SLE Flashcards
what happens to cause lupus?
Lupus is an auto-immune condition where the body creates auto-antibodies against a variety of auto-antigens which results in immune complexes. Inadequate clearance of these immune complexes results in immune response causing tissue inflammation and damage
who does SLE more commonly occur in
Women
Those of Afro-caribbean, south asian and mexican descent
when is SLE commonly diagnosed
during women’s reproductive years. between 40-50, often with a delay to diagnosis.
what is the diagnostic criteria used for diagnosing SLE?
Which criteria must you have?
SLICC.
Need to have at least 1 clinical and 1 serological criteria and a total of 4, to get a diagnosis but if you have biopsy proven lupus nephritis and positive immunology this is also diagnostic.
what are the common features of SLE?
constitutional symptoms (fatigue being a major complaint), cutaneous manifestations, arthralgia and arthritis
what type of arthritis is seen in SLE
non-erosive.
May see Jaccoud’s artropathy sometimes: chronic non-erosive reversible joint disorder that may occur after repeated bouts of arthritis. inflammation of the joint capsule and subsequent fibrotic retraction, causing ulnar deviation of the fingers
name some respiratory features of SLE
o Pleurisy o Pleural effusions o Acute pneumonitis o Diffuse alveolar haemorrhage o Pulmonary hypertension o Shrinking lung syndrome
name some CVS features of SLE
o Pericarditis +/- effusion o Myocarditis o Valvular abnormalities o Coronary heart disease – high risk of morbidity and mortality long-term o Risk of MI 50x
name some neuropsychiatric manifestations of SLE
headache (unremitting), anxiety and mood disorder, seizure, demyelination, GBS, mononeuritis
name some haematological consequences of SLE
o Anaemia of chronic disease
o Autoimmune haemolytic anaemia
o Thrombotic thrombocytopenic purpura (TTP) – MAHA, low platelets, fever, neuro/renal involvement - check aPLS antibodies
o Leukopenia
o Can have associated lymphadenopathy and splenomegaly
o Thrombocytopenia
o Mild or ITP
name some ENA you might find in SLE
Ro/La, Ds-DNA, Dm
what will you find with regard to complement in a patient with active SLE?
complement consumption (will have low levels). C3 is more specific than C4.
what would be standard care for non-acute SLE
high-factor sunblock and hydroxychloroquine. If they had a skin flare you would try topical steroid first
NSAIDs (unless they have renal disease)
what drugs would you use in moderate SLE
mild disease drugs - sunblock, hydroxychloroquine, NSAIDs.
Oral prednisolone or steroid-sparing agents such as methotrexate, azathioprine etc.
If someone had severe SLE how would you treat them
mild disease drugs - sunblock, hydroxychloroquine, NSAIDs.
Oral prednisolone or steroid-sparing agents such as methotrexate, azathioprine etc
Add in high dose steroid, biologics, DMARDS, B cell therapy, cyclophosphamide etc.
what would a bone marrow aspirate in someone with SLE show?
reactive bone marrow
what pattern of ESR and CRP is commonly seen in SLE
ESR disproportionately elevated compared to CRP
which investigations would you order 1st line in a suspected case of SLE
- FBC and differential
- Activated PTT
- Urea and electrolytes
- Erythrocyte sedimentation rate (ESR) and CRP
- Antinuclear antibodies, double-stranded (ds) DNA, Smith antigen (anti-Sm)
- Urinalysis
- Chest x-ray
- ECG
- Would consider complement levels and anti-phospholipid antibodies but not essential as 1st line
what is Sjogren’s syndrome
Chronic auto-immune disorder where there is lymphocytic infiltration and fibrosis of exocrine glands, especially lacrimal and salivary glands.
Can be primary (females, 40-50) or secondary to connective tissue disease e.g. SLE
list some features of Sjogren’s syndrome
decreased tear and saliva production. Parotid swelling, vagianl dryness leading to dyspareunia, dry cough
list some systemic signs of Sjogren’s syndrome
polyarthritis/arthralgia, Raynaud’s syndrome, lyphadenopathy, vasculitis, peripheral neuropathy, fatigue.
how do you test for Sjogren’s syndrome
Schirmer’s test - measures conjunctival dryness.
ANA usually +.
Anti-RO and Anti-LA antibodies may be present.
how do you treat Sjogren’s syndrome
artificial tears, NSAIDs and hydroxychloroquine for arthralgia
what is systemic sclerosis
what are the two types
auto-immune disease of the connective tissue characterised by scleroderma due to accumulation of collagen and by injuries to small arteries.
Localised and diffuse.
Localised - face, hands and feet
how do you manage systemic sclerosis
no cure. Immunosuppression is used for organ involvement and any progressive skin disease.
Need to monitor BP due to risk of pulmonary hypertension as well as renal function.