SLE Flashcards
What proportion of females compared to males get SLE?
9:1
What is the incidence of SLE?
1-25 per 100,000
What is the 10 year survival rate of SLE?
80-90%
Which single gene mutations cause SLE?
C1q, C4A and B, C2, TREX1
What diseases are TREX1 gene mutations also associated with?
Aicardi-Gouteieres syndrome (early onset encephalopathy), RVCL (retinal vasculopathy with cerebral leukodystrophy)
Which types of genes are often involved in SLE?
MHC class, IFN related pathway genes, T/B cell signalling/survival, immune complex clearance
What are the different types of epigenetic changes in SLE?
hypomethylation of DNA, aberrant expression of miRNAs in peripheral blood cells, hypo or hyperacetylation in pro inflammatory genes
What percentage of patients with SLE have a first degree relative with an autoimmune disease?
22.7%
What environmental factors are involved in SLE?
UV light, infection, smoking, silica, pets with lupus
Which drugs can cause drug induced lupus?
procainamide, hydralazine, minocycline, diltiazem, penicillamine, isoniazid, qunidine, IFN-alpha, methyldopa, chlorpromazine, TNF inhibitors
What are the lupus specific mucocutaneous manifestations of lupus?
acute malar rash, generalised diffuse erythema, chronic discoid
What is the pattern of arthralgia in SLE?
symmetrical, polyarticular, migratory often affecting knees, wrists, fingers
What are some of the differences between arthritis in SLE vs RA?
synovial effusions uncommon, morning stiffness less prominent, non deforming, non erosive
What is Jaccoud’s arthropathy?
tenosynovitis, RA-like swan neck deformity and ulnar deviation
What are other musculoskeletal symptoms of SLE?
mylagias, myositis, fibromyalgia, avascular necrosis, tenosynovitis, tendon rupture, subcutaneous nodules
What are the cardiovascular manifestations of SLE?
pericarditis, tachycardia, CCF, arrhythmias, cardiomegaly, valvular disease, coronary artery disease
What is libman sacks endocarditis?
verrucous sterile valvular lesions
What are the pulmonary manifestations of SLE?
pleurisy, pleural effusions, interstitial lung disease, acute lupus pneumonitis, pulmonary hypertension, vanishing lung syndrome, alveolar haemorrhage
What is vanishing lung syndrome?
progressive dyspnoea, elevated hemidiaphragm, reduced lung volumes, restrictive lung defect
What are the gastrointestinal manifestations of SLE?
abnormal LFTs, ileal and colonic perforation, intestinal pseudo-obstruction, regional enteritis, ascites, hepatitis, acute pancreatitis
What are the haematological manifestations of SLE?
anemia, leucopaenia, thrombocytopaenia, lympahedenopathy, splenomegaly
What are the central neuropsychiatric manifestations of SLE?
Aseptic meningitis Cerebrovascular disease (5-18%) Demyelinating syndrome Headache (24-72%) Movement disorder Seizure disorder Myelopathy Acute confusionalstate Anxiety disorder Cognitive dysfunction (55-80%) Mood disorder (14-57%) Psychosis (~5%)
What are the peripheral neuropsychiatric manifestations of SLE?
GuillainBarresyndrome Autonomic neuropathy Mononeuropathy Myasthenia gravis Cranial neuropathy Plexopathy Polyuneuropathy<1%
How is SLE renal disease classified?
Class I-VI based on histopathology
What is the probability of SLE if ANA is negative?
< 0.14%
Does ANA correlate with disease activity?
No
What other rheumatological conditions may have positive ANA?
scleroderma, MCTD, PM/DM, RA, Sjogren’s
What non rheumatic autoimmune conditions may have positive ANA?
hashimoto’s, grave’s disease, autoimmune hepatitis, primary biliary cirrhosis, primary autoimmune cholangitis
Which infections may have positive ANA?
mononucleosis, hepatitis C, subacute bacterial endocarditis, TB, HIV
Which autoantibodies are associated with neonatal lupus?
Ro, La
What is anti dsDNA associated with?
disease activity, nephritis, TNF induced SLE
What are measures of disease activity in lupus?
ESR > CRP, increase anti ds DNA Ab, low C3/C4
What general measures should be implemented in managing all patients with SLE?
avoid triggers (stress, UV light, oestrogen, sulfonamides), vitamin D repletion, CV risk modification, immunisations, manage glucocorticoid side effects, minimise steroid use
What is the treatment for minor disease SLE?
hydroxychloroquine, NSAIDs, steroids, occasionally
What is the treatment for major disease SLE?
steroids, immunosupressive agents (azathioprine, methotrexate, leflunomide, cyclophosphamide, cyclosporine, tacrolimus, mycophenolate), rituximab
What are the benefits of hydroxychloroquine in SLE?
decreased flares, decreased organ damage, improves survival, improves mycophenolate response in lupus nephritis, anti lipidaemic, anti thrombotic
What is the main side effect of hydroxychloroquine?
retinal toxicity
What is the treatment for lupus nephritis?
Class I, II, VI - no immunosuppression Class III, IV, V - immunosuppression ACE inhibitor if proteinuria > 0.5g/day target BP < 130/80 statins if LDL > 2.6 plasma exchange for thrombotic microangiopathy
What type of immunosuppression is used for lupus nephritis?
IV pulse steroids and MMF or CYC
What are the obstetric complications of SLE?
pre-term delivery, pre eclampsia, eclampsia, thrombosis, infection, PPH, C-section, thrombocytopaenia
What are the predictors of obstetric complications of SLE?
active disease, prior lupus nephritis, APL Abs, thrombocytopaenia, primagravida
What are the ‘contraindications’ to pregnancy in patients with SLE?
severe pulmonary hypertension, severe restrictive lung disease, advanced renal insufficiency, advanced heart failure, previous severe pre eclampsia, stroke within previous 6 months, flare within previous 6 months
How should positive anti phospholipid antibodies be treated in pregnancy?
if asymptomatic just low dose aspirin
if previous obstetric issues - aspirin and prophylactic clexane
if previous thrombosis - therapeutic clexane
What is the most serious complication of nenonatal lupus?
complete heart block
