Systemic Lupus Erythematosus (SLE) Flashcards
Systemic lupus erythematosus (SLE) is an i________ a______ c______ t_____ disorder
inflammatory autoimmune connective tissue
“Erythematosus” refers to the typical…
red malar rash across the face
Which group are most at risk of SLE?
Women
Asian, African, Caribbean and Hispanic
Young to middle-aged adults
What are risk factors for SLE?
Genetic defect
UV radiation
Smoking
Viral and bacterial infection
Certain medication
SLE typically takes a r_____-r____ course
relapsing-remitting
The result of chronic inflammation means that SLE can shorten life expectancy with c_______ disease and i____ as significant complications.
cardiovascular disease
infection
Which antibodies are seen in SLE?
Anti-nuclear antibodies (ANA) which act against proteins in the cell nucleus.
How may SLE present?
Malar rash, butterfly shaped across nose and cheeks.
Non-specific symptoms:
Fatigue
Weight loss
Arthralgia
Non-erosive arthritis
Fever
Lymphadenopathy
Splenomegaly
SOB
Pleuritic chest pain
Mouth ulcers
Hair loss
Raynaud’s phenomenon
Oedema due to nephritis
What triggers and worsens the malar rash in SLE?
Sunlight
What may a full blood count show in an SLE patient?
Anaemia of chronic disease
Low WBC count
Low platelets
CRP and ESR may by ____ with active inflammation
raised
C3 and C4 (complement) levels may be ______ in active disease (seen in SLE)
decreased
What may a urinalysis in SLE show?
Proteinuria
Give an example of another condition that may show positive for anti-nuclear antibodies?
Autoimmune hepatitis
(certain other autoimmune conditions)
Which antibodies are highly specific to SLE?
Anti-double strand DNA antibodies
(anti-dsDNA)
Note: only half of SLE patients have these
A_______ antibodies and a______ syndrome can occur secondary to SLE in 40% patients
Antiphospholipid
(increased risk of venous thromboembolism)
What criteria can be used for diagnosis, taking into account both the clinical features and autoantibodies present?
EULAR (European League Against Rheumatism)
or
ACR criteria (American College of Rheumatology criteria)
What is the leading cause of death from SLE?
CVD - chronic inflammation in blood vessels leads to hypertension and coronary artery disease
What can be the cause of anaemia in SLE?
Anaemia of chronic disease
Autoimmune haemolytic anaemia
Bone marrow suppression by medications
Kidney disease
What causes anaemia of chronic disease?
Chronic inflammation causes release of inflammatory cytokines which interfere with normal functioning of iron-regulatory hormone (hepcidin).
Leads to decreased iron absorption from gut, reduced release of iron for macrophages and impaired iron utilisation in developing RBCs.
What lung complications are there from SLE?
Pleuritis (causing sharp pain on inspiration) and Interstitial lung disease (inflammation in lung tissue leading to fibrosis)
What complication of SLE causes sharp chest pain that gets worse on lying flat?
Pericarditis
Due to blood volume in central veins increasing when lying flat (gravity). Increased pericardial pressure, exacerbates chest pain.
Neuropsychiatric SLE (inflammation in CNS) can present with optic neuritis, transverse myelitis (inflammation of spinal cord) or p____
psychosis
Recurrent m____ along with pre-eclampsia, pre-term labour and intrauterine growth restriction are all increased in SLE
miscarriage
V______ t_____ may be associated with antiphospholipid syndrome, secondary to SLE
Venous thromboembolism
Lupus nephritis can progress to….
ESRF
Treatment of SLE aims to control symptoms and reduce complications with minimum m____ and s___ e_____
medications and side effects
What measure help manage the malar rash?
Sun cream and sun avoidance
What are first line medications for SLE?
Hydroxychloroquine
NSAIDs
Steroids
What other condition is hydroxychloroquine used to treat?
Malaria
What are treatment options for more severe SLE?
DMARDs (eg cyclophosphamide, methotrexate)
Biologic therapies
What does DMARD stand for?
Disease-Modifying Antirheumatic Drugs
What are possible biological therapies?
Rituximab (monoclonal antibody targets CD20 on surface of B cells)
Belimumab (monoclonal antibody targets B-cell activating factor)
What is lupus nephritis?
Anti-nuclear antibody (ANA) deposition in endothelium
Secondary to SLE
What initiates the high prevalence of antinuclear antibodies (ANA)?
Damaged DNA from environmental triggers causes apoptosis. This process releases nuclear bodies and nucelar antigens into the blood stream. Clearance of the apoptotic bodies is ineffective due to some genetic defect. So increased nuclear antigens initiates immune response = production of ANA which bind to antigens and form immune complexes which deposit in tissues.
What kind of hypersensitivity reaction is seen in SLE?
Type 3
