4. SYSTEMIC LUPUS ERYTHEMATOSUS (SLE)
BY DR WAQAR
MBBS, MRCP
ASST. PROFESSOR

6. Definition of autoimmune disease
Autoimmune diseases are conditions in which antibodies are formed against the person’s own body tissues
In some diseases, ab. are formed against one organ only ( DM type1, Grave’s disease) while in others, ab. are formed against many tissues

7. DEEFINITION OF SLE
SLE is an inflammatory autoimmune disease in which antibodies are formed against many tissues of the body ( multisystem disease).

8. EPIDEMIOLOGY Geographics : Occurs worldwide
Gender : F:M ratio is 9:1 ( usually young
women, aged 20 to 40).
Race : More in black Americans

9. ETIOLOGY
Like most autoimmune diseases, cause is unknown but some associations are observed:
If one twin is affected, more chances in the other ( ? Genetic role)
More chances in 1st degree relatives
More chances in premenopausal women ( ?
may be hormonal cause estrogens)
Some drugs can cause SLE like picture
* INH * Hydralazine * Penicillamine

10. PATHOGENESIS
Antibodies (ab.) are formed against various tissues of the body (antigens)
These ag-ab complexes initiate inflammatory response this causes S/S

11. Signs & Symptoms S/S of SLE are “very” varied & may be mild to severe
BUT REMEMBER 3 MOST COMMON FEATURES
Fatigue Arthralgias Skin rash

12. Gen. features: Fatigue, fever, wt. loss
2) Joint & Muscles: Most common complaint
*Pain in small joints of hands ( like R.A.)
* Other joints may also be affected
* Usually no joint swelling ( but may be)
* Myalgias
* Jaccoud’s arthitis (rare): Ulnar deviation of
fingers, due to chronic arthritis

14. SKIN RASH IN SLE
3) Skin: Affected in 85% of cases * Butterfly rash on the face a) Involves the cheeks b) Crosses over the nose c) No involvement of the nasolabial fold

16. Skin features (contd.)
* Photosensitivity : Prolonged exposure to sunlight worsens the rash ( & other symptoms also) *Raynaud’s phenomenon: Vasospasm in the digits after exposure to cold. * Hair fall & patches of alopecia * Discoid skin lesions : Seen in “discoid lupus”, a variant of SLE * Painless oral ulcers

17. Raynaud’s Phenomenon

18. Discoid skin lesions in SLE

19. Oral ulcers

20. 4) Lungs :. Pleurisy & pleural effusions (exudate). Atelectasis
4) Lungs : * Pleurisy & pleural effusions (exudate) * Atelectasis * Restrictive lung disease * Lungs may become small ( shrunken lung ) 5) Kidneys( Lupus nephritis) : SLE causes various types of glomerulonephritis which present as either nephrotic syndrome or nephritic syndrome. W.H.O. has classified lupus nephritis into 5 classes based on histology of the renal lesion (class 1 to 5). Please remember the following only: * Class 4: Membrano proliferative GN. Most common renal lesion in SLE & causes nephritic syndrome( MPGN) * Class 5: Membranous nephropathy. Causes nephrotic syndrome.

21. Lupus Nephritis (contd.)
a) Routinely check urine in SLE patients. If any blood or protein, investigate further ( biopsy) b) Treatment of lupus nephritis depends on the class of lesion which is present, so biopsy is important.

22. 6) CVS :. Pericarditis & Myocarditis
6) CVS : * Pericarditis & Myocarditis * Non-bacterial endocarditis of the mitral valve ( vegetations of platelets & fibrin) ( also called Libmann-Sacks endocarditis) * Vasculitis ( any organ) 7) CNS : * Siezures * Depression & psychosis * CVA * other neuro S/S 8) EYES : * Retinal infarcts * Sec. Sjogren’s syndrome

23. 9) GIT : * Mouth ulcers * Vasculitis of the intestinal vessels causing bowel infarction 10) BLOOD : * Anemia * Low WBC * Low platelets (antibodies are formed against RBC, WBC & platelets & cause destruction) Many features are due to vasculitis, eg siezures, CVA, bowel infarction, retinal infarcts etc.

24. INVESTIGATIONS CBC : * Anemia * Low WBC * Low plts. Urea/creatinine :
* Raised in advanced renal disease ( lupus
nephritis)
3) Urine : * Proteinuria * Blood wth RBCs & RBC
casts
4) ESR : Raised in acute flare up
5) CRP : Usually normal (C reactive protein)

25. Investigations (contd.)
6) Autoantibodies : Important ones are : * ANA : Present in 95% of pts. Not specific but done as the first screening step. * Anti double stranded DNA ( anti ds DNA) ( * v. specific for SLE - Present in 70% pts., specially during acute flare) * Anti Ro * Anti La * Anti Sm DRUG INDUCED LUPUS HAS : 1) A.N.A & 2) “Anti-histone” antibody.

26. 7) Complement levels : * C3 & C4 levels in blood are often low in acute flare up of SLE 8) Renal Biopsy : * Done if nephritis is suspected ( abn. urine) * Shows the class of renal disease, which helps us decide about drugs * Renal biopsy shows IgG & C3 deposits in the glomerular apparatus.

27. 9) Brain MRI/CT : Done if CNS involvement is suspected 10) Biopsy of skin or kidney shows: * Deposits of IgG & C3( characteristic)

28. TREATMENT
THERE IS NO CURE FOR SLE Drugs used : * NSAIDs ( diclofenac, ibuprofen etc) * CORTICOSTEROIDS * IMMUNOSUPPRESSIVE AGENTS ( Cyclophosphamide,Myco-pheno-late mofetil, Methotrexate, Azathioprine) * HYDROXYCHLOROQUINE

29. TREATMENT
1) Fatigue, arthralgias, fever, pleuritis * NSAIDS * If NSAIDS don’t help, then Chloroquine/Hydroxychloro. Can cause retinal toxicity, so regular eye checks 2) Skin rash: * Topical steroids * Sunscreens * Hydroxychloroquine tab. 3) Avoid long sun exposure 4) Periods of rest during acute flares

30. Treatment (contd.)
5) Severe flares of arthritis, pleuritis, pericarditis * Short/long term corticosteroids 6) Renal Disease: a) Class 1 & 2: No treatment b) Class 3 to 5: * High dose corticosteroids + immunosuppressive agents given as a course ( for a limited time) 7) CNS disease: Steroids + immunosupp. agents

31. COURSE OF SLE Most patients have relapsing-remitting course.
In these patients, treatment is given during relapse & tapered gradually
Some patients have a chronic persistent course & Rx is given continuously.
DRUG INDUCED LUPUS RESOLVES AFTER
STOPPING THE DRUG

32. PROGNOSIS
Mortality has fallen dramatically in the last 50 yrs due to advanced Rx.
10 yr survival rate is 90%
Causes of death :
- C.A.D.: Most common cause. (It is due to
accelerated atherosclerosis)
- Severe renal disease
- CNS disease( stroke, cerebritis)
- Infections (due to low WBCs)

33. PREGNANCY & SLE
Fertility is normal but increased chances of miscarriages, preterm labour.
SLE is not a contraindication to pregnancy but tell the patient about the risks
Exacerbations can occur during pregnancy & specially after delivery
Corticosteroids, azathioprine & hydroxychloroquine are safe in preg.

34. LIFE THREATENING COMPLICATIONS OF SLE
Renal disease
CNS involvement
Low plts & low WBC
Autoimmune hemolytic anemia
The above need aggressive immunosuppressive
therapy & corticosteroids

35. Points about drug induced Lupus
No CNS or renal involvement
ANA & anti-histone ab. present
Resolves after stopping the drug

36. NEXT SLIDE IS VERY IMPORTANT

37. Allah has made “SALAT” Laazim 5 times a day BUT HE has made good “AKHLAAQ” Laazim 24 hours a day ! SO, BE GOOD TO OTHERS !

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