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

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

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

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

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

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

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

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

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

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

Raynaud’s Phenomenon

Discoid skin lesions in SLE

Oral ulcers

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.

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.

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

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.

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)

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.

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.

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

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

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

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

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

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)

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.

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

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

NEXT SLIDE IS VERY IMPORTANT

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 !

TIRED? LETS REFRESH YOU