Systemic lupus erythematous Flashcards
What is SLE?
- Systemic lupus erythematosus (SLE), also known simply as lupus, is an multisystemic autoimmune disease in which the body’s immune system mistakenly attacks healthy tissue in many parts of the body.
What is the pathophysiology of SLE?
- Autoantibodies are made against a variety of autoantigens (ANA) which form immune complexes
- Inadequate clearance of immune complexes results in a host of immune responses which cause tissue inflammtion and damage
What is the prevalence of SLE?
- Mostly women
- Women of child-bearing age
- African-Caribbeans
- Asians
- Family history
What are the clinical features of SLE?
- Remitting and relapsing illness of variable presentation
- Features are non-specific
- Malaise
- Fatigue
- Myalgia
- Fever
- Organ specific - caused by active inflammation or damage
- Lymphadenopathy
- Weight loss
- Alopecia
- Nail-fold infarcts
- Non-infective endocarditiis
- Raynauds
- Stroke
- Retinal exudates
What is the immunological basis of SLE?
- 95% are ANA +ve
- A high anti-double stranded DNA antibody titre is highly specific but only positive in 60% of cases
- Anti-phospholipid antibody
- 40% are RF +ve
- SLE may be associated with other autoimmune conditions
- Sjogrens
- Autoimmune thyroid disease
What are the three best tests to diagnose SLE?
- Anti-dsDNA antibody titres
- Complement reduction
- This denotes the consumption of complement so their degradation products rise
- ESR
- ESR rises
- CRP normal (only high in arthritis serotitis or infection)
What other secondary tests are carried out to diagnose SLE?
Other tests:
- BP
- Urine for casts or protein
- Lupus nephritis
- FBC
- U&Es
- LFTs
- CRP
Which drugs can lead to drug-induced lupus?
- Isoniazid
- Hydralazine
- Procainamide
- Quinidine
- Chlorpromazine
- Phenytoin
- Minocycline
The disease remits if the drug is stopped
Which drugs worsen idiopathic SLE?
- Sulphonamides
- Oral contraceptive pill
How is SLE treated?
- High factor suncream
- Hydroxychloroquine
- For skin and joint
- Improves survival
- Topical steroids for skin flares
How is SLE remission maintained after initial treatment?
What is a mild flare of lupus compared to a moderate flare? How are both managed?
What is a severe flare of lupus and how is it treated?
Which two pairs of drugs should not be given together?
How is lupus nephritis treated?
- Intensive immunosuppressive with steroids
- Cyclophosphamide
- Mycophenolate
- BP control is vital
- ACEi
- Surgery
- Renal replacement surgery
- Done if disease progresses
- Nephritis recurs in 50% of patients post transplant (rar cause of graft failure)
- Renal replacement surgery
What is anti-phospholipid syndrome? What are the signs and symptoms?
- Can be associated with SLE
- Often occurs as a primary disease
- Antiphospholipid antibodies cause CLOTS:
- Coagulation defects
- Livedo reticularis
- Obstretic (recurrent miscarriages)
- Thrombocytopenia
Which vessels are affected by the thrombotic tendencies of antiphospholipid syndrome?
- Cerebral
- Renal
- Other vessels
How is antiphospholipid syndrome diagnosed?
Persistent antiphospholipid autoantibodies with clinical features
How is antiphospholipid syndrome managed?
- Anticoagulation
- Seek advice in pregnancy
What is the clinical criteria for diagnosing lupus?
- > 4 points (at least 1 clinical and 1 laboratory)
- or biopsy- proven lupus nephritis with positive ANA or anti-DNA
- Clinical criteria
- Acute cutaneous lupus - Malar rash
- Chronic cutaneous lupus - Discoid rash
- Non-scarring alopecia
- Oral/nasal ulcers
- Synovitis
- Serositis
- Urinanalysis
- neurological feautes
- Haemolytic anaemia
- Leucopenia
- Thrombocytopenia
What is the lab criteria for diagnosing lupus?
- > 4 points (at least 1 clinical and 1 laboratory)
- or biopsy- proven lupus nephritis with positive ANA or anti-DNA
Lab Criteria
- +ve ANA
- Anti-dsDNA
- Anti-Smith antibodies
- Antiphospholipid antibodies present
- Low complement
- +ve Direct Coombs test
How is an official diagnosis of lupus made in an appropriate clinical setting?
- > 4 points (at least 1 clinical and 1 laboratory)
- or biopsy- proven lupus nephritis with positive ANA or anti-DNA
What kind of rash is this?
What kind of rash is this?
Discoid rash
