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BPT Rheumatology > SLE > Flashcards

SLE Flashcards

1
Q

What proportion of females compared to males get SLE?

A

9:1

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2
Q

What is the incidence of SLE?

A

1-25 per 100,000

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3
Q

What is the 10 year survival rate of SLE?

A

80-90%

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4
Q

Which single gene mutations cause SLE?

A

C1q, C4A and B, C2, TREX1

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5
Q

What diseases are TREX1 gene mutations also associated with?

A

Aicardi-Gouteieres syndrome (early onset encephalopathy), RVCL (retinal vasculopathy with cerebral leukodystrophy)

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6
Q

Which types of genes are often involved in SLE?

A

MHC class, IFN related pathway genes, T/B cell signalling/survival, immune complex clearance

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7
Q

What are the different types of epigenetic changes in SLE?

A

hypomethylation of DNA, aberrant expression of miRNAs in peripheral blood cells, hypo or hyperacetylation in pro inflammatory genes

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8
Q

What percentage of patients with SLE have a first degree relative with an autoimmune disease?

A

22.7%

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9
Q

What environmental factors are involved in SLE?

A

UV light, infection, smoking, silica, pets with lupus

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10
Q

Which drugs can cause drug induced lupus?

A

procainamide, hydralazine, minocycline, diltiazem, penicillamine, isoniazid, qunidine, IFN-alpha, methyldopa, chlorpromazine, TNF inhibitors

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11
Q

What are the lupus specific mucocutaneous manifestations of lupus?

A

acute malar rash, generalised diffuse erythema, chronic discoid

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12
Q

What is the pattern of arthralgia in SLE?

A

symmetrical, polyarticular, migratory often affecting knees, wrists, fingers

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13
Q

What are some of the differences between arthritis in SLE vs RA?

A

synovial effusions uncommon, morning stiffness less prominent, non deforming, non erosive

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14
Q

What is Jaccoud’s arthropathy?

A

tenosynovitis, RA-like swan neck deformity and ulnar deviation

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15
Q

What are other musculoskeletal symptoms of SLE?

A

mylagias, myositis, fibromyalgia, avascular necrosis, tenosynovitis, tendon rupture, subcutaneous nodules

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16
Q

What are the cardiovascular manifestations of SLE?

A

pericarditis, tachycardia, CCF, arrhythmias, cardiomegaly, valvular disease, coronary artery disease

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17
Q

What is libman sacks endocarditis?

A

verrucous sterile valvular lesions

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18
Q

What are the pulmonary manifestations of SLE?

A

pleurisy, pleural effusions, interstitial lung disease, acute lupus pneumonitis, pulmonary hypertension, vanishing lung syndrome, alveolar haemorrhage

19
Q

What is vanishing lung syndrome?

A

progressive dyspnoea, elevated hemidiaphragm, reduced lung volumes, restrictive lung defect

20
Q

What are the gastrointestinal manifestations of SLE?

A

abnormal LFTs, ileal and colonic perforation, intestinal pseudo-obstruction, regional enteritis, ascites, hepatitis, acute pancreatitis

21
Q

What are the haematological manifestations of SLE?

A

anemia, leucopaenia, thrombocytopaenia, lympahedenopathy, splenomegaly

22
Q

What are the central neuropsychiatric manifestations of SLE?

A 
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%)
23
Q

What are the peripheral neuropsychiatric manifestations of SLE?

A 
GuillainBarresyndrome
Autonomic neuropathy
Mononeuropathy
Myasthenia gravis
Cranial neuropathy
Plexopathy
Polyuneuropathy<1%
24
Q

How is SLE renal disease classified?

A

Class I-VI based on histopathology

25
Q

What is the probability of SLE if ANA is negative?

A

< 0.14%

26
Q

Does ANA correlate with disease activity?

A

No

27
Q

What other rheumatological conditions may have positive ANA?

A

scleroderma, MCTD, PM/DM, RA, Sjogren’s

28
Q

What non rheumatic autoimmune conditions may have positive ANA?

A

hashimoto’s, grave’s disease, autoimmune hepatitis, primary biliary cirrhosis, primary autoimmune cholangitis

29
Q

Which infections may have positive ANA?

A

mononucleosis, hepatitis C, subacute bacterial endocarditis, TB, HIV

30
Q

Which autoantibodies are associated with neonatal lupus?

A

Ro, La

31
Q

What is anti dsDNA associated with?

A

disease activity, nephritis, TNF induced SLE

32
Q

What are measures of disease activity in lupus?

A

ESR > CRP, increase anti ds DNA Ab, low C3/C4

33
Q

What general measures should be implemented in managing all patients with SLE?

A

avoid triggers (stress, UV light, oestrogen, sulfonamides), vitamin D repletion, CV risk modification, immunisations, manage glucocorticoid side effects, minimise steroid use

34
Q

What is the treatment for minor disease SLE?

A

hydroxychloroquine, NSAIDs, steroids, occasionally

35
Q

What is the treatment for major disease SLE?

A

steroids, immunosupressive agents (azathioprine, methotrexate, leflunomide, cyclophosphamide, cyclosporine, tacrolimus, mycophenolate), rituximab

36
Q

What are the benefits of hydroxychloroquine in SLE?

A

decreased flares, decreased organ damage, improves survival, improves mycophenolate response in lupus nephritis, anti lipidaemic, anti thrombotic

37
Q

What is the main side effect of hydroxychloroquine?

A

retinal toxicity

38
Q

What is the treatment for lupus nephritis?

A 
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
39
Q

What type of immunosuppression is used for lupus nephritis?

A

IV pulse steroids and MMF or CYC

40
Q

What are the obstetric complications of SLE?

A

pre-term delivery, pre eclampsia, eclampsia, thrombosis, infection, PPH, C-section, thrombocytopaenia

41
Q

What are the predictors of obstetric complications of SLE?

A

active disease, prior lupus nephritis, APL Abs, thrombocytopaenia, primagravida

42
Q

What are the ‘contraindications’ to pregnancy in patients with SLE?

A

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

43
Q

How should positive anti phospholipid antibodies be treated in pregnancy?

A

if asymptomatic just low dose aspirin
if previous obstetric issues - aspirin and prophylactic clexane
if previous thrombosis - therapeutic clexane

44
Q

What is the most serious complication of nenonatal lupus?

A

complete heart block

BPT Rheumatology (8 decks)

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