renal involvement 15 year survival is 84%
 With renal involvement 15 year survival is 57%.

Q. Discuss the IF pattern in LE?


Ans.
Immunoglobulins ( mainly IgG also IgM, IgA) together with complement (C1,C3) and properdin can be
demonstrated in the DEJ by immunoflourescence. Single immunoglobulin deposition which are less prominent in
DEJ and more striking around the blood vessel----- favours another diagnosis (systemic sclerosis, DM, MCTD, RA,
Sjogren’s disease, pemphigoid, DH, Hypocomplementemic vasculitis, erythema multiforme, porphyria, GA, NLD,
GVHD, amyloidosis, pyoderma gangrenosum, leprosy, PLC).

 DEPOSITION IS MORE IN LESIONS MORE THAN 6 WEEKS, OVER SUN EXPOSED AREAS, UNTREATED
LESION.

 Staining may be HOMOGENOUS ( chronic lesion), THREADY ( active lesion) or STIPPLED pattern ( in
uninvolved skin).

 + in 80% in sunexposed involved skin in SLE AND DLE.

 + in three- forth in sunexposed uninvolved skin of SLE

 + in half in nonexposed uninvolved skin of SLE. Presence of IgG suggests poorer prognosis.

 Both fibrin and properdin have been demonstrated in the lesion of SLE --- PROPERDIN DEPOSITION IN
NORMAL SKIN CORRELATES WITH DISEASE ACTIVITY.

 Deposits are also present in the blood vessels in skin lesions and uninvolved skin.

 Epidermal nuclear deposits usually giving a speckled IgG pattern occurs in the basal cells of epidermis.

In DLE, deposits do not occur in the uninvolved skin. C1q is present in the deposits only in 29% of cases in
DLE (vs. 90% in SLE). These patients have greater risk of SLE.

Q. What is Lupus like syndrome?


Ans:
 There is C2 deficiency
 Decreased incidence and low titres of anti- ds DNA.
 Infrequent occurrence of immunoglobulin and complement deposition in the skin
 Frequent anti- Ro antibody.
 Decreased renal disease.

Q. What are evidences for and against that DLE AND SLE are the same entity?
Ans.
(i) evidences in favour :
 the cutanoeus lesions in both clinicohistologically are the same
14
 certain clinical features are present in both
 Similar lab abnormalities are present in both though more common in SLE.
 DLE patients occasionally go on to develop SLE
 SLE patients develop DLE lesions
 Conditions like LUPUS PANNICULITIS occur in both the two conditions.
(ii) evidences against:
 The risk of patients of DLE developing SLE is very small. Higher in disseminated DLE ( 22%)
compared to localised DLE ( 1.2%).
 This risk is not increased by various forms of stress, UVL, drugs chemicals etc.
 DIF pattern is different.
 Age and sex pattern is different
 3 forbidden clones of lymphocytes synthesizing cellular autoantibodies develop in SLE BUT only one
develop in DLE.

Those patients ( HLA-B8 +) who convert from DLE to SLE and those with SLE who have discoid lesions
must be genetically predisposed to both the conditions.

CONCLUSION: PATIENTS OF DLE WITH HEMATOLOGICAL AND SEROLOGICAL ABNORMALITIES ARE NOT
CASES OF SLE IN DISGUISE BUT ARE CASES OF DLE , A SAPERATE ENTITY FROM SLE WITH DIFFERENT
GENETIC BACKGROUND.

Q. Risk factors for the development of SLE in a patient with DLE?


Ans.
(i) Diffuse nonscarring alopecia
(ii) Disseminated DLE
(iii) Female with DLE before 40 years.
(iv) HLA-B8 type
(v) Generalised lymphadenopathy
(vi) Periungual nail fold capillaries
(vii) Raynaud’s phenomenon
(viii) SCLE/ACLE skin lesions
(ix) LE nonspecific skin lesions like vasculitis.
(x) Anaemia, leucopenia, high ESR, persistently positive ANA, hypergammaglobulinemia, BFPT for syphilis,
positive sunprotective nonlesional lupus band test.
(xi) Elevated soluble IL2 receptors.

Q. Define DLE ?
Ans. It is a relatively benign condition of the skin most frequently involving the face and characterized by
VARIOUS SIZED, USUALLY COIN SHAPED, ERYTHEMATOUS, WELL DEFINED SCALY PLAQUES WHICH TEND TO HEAL
WITH ATROPHY, SCARRING AND PIGMENTATION. THE SCALES ARE ADHERENT AND PLUG THE FOLLICULAR
ORIFICES.

The histology is characteristic.

There are hematological and serological changes in about half of the patients and these changes, with
other evidence, suggest an autoimmune etiology.

LOCALIZED DLE: The lesions are confined to the face above the chin.
DISSEMUNATED DLE: Lesions occur below the chin also. Hematological and serological abnormalities are more
frequently seen. More chance of developing SLE.

Q. what is the sex incidence and age incidence of DLE?


Ans. F: M = 2:1, age of onset = 4th decade.

Q. any familial incidence ?


Ans. Yes, 4 %.

Q. Discuss the etiology of DLE.


Ans.
(1) Genetic:
> Associated with HLA-B7, B8, Cw7, DR2, DR3 AND DQw1.
(2) Environmental:
15
> Sunlight, Extreme cold, Strong winds, Premenstrual period and Early pregnancy.
(3) Drugs
> PENNICILAMINE, GRISEOFULVIN, DAPSONE.
(4) Viruses.

Q. Discuss the histopathology of DLE? Compare it with SLE AND SCLE.


Ans.
STRATUM CORNEUM: there is hyperkeratosis with follicular plugging.
EPITHELIUM:
 Thinning and flattening of the stratum malphigii.
 Hydropic degeneration of basal cells (liquefaction degeneration): most significant finding.
 Dyskeratosis
 Basilar keratinocytes show individual cell necrosis (apoptosis ) ---- present as round- ovoid , homogenous
eosinophilic structures 10 micrometer called CIVATTE BODIES present in lower epidermis or in the
papillary dermis. They are PAS + , diastase resistant and on DIF contain IgG,M, complement and fibrin.
 Basilar keratinocytes acquire elongated contours like their superficial counterparts rather than retaining
their normal columnar appearance ---- squamotisation.
 The undulating rete ridge pattern is gone ad replaced by a linear array of squamotised keratinocytes.

BASEMENT MENBRANE:
 Thickening and tortousity in long standing cases.
 PAS+, diastase resistant material is found not only in the DEJ but along the follicular dermal junctions as
well. These findings correlate with immunoreactant deposits.
 Capillary walls show thickening, homogenization and increase in PAS staining intensity.

STROMA:
 Predominantly lymphocytic infiltrate arranged along DEJ, around hair follicles and appendages
(with hydropic degeneration of basal cells).
 By impinging on the pilosebaceous units, infiltrate causes gradual atrophy and disappearance.
 Also dermis shows----- EDEMA, RBC extravasation and MELANOPHAGES, INTERSTITIAL MUCIN DEPOSITION.

SUMMARY: The salient features in the HPE of DLE is ( at least 2 must be present)
(A) Liquefaction degeneration of basal cell layer.
(B) Degenerative changes in the connective tissue characterized by hyalinization, edema and
fibrinoid change most marked immediately below the epidermis.
(C) A patchy dermal lymphocytic infiltrate with few plasma cells and histiocytes particularly
around the appendages.

SPECIAL HPE features IN THE VARIENTS OF DLE :


(A) VERRUCOUS DLE :
 PAPILLOMATOSIS
 LARGE NUMBERS OF DYSKERATOTIC KERATINOCYTES.

(B) TUMID DLE:


 DERMAL FORM OF LE without surface or epithelial changes
 SUPERFICIAL AND DEEP DERMAL INTERSTITIAL AND PERIVASCULAR LYMPHOCYTIC INFILTRATE
ASSOCIATED WITH STROMAL MUCIN DEPOSITS.

(C) LE PROFUNDUS:
 SUBCUTANEOUS TISSUE MAYBE INVOLVED WITH OR WITHOUT INFLAMMATION IN THE DERMIS AND
DEJ.
 LOBULAR PANNICULITIS ---- fat necrosis is followed by fibrin deposition leads to hyalinization of
adipose lobules.
 Stromal mucin deposition in well eshtablished cases.
 Vascular changes like endothelial prominence, thrombosis, calcification and perivascular
fibrosis .

SCLE: It differs from SCLE in that the changes are most intense in the DEJ. They consist of -----
 Less prominent hyperkeratosis and inflammatory infiltrate than discoid lesions
16
 Hydropic degeneration of basal cells sometimes severe enough to form clefts.
 Colloid bodies ( common)
 Dermal edema
 Focal RBC extravasation and dermal fibrinoid deposits (common).

SLE: LIKE SCLE.


INFILTRATE MAY GO UPTO THE SUBCUTIS.

Q. Discuss the clinical features of DLE.


Ans
Type of DLE Features Sites
1. Classic DLE: Well defined coin shaped erythematous Face (check, bridge of the
The classical plaques covered by prominent adherent nose), scalp, ears, V areas of
‘tin tack sign’ scales that extend into the orifices of the neck, extensor aspect of
is also found in dilated hair follicles. They expand with the arm, forearm, hands, back
pemphigus erythema and pigmentation in the and sides of the toes.
foliaceous. periphery while in the centre there is
atrophy, telangiectasia and
hypopigmentation.
2.warty LE Warty hyperkeratotic lesion with red Mostly seen on the nose,
raised edge. temples, ears, scalp, palms and
soles.
3.tumid LE Tissues are SWOLLEN, BRAWNY, WARM May involve a whole cheek or
AND TENSE. The surface shows reddish limb.
mottled appearance due to scarring. Many
centimeter in diameter
4.annular Centre Of the plaque is depressed and Face, neck, behind ears.
atrophic sclerotic resembling morphea
5.LE gyratum Migratory gyrate annular erythema with
repens HPE features of DLE. LUPUS BAND TEST
NEGATIVE. There may be underlying
carcinoma.
6.Chilblain Chilblains develop usually after some Chilblain over acral areas,ears,
lupus (6%). years of development of DLE lesions on nose
More in female the face. DLE usually remit but chilblain
persists longer than cool months.

Variant of DLE Features site


7. LE profundus. An Nodular lesions of various sizes, Cheeks (most common),
unusual variety of LE in firm, rubbery, sharply demarcated face, arms, breasts ( lupus
which cut. Infiltrate occurs and persistent. The overlying skin mastitis, may herald SLE),
primarily in the deeper is normal. Healing with depressed buttocks, trunk or legs.
portions of corium with areas or areas of anetoderma. 50% have SLE ( less severe
only microscopic than the typical SLE)
epidermal changes.
8.LE hyperthrophicus Violaceous scaly tender lesion Face, arms, dorsal aspect of
et profundus rapidly enlarges and develops into hands and back.
a hard, brown black tarlike plaque.
9. rosaceous LE: 7.5% Reddish nodular lesions on the FACE
nose, cheeks, forehead and
sometimes the chin A/W diffuse
redness and easy flushing. NO
PUSTULES.
10. BULLOUS DLE
11. ROWELL’S The EM lesions last from a few Sites of EM are ---UPPER
SYNDROME: ERYTHEMA days to over a month. There are no PART OF THE BODY
MULTIFORME occurs in precipitating factors for EM. including face
association with DLE/SLE. PATIENTS FREQUENTLY HAVE
Speckled type of ANA is PERNIOTIC LESIONS ALSO.
A/W Rheumatoid factor
17
and anti-La(SS-B)

12. MUCOSAL DLE: 25% Mostly affects the oral mucosa as LP like lesions,erythematous
of DLE patients have patches with depressed centre, superficial ulcerations, lip
mucosal lesions. crusting, scar, leukoplakia and SCC.
Lower palpebral conjunctivitis, superficial punctuate keratitis,
Redness in the vulva and anus.
13. NAIL DLE Nail fold- erythema, telangiectasia,
clubbing, paronychia
Nails – red lunula, pitting,
leukonychia striata, onycholysis,
subungal hyperkeratosis.
14. LE telangiectoides: C/B persistent blotchy reticulate in the face, neck, ears, back
occurs in SLE & DLE. telangiectasia . Healing by of hands, breasts, heels and
punctuate atrophic scarring. sides of feet

Q. What are the associations of DLE?


Ans.
 Facial telangiectasia
 Bilateral enlargement of parotid
 Livedo reticularis
 Porphyria
 Pemphigus
 Myasthenia, thymoma
 Hereditary C2 deficiency.

Q. what percentage of DLE patients have scalp involvement?


Ans: Scarring alopecia develops in one third and usually permanent.

Q. What are the lab abnormalities in DLE


Ans. Frequency of lab abnormalities is less in DLE compared to SLE
 HEMATOLOGICAL----
 Anemia, leucopenia, thrombocytopenia ( one third)
 Raised ESR (20%).
 Positive Coomb’s test.
 Cryoglobulins and cold agglutinins
 Hypergammaglobulinemia
 BFPT for syphilis --- 26%.
 LE cell test --- 1.7%
 ANA ---- 35%, HOMOGENOUS = TWICE SPECKLED.
 Anti-ssDNA + in one fifth.--------- A/W WIDESPREAD AND PROGRESSIVE DISEASE.
 Anti- dsDNA + in 0-27% cases.

Q. Which cases of DLE take a long time to remit ?


Ans.
 Lesions of long standing with much scaling and scarring
 H/O Raynaud’s phenomenon, chilblains
 Scalp involvement.

Q. What percentage of LE patients present with SCLE ?


Ans. 10%.

Q. What is the presentation of SCLE?


Ans. There are mainly 2 types of lesions :
 NONSCARRING PAPULOSQUAMOUS ( two third)
 ANNULAR POLYCYCLIC ( one third)

Lesions mainly occur above waist and particularly around the neck, on the trunk and outer aspects of the
arms. The borders may show vesciculation and crusting. FOLLICLAR PLUGGING AND HYPERKERATOSIS are not
18
prominent and the lesions resolve leaving grey white hypopigmentation and telangiectasias. The pigmentary
changes eventually resolve spontaneously.

Other features are -----


 Photosensitivity ( 50%)
 Diffuse nonscarring alopecia ( 50%)
 Mouth ulcers
 Livedo
 Periungual telangiectasia
 Localised scarring DLE.

Q. How many fulfill the ARA criteria?


Ans. 50%. Arthritis is the most frequent systemic feature. Other systems involvement are rare.

Q. ANA is positive in how many ?


Ans. 60-80%. Homogenous variety.

Q. anti-Ro and anti-La positive in how many ?


Ans. 70-90% and 30-50%.

Q. lesional lupus band test positive in how much ?


Ans. 60%. Dust like particle pattern of IgG deposition around epidermal basal keratinocytes is more specific of
SCLE. Reflects in vivo bound Ro/SS-A antibody.

Q. HLA predisposition in SCLE ?


Ans. HLA B8 and DR3.

Q. What drugs precipitate SCLE ?


Ans.
 Griseofulvin
 Terbinafine
 ACE inhibitor
 CCBs
 Hydrochlorthiazide
 Sulfonylureas.

Q. discuss the treatment of LE.


ANS.
TREATMENT OF DLE
1. GENERAL MEASURES:
 Sun protection
 sunscreen
2. FOR RAYNAUD’S:
 Pentoxyphylline
 Other vasodilator ( CCBs)
3. TREATMENT PROPER :
(A) TOPICAL : Topical/IL steroids; IL Interferon alpha; laser.
(B) SYSTEMIC:
--- ORAL ANTIMALARIAL: Hydroxychloroquin :400mg BD or 800mg BD for 6 months.
After stopping treatment, 50% relapsed within 6 months and repeat courses are effective.
However severity of relapses decreases with time.
---- SYSTEMIC GLUCOCORTECOID: daily pred 5mg OD/TDS
OR, Pulse therapy ( Psoriasiform scalp lesions)

----- BETA CAROTENE 50mg TDS


----- CLOFAZIMINE 100 mg daily
----- DAPSONE 100 mg daily
----- ETRETINATE (1mg/kg/day): with chloroqiun was effective in WARTY VARIETY.
----- ISOTRETINOIN ( 80mg/day)
19
---- THALIDOMIDE: For cases not responding to antimalarials, topical steroids and
sunscreen. 100-200 mg /day for 4-6 weeks then taper to 25-100 mg as maintenance. After
stopping treatment, 71% relapsed but further courses were effective
(C) MISCELLENEOUS :
 Cyclophoshamide ( 50-200mg daily)
 Azathioprin
 Large doses of vitamin E
 Sulfasalazine 0.5mg TDS.
 Phenytoin 100mg TDS.

(D) SURGERICAL INTERVENTION


---- LASER
---- DERMABRASION
---- EXCISION.

TREATMENT OF SLE
1. GENERAL MEASURES ----
> Sun protection
> Bed rest during exacerbation
> Avoid secondary infection, mental and physical stress
> Correct anemia

2. SPECIFIC TREATMENT IN SPECIAL CONDITIONS


> for hypertension ---- hydralazine
> diuretics for edema
> anticonvulsants for epilepsy
> NSAIDS for arthralgia
3. TREATMENT PROPER ----
topical : steroids, tacrolimus
systemic :
 Corticosteroids : in acute cases start with 60mg daily---- taper gradually to a maintenance of 5-
15 mg daily.
 Antimalarials
 Immunosuppresives :
 Cyclophosphamide : 1.5 – 2.5 mg/kg daily. Indicated in very severe disease, organ or life
threatening potentially reversible.
 Azathioprine : in moderately severe disease with slow detoriaration alongwith high dose steroid
 Methotrexate : 7.5 mg weekly in steroid resistant cases without renal and CNS involvement. 10-20
mg in mucocutaneous lesions.
 Cyclosporin: 3-5 mg/kg used in resistant cases.
 Methyprednisolone pulse: for patients not controlled by oral prednisolone and
immunosuppresives.
 Plasmapheresis: In patients with high levels of immune complexes whose condition is
deteriorates despite other therapy. 3-4 days/week over a period of 2-3 weeks. Useful in
managing life threatening complications like fulminating vasculitis or CNS disease.
 IV gamma globulin
 Dapsone: 200-300 mg daily.
 Thalidomide
 Retinoid
 Interferon alpha, vitamin E, phenytoin, sulphasalazine, danazol, phototherapy

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