Systemic Autoimmune Disease

Question 1

SLE: hypersensitivity type, triggers

Answer 1

CAUSE
Type 3 hypersensitivity ie. immune complex —> inflammation and destruction of tissue, activation of complement.

Triggered by:
-Sun
-Infection incl EBV
-Stress (physical or emotional)
-Smoking
-Infection

Drug-induced SLE is seperate, reversible entity:
-DRUGS: Sulfur, diuretics, penicillins, procainamide, isoniazid, hydralazine, antimalarials

Question 2

SLE diagnostic criteria:

Answer 2

Suspect in women of reproductive age who present with multiple/ recurrent/ unexpected symptoms:

More than 4 of:

Malar (butterfly) rash
Discoid rash
Photosensitivity

Seizure
Psychosis

Oral ulcers

Pleuritis
Pericarditis
Arthritis (non erosive)

Proteinutria or casts in urine
Haeme: MAHA-haemolytic anaemia, thrombocytopaenia, leukopaenia

Immune testing:
ANTINUCLEAR ANTIBODY
—> Most specific (not pathognomic)
—> 98% will be positive
+Anti phospholipid, Anti smooth muscle, Anti dsDNA

Question 3

SLE management:

Answer 3

Generally suspected:
- Examine all systems carefully
- History re triggers and drugs
- Screen: FBC, urine
- Outpatient ANA
- Avoid triggers

Flare- DW Rheum:
1- STERIOID
—> Methylpred, prednisolone,

2- OTHER IMMUNOSUPPRESSANTS
-Azathioprine
-Hydroxyquinolone
-MTX
-Mycophenylate

SEVERE:
—> Cyclophosphamide (chemo)

Question 4

What is the significance of SLE in chest pain?

Answer 4

Likely to be something bad
In usually benign patient group: young women.

Increased risk of:
CAD 50-fold
VTE (incl PE) 30-fold

Higher risk of pleural effusion, pericarditis, myocarditis, tamponade, dissection….

Question 5

What is lupus anticoagulant?

Answer 5

An immunoglobulin that increases the risk of venous and arterial thromboembolism

Up to 30% of SLE patients will be + for it

AKA antiphospholipid syndrome

Question 6

Answer 6

Malar rash
50% SLE
After sun exposure

Question 7

Sarcoidosis:

Answer 7

Abnormal collections of granulomas (inflammatory cells).
Commonly in:
LUNGS 90% of the time
—> Hilar lymphadenopathy
Brain
Heart
—> Conduction disturbance
—> Restrictive cardiomyopathy
Skin
—> Lupus Pernio rash (pathognomic)
+Hypercalcaemia

Multiple symptoms possible- Inflamm, infiltrative
Most patients are asymptomatic

Steroids if needed.