Mixed connective tissue disease

Question 1

MIxed connective tissue disease displays features of which 3 conditions?

Answer 1

systemic lupus erythematosus (SLE)
systemic sclerosis (SSc)
myositis

Question 2

Antibodies found in mixed connective tissue disease

Answer 2

anti-U1 ribonucleoprotein (RNP) antibodies.

Question 3

Typical demographic

Answer 3

Females>males (3:1)
Age 30-40

Question 4

Common features at presentation

Answer 4

Raynaud’s phenomenon precedes other symptoms (90%)
Polyarthralgia/arthritis
Myalgia
dactylitis

Question 5

Dermatological features

Answer 5

photosensitive rash
scleroderma-like changes
alopecia

Question 6

Respiratory features

Answer 6

pleuritis
pulmonary hypertension
interstitial lung disease

Question 7

Haematological features

Answer 7

anaemia
lymphadenopathy
splenomegaly
rarely TTP

Question 8

Cardiac features

Answer 8

pericarditis
pericardial effusion
accelerated coronary artery disease

Question 9

Renal features

Answer 9

glomerulonephritis (tends to be milder than SLE)

Question 10

Neuropsychiatric features

Answer 10

seizures
mood disturbance

Question 11

Describe the pattern of antibodies which are positive and negative in mixed connective tissue disease

Answer 11

ANA positive
anti Ds-DNA negative
Anti-Scl70 negative
Anti-U1 RNP positive

Question 12

Management of mixed connective tissue disease

Answer 12

Calcium channel blockers for Raynaud’s
PPI for reflux
Endothelin receptor antagonists/prostacyclin analogues in pulmonary hypertension
Smoking cessation
Moderate exercise