Systemic lupus erythematosus

Question 1

What?

Answer 1

Chronic systemic autoimmune disease

Immune system attacks cells & tissue resulting in inflammation & tissue damage

Question 2

Who?

Answer 2

Females
Childbearing years (20-40)
Afro- Caribbean, Hispanic American, Asian and Chinese ethnicity

Question 3

Why does this arise?

Answer 3

Due to overlap of genetic factors + hormonal factors + environmental factors + immunological factors

Question 4

Constitutional symptoms?

Answer 4

Fever
Fatigue
Weight loss

Question 5

MSK symptoms?

Answer 5

Arthralgia
Myalgia
Inflammatory arthritis 
Arthropathy
AVN of femoral head

Question 6

Muco- cutaneous symptoms?

Answer 6

Malar rash - butterfly rash
Photosensitivity
Discoid lupud
Subacute cutaneous lupu
Oral/ nasal ulceration
Raynaud's

Question 7

Cutaneous features?

Answer 7

Subacute cutaneous or discoid lupus 
Acute cutaneous lupus 
Non-scarring alopecia 
Oral ulceration 
Lupus rashes esp facial rash – often photosensitive (precipitated by UV light exposure) 
Butterfly rash on cheekbones 
Sparing of naso-labial fold

Question 8

Renal features?

Answer 8

Lupus nephritis

Question 9

Respiratory features?

Answer 9

Pleurisy 
Pleural effusion 
Pneumonitis  
Pulmonary embolism 
Pulmonary hypertension 
Interstitial lung disease

Question 10

Haematological features?

Answer 10

Leukopenia
Lymphopenia
Anemia (may be hemolytic), and thrombocytopenia

Question 11

Neuropsychiatric features?

Answer 11

Seizures, psychosis, headache, aseptic meningitis

Question 12

Cardiac features?

Answer 12

Pericarditis, pericardial effusion, pulmonary hypertension, sterile endocarditis and accelerated ischemic heart disease.

Question 13

GI features?

Answer 13

AI hepatitis
Pancreatitis
Mesenteric vasculitis

Question 14

Investigations?

Answer 14

FBC (anaemic, thrombocytopenia)
Immunology
Urinalysis
Imaging studies

Question 15

Immunology?

Answer 15

Anti- nuclear antibody (not specific, must be +)
Anti- dsDNA antibody (specific)
Anti- Sm - specific but low sensitivity
Anti Ro, anti La and anti- RNP (may be seen)
Low complement levels shows active disease

Question 16

Classification?

Answer 16

EULAR/ ACR SLE classification

Score 10

Question 17

Management of mild?

Answer 17

Hydroxychloroquine
Topical steroids
NSAIDs

Question 18

Management of moderate?

Answer 18

Inflammatory arthritis or organ involvement
Immunosuppression (azathioprine, mycophenolate mofetil, methotrexate)
Corticosteroids

Question 19

Management of severe?

Answer 19

Severe organ disease, haematological disease

IV steroids and cyclophosphamide

Question 20

All patients?

Answer 20

Sun protection measures
Hydrochloroquine
Monitor BP
Lifestyle
Minimise steroid use
Monitor using SLEDAI score

Question 21

Monitoring?

Answer 21

Check anti- dsDNA antibodies & complement levels regularly
Urinalysis for blood or protein
CV risk factors