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Definition of SLE

A multisystemic autoimmune inflammatory disorder in which autoantibodies to a variety of autoantigens result in the formation and deposition of immune complexes

Classic presentation of SLE

Woman of childbearing age presenting with a triad of fever, joint pain and rash

3

Epidemiology of SLE

- affects 0.2% of the population


- 90% female


- child-bearing age


- more common in Afro-Carribeans & Asians

4

Clinical features of SLE

A relapsing-remitting picture with constitutional features, such as fatigue, weight loss, fever & myalgia




A RASH POINts and MD


- arthritis: involves peripheral joints, early morning stiffness


- Raynaud's/renal: proteinuria, increased BP


- ANA: positive in 95%


- serositis: pleuritis, pericarditis


- haematological: AIHA, decreased WCC & platelets


- photosensitivity


- oral ulcers


- immune phenomenon


- neurological: psychoses, seizures


- malar rash


- discoid rash

12

Immune phenomenon in SLE

- anti-double stranded DNA (anti-dsDNA): 60% specificity


- anti-smooth muscle (anti-Sm)


- anti-phospholipid


- extractable nuclear antibodies: positive in 30%, includes anti-Sm, anti-Ro, anti-La & anti-ribonucleoprotein (RNP)

4

Rashes in SLE

- malar rash: facial erythema sparing the nasolabial folds


- discoid rash: pigmented hyperkeratotic papules that become atrophic depressed lesions

2

Diagnosis of SLE

American Rheumatism Association criteria require four of the following:


- malar rash


- discoid rash


- photosensitivity


- oral ulcers


- arthritis


- serositis


- renal disorder


- neurological features


- haematological disorder


- immunological features


- ANA disorder.

10x

Monitoring disease activity in SLE

- anti-dsDNA titres


- complement: decreased C3 & C4


- increased ESR

Causes of drug-induced SLE


- antibodies

Will have anti-histone antibodies


- procainimide


- phenytoin


- hydralazine


- isoniazid

4

Maintenance therapy in SLE

Manage in specialist clinics


- joints: NSAIDs, hydroxychloroquine (DMARD)


- skin: sunblock, +/- low dose steroids

3

Severe flares of SLE

AIHA, lupus nephritis, pericarditis, CNS disease


- high dose prednisolone


- IV cyclophosphamide




- ACE inhibitors: proteinuria from lupus nephritis

3

Complications of treatment of SLE

- haemorrhagic cystitis


- pneumocystis pneumonia


- steroid side effects

3

Prognosis in SLE

80% survival at 15yrs

Antiphospholipid syndrome definition

An acquired autoimmune disorder that manifests clinically as recurrent thrombosis

Causes of antiphospholipid syndrome

- primary: 70%


- secondary to SLE: 30%

2

Immune complexes in antiphospholipid syndrome

Antiphospholipid antibodies


- anti-cardiolipin


- lupus anticoagulant

2

Features of antiphospholipid syndrome

CLOTs (DVT are venous, stroke is arterial)


- coagulation defect: increased aPTT


- livedo reticularis


- obstetric complications: recurrent 1st trimester abortion


- thrombocytopenia

4

Livedo reticularis

Treatment of antiphospholipid syndrome

- low dose aspirin


- warfarin: if recurrent thrombosis (INR: 3.5)

2