SLE

Question 1

What is Lupus?

Answer 1

-Chronic inflammatory multi-system disease
-Most common in young women (9:1), onset 20-40 yrs, Afro-Caribbean
-Incidence risen substantially in 50 years
-Constitutional symptoms, skin rash and arthralgia commonest complaints

Question 2

Pathophysiology in SLE

Answer 2

-Autoimmune disease: SLE a type 3 hypersensitivity reaction
-Associated with HLA B8, DR2, DR3
-Thought to be caused by immune system dysregulation leading to immune complex formation
-Immune complex deposition can affect any organ including the skin, joints, kidneys and brain

Question 3

Morbidity, Mortality and Causes of death in SLE

Answer 3

-10% mortality over 20 years and mean age of death is 53
-Patients still dying 25 yrs earlier than mean for UK

-Infection
-Lupus nephritis (LN), renal failure and its complications
=LN is silent and ~50% will develop some form of renal disease. 20% of LN will have ESRD in 10 years (mean age death at 40)
-Cardiovascular disease
-CNS lupus

Question 4

Clinical constitutional symptoms

Answer 4

-Fatigue- 80-100%
-Fever- 50%
-Myalgia
-Weight loss
-Lymphadenopathy

Question 5

Clinical skin manifestations of lupus

Answer 5

-SCLE: subacute cutaneous lupus erythematosus;
-ACLE: acute cutaneous lupus erythematosus;
-LE: lupus erythematosus;
-DLE: discoid lupus erythematosus (scaly, erythematous, well-demarcated rash in sun-exposed areas, may progress to become pigmented and hyperkeratotic before atrophic)
-SLE: systemic lupus e

Question 6

Clinical features of acute cutaneous LE

Answer 6

-Macular rash/ malar butterfly rash that spares nasolabial folds
-Photosensitivity
-Generalised ACLE

Question 7

Clinical features of subacute LE

Answer 7

-80% are anti Ro/La+ve and may have features of Sjogren’s Syndrome Arthralgias and oral ulcers present but cytopenias and serositis occur less frequently.
-Neonatal lupus also associated with anti Ro/La+ve antibodies. Complete heart block may also occur in 2nd trimester leading to miscarriage

Question 8

Clinical features of chronic cutaneous LE

Answer 8

-A well-defined, erythematous plaque with scale, pigmentary alteration, and scarring is present on this patient with discoid lupus erythematosus
-Discoid lupus erythematosus affecting the scalp and face. There are discoid plaques over eyebrows, forehead, and
scalp, with post inflammatory peripheral pigmentation, scarring, and alopecia.

Question 9

Joint manifestations of SLE

Answer 9

-Arthralgia
-Symmetrical, non-erosive synovitis
-Jaccoud’s arthropathy (reducible deformities): secondary to ligament loosening rather than bone or cartilage destruction

Question 10

Mucosal manifestations of SLE

Answer 10

-Mouth ulcers
-Sicca symptoms (secondary Sjögren’s syndrome)

Question 11

Clinical lung features of SLE

Answer 11

-Pleurisy
-Fibrosing alveolitis

-PE
-Secondary Infection
-Acute lupus pneumonitis ± Pulmonary haemorrhage
-Chronic interstitial lung disease
-Pulmonary hypertension
-“Shrinking lung”= decreased lung volume without parenchymal disease often due to diaphragmatic weakness

Question 12

Cardiac manifestations of SLE

Answer 12

-Pericarditis
-Myocarditis

-Libman-Sacks endocarditis
-Coronary artery disease (premature)

Question 13

Vasculitic rashes of SLE

Answer 13

-Small vessel vasculitis= digital infarcts
-Livedo reticularis
-Raynaud’s phenomenon

Question 14

Describe lupus nephritis

Answer 14

-Can rapidly lead to irreversible renal tissue damage- diffuse proliferative glomerulonephritis
-Renal disease has been the most common cause of death in patients with lupus
-Routinely check: U&E, urinalysis, BP
-Consider renal biopsy if: proteinuria > 0.5 g/24 hours
Casts, red or white cells in urine creatinine clearance reduced (<80ml/min)

Question 15

Neurological manifestations of SLE

Answer 15

-Headache/Migraine
-Anxiety and depression!
-Stroke syndromes
-Seizures!
-Cognitive dysfunction
-Psychosis!
-Chorea
-Mononeuropathy (single/multiplex)
-Polyneuropathy
-Spinal cord lesions

Question 16

Describe Anti-phospholipid antibody syndrome

Answer 16

-Recurrent venous thrombosis
=deep vein thrombosis 32%
=pulmonary embolus 9%
-Recurrent arterial thrombosis
=myocardial infarction 8%
=stroke (cerebro-vascular accident) 13%
-Pregnancy morbidity (Recurrent miscarriage, late miscarriage, severe pre-eclampsia) 8%
-Livido Reticularis 20%
-Superficial thrombophlebitis 9%
-Thrombocytopenia 22%
-Also more rarely cardiac valve disease, pulmonary hypertension, thrombocytopenia, cutaneous ulcers and adrenal insufficiency due to haemorrhagic infarction, and
cognitive deficits

Question 17

Laboratory features of APLS

Answer 17

-Thrombocytopenia
-Presence of lupus anticoagulant and/or anticardiolipin antibodies (principal autoantigen is beta2-glycoprotein)
-Always test for aPLs at baseline (+ve LA, aCL, anti beta-2 glycoprotein-1. X2 12 weeks apart-Paradoxical rise in APTT

Question 18

Treatment of APLS

Answer 18

-For those with thrombosis, long-term anticoagulation with warfarin reduces thromboembolic events target INR 2-3)
-Management/prevention of pregnancy complications includes the use of aspirin ± LMW heparin

Question 19

Investigations in SLE

Answer 19

-Antibodies
=Anti-nuclear antibody-5% of healthy people
=Anti-ds DNA, Anti-Smith (Sm) and low complement is highly predictive but only with relevant clinical features
=Anti Ro and La-found in other CTDs e.g Sjogrens Syndrome
=C3> sensitive than C4 and if normal have high predictive value to exclude active disease

-CXR, ECG, urinalysis

-Monitoring
=ESR
=CRP (can be normal in active disease)
=Complement levels (C3, 4) low during active disease
=Anti-dsDNA titres

Question 20

Treatment of SLE

Answer 20

-Sunblock, education, infection, fertility

-Symptomatic treatment: analgesics, NSAIDs
-Hydroxychloroquine (skin & joints)!

-Careful assessment and treatment of cardiovascular risk
-Immunosuppressive treatment
=Corticosteroids - topical, low dose or high dose (oral/IM/IV/IA)
-Rituximab (for refractory lupus). Belimumab
BSR guidelines

Question 21

Definition of mild disease activity

Answer 21

-SLEDAI-2K score of <6
-Clinically stable disease with no life-threatening organ involvement
-Not likely to cause scarring or damage.

Question 22

Definition of moderate disease activity

Answer 22

-SLEDAI-2K score in the range of 6-12
-Would cause significant chronic scarring

Question 23

Definition of severe disease activity

Answer 23

-Organ or life threatening
-SLEDAI-2K score of >12
-Requires rapid potent immunosuppression.