SLE

Question 1

Epidemiology?

Answer 1

Females in 30-50 age range

Question 2

What type of hypersensitivity?

Answer 2

Type III hypersensitivity (immune complexes)

Question 3

Which antibodies are present?

Answer 3

Anti-phospholipid
Anti-nuclear antigens:
Anti-dsDNA
Anti-histone
Anti-extractableNA (ENA) - Ro/La/RNP
RF
Anti mitochondrial:
Anti-cardiolipin

Question 4

Pathophysiology:

Answer 4

IC deposition
Direct cytotoxicity
NK cells and macrophages
Complement consumption
Activation of neutrophils

Question 5

Skin symptoms?

Answer 5

Photosensitive rash
Alopecia
Malar (butterfly) rash
Discoid rash on face, chest and trunk, rarely legs (erythematous raised patches with adherent keratotic scaling and follicular plugging)

Question 6

Joint symptoms?

Answer 6

Non-erosive arthritis
Tendinitis
Fibromyalgia - vague muscle pain, poor response to NSAIDs/analgesia

Question 7

Sicca symptoms?

Answer 7

Dryness of exocrine glands: salivary, lacrimal, genital tract
Oral ulcers

Question 8

Renal symptoms?

Answer 8

Oedema

Question 9

Vascular signs?

Answer 9

Raynaud’s phenomenon
Hypertension (may be a sign of lupus nephritis)
Lymphadenopathy (cervical and axillary, non-tender)
Vascular and venous thrombosis due to the presence of anti-phospholipid Abs

Question 10

Ches pain and SOB signs?

Answer 10

Pleuritis > pericarditis > peritonitis
Myocarditis, endocarditis
Premature arethromatous coronary artery disease
Rare: coexistent pleural effusions and shrinking lung syndrome

Question 11

Leukopenia normally cause by what?

Answer 11

Lymphopenia rather than neutropenia

Question 12

Investigations?

Answer 12

FBC
Activated partial thromboplastin time (PTT) - may be prolonged if anti-phospholipid Abs are present
U+E and creatinine
ESR and CRP
Auto-abs
Urinalysis for haematuria/proteinuria/casts
CXR for infiltrates, cardiomegaly, PE
ECG

Question 13

Treatment for joint symptoms and serositis?

Answer 13

Hydroxychloroquine
+Steroids
+Lifestyle
+Methotrexate and folic acid (if requiring steroids)

Question 14

Treatment for mucocutaneous disease?

Answer 14

Lifestyle and supportive
+Hydroxychloroquine
+Steroids
+Methotrexate and folic acid (if requiring steroids)

Question 15

Treatment for lupus nephritis?

Answer 15

Induction therapy + corticosteroid + hydroxychloroquine + lifestyle
+Maintenance regimen plus corticosteroid

Question 16

Treatment for neuropsychiatric lupus?

Answer 16

Cyclophosphamide + corticosteroid + lifestyle
+IV immune globulin
+Plasmapheresis
+CNS pharmacotherapy

Question 17

Why don’t you prescribe NSAIDs in SLE?

Answer 17

Increases the risk of strokes, MI and renal failure

Question 18

Onset:

Answer 18

After menarche

Decline after menopause

Question 19

Signs of subacute cutaneous lupus?

Answer 19

Most photo-sensitive rash in most areas

Associated with the Ro and La antibodies

Question 20

What is chilblain lupus?

Answer 20

Cold lesions that look like vasculitis

Question 21

Signs of small vessel lupus vasculitis?

Answer 21

Splinter haemorrhages
Digital infarcts
Palpable purpura
Vasculitic ulcers
Urticarial vasculitis

Question 22

Signs of medium/large vessel lupus vasculitis?

Answer 22

Digital ischaemia
Gangrene
Needing amputation

Question 23

Jaccoud’s arthritis:

Answer 23

Affects tendons and is not erosive so retain full range of movement - ask to make a fist

Question 24

Lupus atherosclerosis?

Answer 24

CVD

Peripheral vascular disease

Question 25

Features of lupus pneumonitis:

Answer 25

Acute organising pattern

Question 26

Neuropsychiatric lupus:

Answer 26

Psychosis
Seizures
Stroke
Transverse myelitis

Question 27

Lupus nephritis:

Answer 27

Nephrotic syndrome
Rapidly progressive GN (classes III + IV)
Proteinuria
Renal failure
Granular casts/dysmorphic fragmented red cells

Question 28

Biologic treatments:

Answer 28

Belimumab (anti B lys)

Rituximab

Question 29

Sydney classification criteria of anti-phospholipid syndrome:

Answer 29

1. Vessel thrombosis
2. Spontaneous abortions/premature birth/fetal death
3. Anti-phospholipid Abs tested positive at least twice over 12 weeks apart (aCL, LA, beta-2GP1)

Question 30

Risk factors for aPL thrombosis:

Answer 30

Oestrogen
Pregnancy/puerperium
COX-2 inhibtitors
Long-distance travel

Question 31

Cerebral APS:

Answer 31

Ischaemia - stroke/TIA
Transverse myelitis
Give anticoagulation for focal manifestation
Give immunosuppression for diffuse manifestation

Question 32

aPL in pregnancy can cause…

Answer 32

Uteroplacental insufficiency and IUGR (fatal death + stillbirth)
Pre-eclampsia
Abruption
Premature delivery
Thrombosis
Give heparin and aspirin

Question 33

Nature of a course of anti-coagulation in aPL?

Answer 33

Indefinite even if become -ve
INR venous target is 2-3
INR arterial target is 3-4

Question 34

Differential between SLE Raynaud’s and primary Raynaud’s?

Answer 34

SLE is asymmetrical with macro nail-fold capillary loops, digital infarcts, auto-Abs and thumb involvement

Question 35

Treatment for Raynaud’s:

Answer 35

Keep warm, CCB: nifedipine

For advanced secondary: Sildenafil, prostacyclin infusions, Bosetan

Question 36

What does the CREST mnemonic signify in regards to systemic sclerosis?

Answer 36

Calcinosis
Raynaud's
Esophigitis
Scleroderma
Telangotasia

Question 37

Manifestations of systemic sclerosis:

Answer 37

Onion skinning in the renal arteries
GI symptoms
Pulmonary hypertension
Fibrotic, diffuse

Question 38

Treatment of systemic sclerosis:

Answer 38

Early: low dose steroids and immunosuppression
Late: symptom control

Question 39

What can ILD respond to if caught early?

Answer 39

Mycophenolate

Question 40

Dermatomyositis:

Answer 40

Heliotropic rash
Muscle weakness
Extended lesions on back of hands, elbows and knees

Question 41

Polymyositis:

Answer 41

Cancer associated
Inclusion body myositis
Muscle weakness - neck flexors, shoulder abduction and hip flexors
Raynaud's
Joint pain
Fever
Rashes