MCTD - finished

Question 1

Define MCTD

Answer 1

A rheumatic disease characterised by overlapping clinical features similar to those of SLE, scleroderma and PMDM.

Question 2

Features supporting MCTD being its own entity?

Answer 2

High titers of anti-U1 ribonucleoprotein complex ANA in the absence of other significant ANA’s
Normal macrophage clearance of immune complexes compared with SLE
T-cells abnormalities differ from those in other rheumatic diseases

Question 3

Aetiology of MCTD: Evidence for it being immune mediated:

Answer 3

Marker hypergammaglobulinaemia
Extremely high titres of anti-RNP antibodies
Mild/moderate hypocomplimentaemia
Circulating immune complexes

Question 4

Epidemiology of MCTD

Answer 4

80% female

Typical age of onset 15-25

Question 5

What are the main tissues affected by MCTD?

Answer 5

Skin
Joints
Muscles

Question 6

What are the main skin pathologies MCTD?

Answer 6

Scleroderma-like changes
Heliotrope rash of eyelids
Lupus like rashes
Diffuse (non-scarring) alopecia
Telangiectasia

Question 7

What are the main joint pathologies in MCTD?

Answer 7

Arthritis - can be erosive and deforming

Question 8

What are the main muscle pathologies in MCTD?

Answer 8

Inflammatory infiltrates

Myopathy (degeneration of muscle fibres due to inflammation)

Question 9

Clinical manifestations of MCTD?

Answer 9

Arthritis (90%)
Swollen, puffy hands (90%)
Raynauds phenomenon (90%)
Anaemia and leocopoenia (50%)
Oesophageal hypomotility (50-80%)
Pulmonary involvement (80%)
Lymphadenopathy (25%)
Pericarditis (25%)

Question 10

What is the typical cinical manifestations for MCTD?

Answer 10

PRAISE that we dont have MCTD

Pulmonary disease
Raynauds phenomenon
Arthritis/polyarthralgia
Inflammatory proximal myopathy
Swollen hands
oEsophageal hypomotility