MTCD/UCTD

Question 1

Clinical characteristics of MCTD

Answer 1

Clinical signs from at least two of the following diseases

• JRA
• SLE
• JDM
• Systemic Scleroderma

Positive serological findings

• High titer of antibodies to U1 snRNP and the 70-kD A and C polypeptides
• Anti-U1RNA antibodies
• Presence of HLA DR4

Question 2

Age and Sex ratio

Answer 2

11 years (range  to 16) 
3 times more frequently in females than males

Question 3

Important players in pathogenesis

Answer 3

RNP reactive Cd4T cells

Vegf and endostatin

Question 4

Clinical manifestations

Answer 4

• Typically develop sequentially
• Rashes of cSLE and JDM are common at onset
• RP and arthriti are the most common manifestations at onset
• Sclerodermatous skin changes are slow to develop but may be prominent late in disease
• Occasionally myositis is the predominant syndrome
• Dysphagia and bowel dysmotility may occur
• Manifestations of sicca syndrome occur in 1/3
• Can have pulm functional impairments
• Nephritis is less common and less severe than in cSLE

Question 5

Long term outcomes

Answer 5

Pulmonary HTN
Severe Thrombocytopenia (often resistant to conventional therapy)