Mixed connective tissue disease

Question 1

M:F predominance of MCTD?

Answer 1

• strong F:M (9:1)

Question 2

MCTD is characterized by overlap of at least 2 of the following CTD:

Answer 2

• SLE
• PM/DM
• scleroderma
• RA

Question 3

Which antibodies are thought to have pathogenic role in MCTD?

Answer 3

Anti-U1RNP

Question 4

What is the HLA association with MCTD?

Answer 4

HLA-DR4

Question 5

Most common constellation of findings in MCTD:

Answer 5

• raynauds
• esophageal dysfunction
• swollen fingers/hands
• arthralgias
• inflammatory myopathy

Question 6

What is the most serious complication of MCTD?

Answer 6

• pulmonary HTN (accounts for 40% of deaths in MCTD patients)

Question 7

What will ANA of MCTD show?

Answer 7

• speckled nuclear pattern

Question 8

Which autoantibodies will have high titers in MCTD?

Answer 8

anti-U1RNP (serologic hallmark of MCTD)

Question 9

How can you differentiate MCTD from SLE?

Answer 9

• MCTD will have negative anti-dsDNA and anti-smith ab

Question 10

What is first line tx for severe arthritis in MCTD? What do you have to closely monitor when administering this?

Answer 10

• MTX

- frequent pulmonary fibrosis occurs in MCTD patients on MTX

Question 11

first line tx fort MCTD?

Answer 11

Prednisone

Question 12

Prognosis and response to tx for MCTD?

Answer 12

• good response to prednisone and favorable prognosis

Question 13

____% of MCTD patients will evolve into one of the six diffuse CTDs.

Answer 13

40%

Question 14

1 cause of death in MCTD?

Answer 14

Pulmonary artery HTN