8 - Mixed Connective Tissue

Question 1

S/s of undifferentiated connective tissue disease?

Answer 1

Nonspecific symptoms

• Arthralgias
• Myalgias
• Fatigue
• Cold sensitivity

Raynauds phenomenon
- unexplained synovitis

Question 2

Labs associated w undifferentiated connective tissue disease?

Answer 2

ANA (weakly pos)

Nail fold capillary micro (scleroderma)

U1-RNP antibodies + polyarthritis (MCTD)

ANA+ anti dsDNA (SLE)

Nucleolar antibodies + raynauds (scleroderma)

Synthetase and PM/Scl antibodies (myositis overlap syndrome)

Question 3

What is scleroderma overlap syndrome?

Answer 3

Pts exhibiting feature of >1 distincy clinical entity

Question 4

Scleroderma overlap syndromes?

Answer 4

Varients such as:
- CREST
Myositis and sclerodactyl and MCTD

Question 5

What is usally the 1st clinical feature to overlap with scleroderma overlap syndromes?

Answer 5

Raynauds

Question 6

Scleroderma overlap syndrome diagnostic findings?

Answer 6

Nail fold: Thickened and dilated capillaries

Pathologic autoantibodies (SCL-70)

CREST - overlap w biliary cirrhosis

Pulmonary fibrosis and pulm HTN

Question 7

What are the main causes of morbidity/mortality with scleroderma overlap syndrome?

Answer 7

Pulmonary fibrosis

Pulmonary HTN

Question 8

What makes mixed connective tissue disease different from SLE?

Answer 8

Sunlight doesnt precipitate MCTD as it does with SLE

Question 9

Environmental factors connected with MCTD?

Answer 9

Vinyl chloride

Silica

Question 10

MCTD S/S

Answer 10

Raynauds
Renal - glomerulonephritis
CNS - MC CN V neuropathy
Pulmonary HTN

Question 11

Early complaints of MCTD?

Answer 11

Easy fatiguability
Poorly defined myalgia
Arthralgias
Raynauds phenomenon
Swollen hands/puffy fingers

Question 12

MC cause of death with MCTD?

Answer 12

Pulmonary HTN

Question 13

Lab findings for MCTD?

Answer 13

High ANA tigers (speckled pattern)

Anti-U1-RNP antibodies

Question 14

Tx for MCTD?

Answer 14

No trials established tx

Treat the clinical features