SLE and CTD Flashcards
Where can SE affect?
Any part of the body
Briefly, what occurs in SLE?
Immune syste attacks body resulting in inflam and tissue damage. Antibody-immune complexes precipitate and cause further immune response, Immune complexes get stuck in and damage the basement membrane of blood vessels.
What aspects of genetic, enviromental and hormonal affect the pathogenesis of SLE?
Females > males urban > rural Increased incidence in asains, hispanics, afro-carribeans and afro-americans silica dust UV light EBV oestrogen exposure
What are the cardinal features of SLE?
Fever lethargy weight loss poor appetite malaise
What mucocutaneous features are often present in SLE?
Photosensitivity malar rash with sparing of nasolabial fold mouth ulcers alopecia areata Raynauds
How can SLE present in the cardiovascular system?
Pleurisy Infections Pulmonary HTN Pulmonary infarct diffuse lung infiltration and fibrosis
Why do patients with SLE require frequent urinalysis?
Renal disease from SLE presens with few symptoms very late on once the patient is in severe renal failure so do not want to miss this
What haematological features can be present in SLE?
Lymphadenopathy
leucopenia
anaemia
thrombocytopenia
How do you differentiate severe SLE from mild SLE?
Mild - skin rash, fatigue, mouth ulcers
Moderate - Sinusitis, joint swelling
Severe - Renal or neurological impairment
What investigations are required for SLE?
FBC urinalysis ENA complement levels ANA Anti-dsDNA
How useful are meauring autoantibodies in SLE?
Anti-nuclear antibody - gateway to CTD but not specific to SLE
Anti-dsDNA - highly specific to SLE and levels psitivelly correlate with disease activity
In anti-phospholipid syndrome, what autoantibodies are positive?
Anti-cardiolipin (also + in 30% SLE and 5% of healthy population)
lupus anticoagulant activity
What does anti-phospholipid syndrome cause?
Arterial/ venous thrombosis
Responsible for 15% of recurrent foetal loss
Responsible for 20% recurrent thrombosis in YP
Does anti-phospholipid syndrome affect men more than women?
No
What are other features of anti-phospholipid syndrome?
Superficial thrombophlebitis
mild/ moderate thrombocytopaenia
migrane
Libman Sachs endocarditis
What treatment is required for anti-phospholipid syndrome?
Life-long anticoagulation
aspirin and heparin during pregnancy
attention to vascular risk factors
Describe Sjogrens syndrome.
Lymphocyte infiltration of exocrine glands causing xerostomia (dry mouth) and keratoconjunctivitis sicca (dry eyes)
Which auto-antibodies can be positive in Sjogren’s syndrome?
anti-Ro
anti-La
Is Sjogren’s syndrome more common in men or women?
Women
What treatment can be given for Sjogren’s syndrome?
eye drope punctal plugs saliva replacement Pilocarpine (cholinergic agonist) HCQ Steroids and immunosuppression Attend to CV risk factors and dental hygiene
What auto-antibodies can be positive in systemic sclerosis?
anti-centromere antibodies
anti-Scl-70 antibodies
How is systemic sclerosis classified?
Limited or diffuse
What organs can systemic sclerosis involve?
skin kidneys gut muscle joints heart
What are the management options available for systemic sclerosis?
CaCB ACE inhibitor (HTN) Prednisolone (acute) Immunosuppression (inflammation) Prostacyclin (Iloprost) Bosentan, Sildenafil (pumonary HTN)
What auto-antibody is present in mixed connective tissue disease?
Anti-RNP
