SLE Flashcards
Epidemiology?
Females in 30-50 age range
What type of hypersensitivity?
Type III hypersensitivity (immune complexes)
Which antibodies are present?
Anti-phospholipid Anti-nuclear antigens: Anti-dsDNA Anti-histone Anti-extractableNA (ENA) - Ro/La/RNP RF Anti mitochondrial: Anti-cardiolipin
Pathophysiology:
IC deposition Direct cytotoxicity NK cells and macrophages Complement consumption Activation of neutrophils
Skin symptoms?
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)
Joint symptoms?
Non-erosive arthritis
Tendinitis
Fibromyalgia - vague muscle pain, poor response to NSAIDs/analgesia
Sicca symptoms?
Dryness of exocrine glands: salivary, lacrimal, genital tract
Oral ulcers
Renal symptoms?
Oedema
Vascular signs?
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
Ches pain and SOB signs?
Pleuritis > pericarditis > peritonitis
Myocarditis, endocarditis
Premature arethromatous coronary artery disease
Rare: coexistent pleural effusions and shrinking lung syndrome
Leukopenia normally cause by what?
Lymphopenia rather than neutropenia
Investigations?
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
Treatment for joint symptoms and serositis?
Hydroxychloroquine
+Steroids
+Lifestyle
+Methotrexate and folic acid (if requiring steroids)
Treatment for mucocutaneous disease?
Lifestyle and supportive
+Hydroxychloroquine
+Steroids
+Methotrexate and folic acid (if requiring steroids)
Treatment for lupus nephritis?
Induction therapy + corticosteroid + hydroxychloroquine + lifestyle
+Maintenance regimen plus corticosteroid
Treatment for neuropsychiatric lupus?
Cyclophosphamide + corticosteroid + lifestyle
+IV immune globulin
+Plasmapheresis
+CNS pharmacotherapy
Why don’t you prescribe NSAIDs in SLE?
Increases the risk of strokes, MI and renal failure
Onset:
After menarche
Decline after menopause
Signs of subacute cutaneous lupus?
Most photo-sensitive rash in most areas
Associated with the Ro and La antibodies
What is chilblain lupus?
Cold lesions that look like vasculitis
Signs of small vessel lupus vasculitis?
Splinter haemorrhages Digital infarcts Palpable purpura Vasculitic ulcers Urticarial vasculitis
Signs of medium/large vessel lupus vasculitis?
Digital ischaemia
Gangrene
Needing amputation
Jaccoud’s arthritis:
Affects tendons and is not erosive so retain full range of movement - ask to make a fist
Lupus atherosclerosis?
CVD
Peripheral vascular disease
Features of lupus pneumonitis:
Acute organising pattern
Neuropsychiatric lupus:
Psychosis
Seizures
Stroke
Transverse myelitis
Lupus nephritis:
Nephrotic syndrome
Rapidly progressive GN (classes III + IV)
Proteinuria
Renal failure
Granular casts/dysmorphic fragmented red cells
Biologic treatments:
Belimumab (anti B lys)
Rituximab
Sydney classification criteria of anti-phospholipid syndrome:
- Vessel thrombosis
- Spontaneous abortions/premature birth/fetal death
- Anti-phospholipid Abs tested positive at least twice over 12 weeks apart (aCL, LA, beta-2GP1)
Risk factors for aPL thrombosis:
Oestrogen
Pregnancy/puerperium
COX-2 inhibtitors
Long-distance travel
Cerebral APS:
Ischaemia - stroke/TIA
Transverse myelitis
Give anticoagulation for focal manifestation
Give immunosuppression for diffuse manifestation
aPL in pregnancy can cause…
Uteroplacental insufficiency and IUGR (fatal death + stillbirth) Pre-eclampsia Abruption Premature delivery Thrombosis Give heparin and aspirin
Nature of a course of anti-coagulation in aPL?
Indefinite even if become -ve
INR venous target is 2-3
INR arterial target is 3-4
Differential between SLE Raynaud’s and primary Raynaud’s?
SLE is asymmetrical with macro nail-fold capillary loops, digital infarcts, auto-Abs and thumb involvement
Treatment for Raynaud’s:
Keep warm, CCB: nifedipine
For advanced secondary: Sildenafil, prostacyclin infusions, Bosetan
What does the CREST mnemonic signify in regards to systemic sclerosis?
Calcinosis Raynaud's Esophigitis Scleroderma Telangotasia
Manifestations of systemic sclerosis:
Onion skinning in the renal arteries
GI symptoms
Pulmonary hypertension
Fibrotic, diffuse
Treatment of systemic sclerosis:
Early: low dose steroids and immunosuppression
Late: symptom control
What can ILD respond to if caught early?
Mycophenolate
Dermatomyositis:
Heliotropic rash
Muscle weakness
Extended lesions on back of hands, elbows and knees
Polymyositis:
Cancer associated Inclusion body myositis Muscle weakness - neck flexors, shoulder abduction and hip flexors Raynaud's Joint pain Fever Rashes
